Histology-specific clinical trial of lenvatinib and pembrolizumab in patients with sarcoma

Fetched: October 16th, 2024, 2:00am UTC by Sujana Movva

Clin Cancer Res. 2024 Oct 15. doi: 10.1158/1078-0432.CCR-24-2519. Online ahead of print.

ABSTRACT

PURPOSE: Survival of patients with metastatic sarcoma remains poor, and there is pressing need for new therapies. Most sarcoma subtypes are not responsive to immune checkpoint inhibition alone. Lenvatinib, a multi-receptor tyrosine kinase inhibitor targeting tumor vasculature, has immunomodulatory activity that contributes to its antitumor effects. Therefore we hypothesized that combination of lenvatinib and pembrolizumab would lead to improved clinical outcomes in patients with sarcoma.

METHODS: This was an open-label, single-arm study of lenvatinib and pembrolizumab in the following cohorts A: leiomyosarcoma, B: undifferentiated pleomorphic sarcoma (UPS), C: vascular sarcomas (angiosarcoma and epithelioid hemangioendothelioma), D: synovial sarcoma or malignant peripheral nerve sheath tumor (MPNST), and E: bone sarcomas (osteosarcoma and chondrosarcoma). The primary endpoint was best overall response (BOR) rate documented by RECIST v1.1 by 27 weeks in each cohort, with a threshold of â‰¥2 responses among 10 patients. Secondary endpoints included progression-free survival, overall survival, duration of response and safety.

RESULTS: Forty-six patients were evaluable for the primary endpoint which was met in the UPS and MPNST/synovial cohorts (BOR rates by 27 weeks of 25% and 30%,respectively). There were 7 partial responses overall with additional responses noted in angiosarcoma and osteosarcoma. Treatment-related adverse events of any grade, and Grade 3 or higher, occurred in 50/51 (98%) and 29/51 (57%) of patients respectively.

CONCLUSIONS: We observed durable responses in MPNST, synovial sarcoma and osteosarcoma. Patients with UPS and angiosarcoma also responded. Further exploration of this approach is warranted to confirm activity and determine optimal dosing schedules.

PMID:39405335 | DOI:10.1158/1078-0432.CCR-24-2519
Primary Pulmonary Epithelioid Angiosarcoma Presenting in a 59-Year-Old Woman: A Report of a Rare Case

Fetched: October 16th, 2024, 2:00am UTC by Muhammad Tahir

Cureus. 2024 Sep 11;16(9):e69191. doi: 10.7759/cureus.69191. eCollection 2024 Sep.

ABSTRACT

Primary pulmonary angiosarcomas are rare malignancies, with aggressive clinical behavior and poor prognosis. Here we present a case of a rare primary pulmonary epithelioid angiosarcoma in a 59-year-old woman who initially presented with right-sided chest pain and shortness of breath. Chest X-ray revealed right lower lobe atelectasis, while a chest computed tomography angiography (CTA) showed a large right hydrothorax with collapse of most of the right lung. A right lower lobe resection was performed and histologic and immunohistochemical analyses were consistent with a primary pulmonary epithelioid angiosarcoma. The patient was discharged, given supportive care, and died 12 days following her last operation.

PMID:39398746 | PMC:PMC11469199 | DOI:10.7759/cureus.69191
Neoplasia in pet guinea pigs: a retrospective analysis of 2,474 autopsy examinations

Fetched: October 16th, 2024, 2:00am UTC by Christof A Bertram

J Vet Diagn Invest. 2024 Oct 14:10406387241288642. doi: 10.1177/10406387241288642. Online ahead of print.

ABSTRACT

Neoplasia is a common disease in guinea pigs (Cavia porcellus); however, few studies have evaluated the prevalence of neoplasia in all organ systems. We retrospectively analyzed the tumor prevalence in pet guinea pigs and the frequency of metastasis in a multi-institutional study population of 2,474 autopsy cases. Tumors were found in 508 guinea pigs (prevalence: 20.5%), of which 95 cases had >1 tumor, resulting in a total of 627 tumors. The tumor prevalence increased from 1.4% in animals <0.5-y-old to 53.6% for guinea pigs >5-y-old. The most common tumor type was lymphoma or leukemia, affecting 174 guinea pigs (tumor prevalence: 7.0%). Lymphomas or leukemias were disseminated to various organs and/or lymph nodes in 146 (83.9%) cases and localized to 1 organ or 1 lymph node in 28 (16.1%) cases. Primary non-lymphoid tumors were most frequent in the female genital tract (62 of 1,235 cases, mostly uterus), respiratory system (116 of 2,474), skin including mammary gland (81 of 2,474), endocrine system (66 of 2,474, mostly thyroid gland), and alimentary tract (35 of 2,474). Tumors of the alimentary tract were dominated by gastrointestinal stromal tumors. Metastasis was detected in 42 of 453 non-lymphoid tumors (9.3%), with a surprisingly low frequency for pulmonary carcinoma and splenic hemangiosarcoma compared to other species. Our postmortem study demonstrates a high prevalence of disseminated lymphoma or leukemia in pet guinea pigs at the time of death or euthanasia. Additional studies are needed to further characterize these tumors.

PMID:39397656 | DOI:10.1177/10406387241288642
An Infiltrative Case of Angiosarcoma Causing Portal Hypertension

Fetched: October 16th, 2024, 2:00am UTC by Gina Bae

ACG Case Rep J. 2024 Oct 10;11(10):e01532. doi: 10.14309/crj.0000000000001532. eCollection 2024 Oct.

ABSTRACT

Hepatic angiosarcoma is a rare and aggressive liver tumor. We report a case study of an 82-year-old elderly gentleman who presented with failure to thrive and ascites. Initially suspected to be cirrhosis, biopsy results eventually concluded angiosarcoma of the liver. Our patient presented with an infiltrative form, rather than distinct masses, which led to portal hypertension and ascites. The variance in symptomatology and radiology presentations make a diagnosis of hepatic angiosarcoma challenging and require a high index of suspicion.

PMID:39391806 | PMC:PMC11466120 | DOI:10.14309/crj.0000000000001532
Treatment of malignant primary cardiac tumors requires attention to cardiovascular complications: a single-center, retrospective study

Fetched: October 16th, 2024, 2:00am UTC by Kanami Furukawa

Jpn J Clin Oncol. 2024 Oct 10:hyae138. doi: 10.1093/jjco/hyae138. Online ahead of print.

ABSTRACT

BACKGROUND: Malignant primary cardiac tumors require multimodal approaches including surgery, chemotherapy and radiotherapy, but these treatments can be associated with cardiovascular complications. However, few reports have described the cardiovascular complications related to primary cardiac tumor treatment because of their rarity.

METHODS: Clinical records of patients with primary cardiac tumors treated at Kyushu University Hospital from January 2010 to August 2021 were retrospectively examined.

RESULTS: Of the 47 primary cardiac tumor patients, 13 (28%) were diagnosed with malignancy, including 5 angiosarcomas, 3 intimal sarcomas, 3 diffuse large B-cell lymphomas, 1 Ewing's sarcoma and 1 fibrosarcoma. Cardiovascular events were observed in 10 patients (77%), including cardiac dysfunction in 6 patients, arrhythmias in 5 patients, right heart failure in 2 patients, and excessively prolonged prothrombin time due to the combination of warfarin and chemotherapy in 1 patient. Two patients who showed notable cardiac complications are described. Case A involved a 69-year-old woman who underwent surgery for a left atrial intimal sarcoma, followed by postoperative chemotherapy with doxorubicin plus ifosfamide and radiotherapy. After three cycles of chemotherapy and sequential radiotherapy, her left ventricular ejection fraction decreased to 34%, and ongoing heart failure therapy was required. Case B involved a 66-year-old man who received chemotherapy for primary cardiac lymphoma, resulting in tumor shrinkage. However, due to tumor involvement of the intraventricular septum, atrioventricular block developed, requiring cardiac pacemaker implantation.

CONCLUSION: High incidences of cardiac failure and arrhythmias were observed during multimodal treatments for malignant primary cardiac tumors. Proper management of complications may lead to a favorable prognosis in patients with malignant primary cardiac tumors.

PMID:39385509 | DOI:10.1093/jjco/hyae138
Spontaneous splenic rupture as a primary manifestation of angiosarcoma: a case report

Fetched: October 16th, 2024, 2:00am UTC by Ameer Awashra

J Surg Case Rep. 2024 Oct 8;2024(10):rjae633. doi: 10.1093/jscr/rjae633. eCollection 2024 Oct.

ABSTRACT

Angiosarcoma, a rare endothelial cell tumor, accounts for < 2% of all sarcomas, typically affecting elderly patients. We present the case of a 62-year-old male with a history of hypertension and ischemic heart disease, who initially presented with severe lower back pain. Subsequent imaging revealed a ruptured splenic lesion and multiple liver lesions, leading to a diagnosis of stage 4 angiosarcoma. Post-splenectomy, the patient exhibited pathological vertebral fractures and elevated liver function tests, consistent with metastatic disease. Despite aggressive surgical intervention and supportive care, the patient's condition remained critical. The treatment plan included palliative chemotherapy, spine radiotherapy, and ongoing pain management, with close monitoring of disease progression and response to therapy. This case describes the diagnostic challenges and poor prognosis associated with metastatic angiosarcoma.

PMID:39380797 | PMC:PMC11460614 | DOI:10.1093/jscr/rjae633
A Rare Encounter: Hepatomegaly Unmasked as Multiple Giant Hepatic Hemangiomas

Fetched: October 16th, 2024, 2:00am UTC by Vasundara Gopalan

Cureus. 2024 Sep 6;16(9):e68814. doi: 10.7759/cureus.68814. eCollection 2024 Sep.

ABSTRACT

A hepatic hemangioma is a benign liver tumor made up of a number of blood-filled chambers surrounded by liver-supplied endothelial cells. Most liver hemangiomas are asymptomatic and are only discovered during imaging studies for other conditions. Ultrasound is used for initial screening followed by a computed tomography scan, which shows slow enhancement due to small vessels and can be used to diagnose the location, number, and size of a hepatic hemangioma. A large liver hemangioma can range in size from 10 centimeters to more than 20 centimeters and can cause symptoms and complications that require prompt intervention. Hepatic hemangiomas can co-occur with other localized hepatic lesions; a careful diagnosis is necessary to distinguish them. In this case study, a 48-year-old woman complained of a stomachache that had persisted for three months. Following an initial clinical evaluation, hepatomegaly was found, and contrast-enhanced computed tomography (CECT) abdomen and pelvis was performed, revealing numerous giant hepatic hemangiomas. Significant improvements were noted in the patient's condition with tumor embolization.

PMID:39376836 | PMC:PMC11458059 | DOI:10.7759/cureus.68814
Laparoscopic excision of an incidental vascular tumor

Fetched: October 16th, 2024, 2:00am UTC by Sofia Boligo

J Surg Case Rep. 2024 Oct 5;2024(10):rjae624. doi: 10.1093/jscr/rjae624. eCollection 2024 Oct.

ABSTRACT

Anastomosing hemangioma (AH) is an exceedingly rare benign vascular tumor, often mistaken for malignant neoplasms due to its histological features. First described in 2009, AH has been documented in various sites, including the kidney, liver, and adrenal gland. This report presents a 64-year-old man who, following a bicycle accident, underwent imaging that revealed a nodule suspicious for a paraganglioma in the right para-caval region. Despite the high radiotracer uptake on 68Ga-DOTANOC PET scan, which suggested paraganglioma, the patient remained asymptomatic and laboratory tests ruled out excessive catecholamine secretion. Surgical excision led to a diagnosis of AH, distinguished by its unique anastomosing vascular pattern and histological similarity to angiosarcoma. This case underlines the diagnostic challenges and potential for overtreatment of AH, highlighting the need for heightened awareness and careful histopathological and imaging evaluation to avoid misdiagnosis and ensure appropriate management.

PMID:39376719 | PMC:PMC11456863 | DOI:10.1093/jscr/rjae624
Radiation-Induced Angiosarcoma Arising in Bilateral Breast: A Case Report

Fetched: October 16th, 2024, 2:00am UTC by Wakako Tsuji

Cureus. 2024 Sep 6;16(9):e68782. doi: 10.7759/cureus.68782. eCollection 2024 Sep.

ABSTRACT

Radiation-induced angiosarcoma is a highly aggressive malignancy. We encountered a case of angiosarcoma arising in the bilateral breast seven years after partial mastectomy and the last radiation therapy. As recommended, wide resection with skin grafting was performed to ensure negative surgical margins. After surgery, taxane-based chemotherapy was administered. However, adjuvant chemotherapy was discontinued because of drug-induced institutional pneumonia. Eighteen months postoperatively, angiosarcoma recurred in subcutaneous, lymph nodes, lungs, and bones. The disease progressed too rapidly, and the patient died two months after recurrence.

PMID:39371746 | PMC:PMC11456261 | DOI:10.7759/cureus.68782
Genomic landscape and preclinical models of angiosarcoma

Fetched: October 16th, 2024, 2:00am UTC by Annaleigh Benton

Mol Oncol. 2024 Oct 5. doi: 10.1002/1878-0261.13744. Online ahead of print.

ABSTRACT

Angiosarcoma is a cancer that develops in blood or lymphatic vessels that presents a significant clinical challenge due to its rarity and aggressive features. Clinical outcomes have not improved in decades, highlighting a need for innovative therapeutic strategies to treat the disease. Genetically, angiosarcomas exhibit high heterogeneity and complexity with many recurrent mutations. However, recent studies have identified some common features within anatomic and molecular subgroups. To identify potential therapeutic vulnerabilities, it is essential to understand and integrate the mutational landscape of angiosarcoma with the models that exist to study the disease. In this review, we will summarize the insights gained from reported genomic alterations in molecular and anatomic subtypes of angiosarcoma, discuss several potential actionable targets, and highlight the preclinical disease models available in the field.

PMID:39367667 | DOI:10.1002/1878-0261.13744
Computed tomographic features of pulmonary and extrapulmonary lesions can be useful in prioritizing the diagnosis of hemangiosarcoma metastases in dogs

Fetched: October 16th, 2024, 2:00am UTC by Mirko Mattolini

Am J Vet Res. 2024 Oct 3:1-9. doi: 10.2460/ajvr.24.08.0219. Online ahead of print.

ABSTRACT

OBJECTIVE: The aim of this study was to evaluate the CT features of pulmonary metastases in dogs with hemangiosarcoma (HSA) at various sites. Additionally, the CT characteristics of extrapulmonary metastases in the same population were assessed.

METHODS: Retrospective, observational, and descriptive study conducted from April 2013 to January 2024. Dogs with histologically confirmed HSA and suspected or cytologically/histologically confirmed lung metastases were included. Dogs were excluded if they had a second primary tumor or only 1 unsampled pulmonary nodule.

RESULTS: 33 dogs were included, with 26/33 [78.8%] having more than 10 metastatic pulmonary nodules. Most nodules were generalized (24/33 [72.7%]), miliary (29/33 [87.9%]) to subcentimetric (32/33 [97%]) in size, well-defined margins (29/33 [87.9%]), or a perilesional halo sign (24/33 [72.7%]). When more than 10 nodules were present, a generalized distribution was prevalent, while a peripheral location was more common when 2 to 10 nodules were present (P < .0001). In 32/33 (97%) cases, a pulmonary vessel was directly connected to the nodule (feeding vessel). After contrast administration, most lung metastases appeared homogenous (26/33 [78.8%]), although some showed areas of intense enhancement (5/33 [15.1%]) a feature also observed in extrapulmonary metastases with varying frequency (0% to 85.7%).

CONCLUSIONS: Pulmonary HSA metastases were characterized by generalized, small (miliary/subcentimetric), well-defined nodules, commonly associated with a halo sign and feeding vessel. Intralesional areas of spotty postcontrast linear or amorphous strong hyperdensity were frequently observed especially in extrapulmonary metastases.

CLINICAL RELEVANCE: These features may help radiologists and clinicians orient their diagnosis toward metastatic HSA.

PMID:39362269 | DOI:10.2460/ajvr.24.08.0219
Persistent Thrombotic Hemangioma With Organizing/Anastomosing Features: A Case Report of a Guanine Nucleotide-Binding Protein Alpha Subunit (GNA)-Mutated Cutaneous Vascular Lesion

Fetched: October 16th, 2024, 2:00am UTC by Svetlana Bobkova

Cureus. 2024 Sep 2;16(9):e68446. doi: 10.7759/cureus.68446. eCollection 2024 Sep.

ABSTRACT

Thrombotic hemangioma with organizing/anastomosing features (THOA) is a newly identified variant within the spectrum of hemangiomas that harbor mutations in the guanine nucleotide-binding protein alpha subunit (GNA) genes (like GNAQ or GNA11). While THOA shares similarities with anastomosing hemangioma, it possesses distinct clinical and morphological characteristics that make it a separate entity. All reported cases of THOA have demonstrated benign behavior. However, histologic features such as anastomosing vascular growth, mitotic figures, and endothelial hobnailing may raise concerns for a low-grade malignant vascular neoplasm. We report the case of a 74-year-old female with an unremarkable medical history who presented with a vascular lesion on her upper torso. The lesion persisted after the initial biopsy and was re-excised, displaying similar histologic characteristics. Next-generation sequencing (NGS) revealed a GNAQ mutation (p.Q209H) in both samples. Notably, a TP53 mutation (p.R273H) was detected in the first specimen but was absent in the subsequent excision. The lesion was diagnosed as persistent THOA. This case report discusses the salient features, genetic profile, and prognosis of this uncommon lesion.

PMID:39360118 | PMC:PMC11446179 | DOI:10.7759/cureus.68446
A unique presentation of pericardial epithelioid angiosarcoma with multifaceted complications

Fetched: October 16th, 2024, 2:00am UTC by Moied Al Sakan

Ann Med Surg (Lond). 2024 Aug 30;86(10):6266-6271. doi: 10.1097/MS9.0000000000002521. eCollection 2024 Oct.

ABSTRACT

INTRODUCTION AND IMPORTANCE: Angiosarcomas are rare tumors of endothelial origin and may arise in any organ. Epithelioid angiosarcomas are a subtype of angiosarcoma that are rapidly progressive and typically fatal.

CASE PRESENTATION: The authors report a case of a 25-year-old previously healthy female who presented initially for dyspnea and palpitations, on further evaluation she was found to have large bilateral pleural effusions and cardiac tamponade.

CLINICAL DISCUSSION: Pericardiocentesis and thoracentesis were performed alongside biopsies that revealed atypical cellular proliferation.Fluorodeoxyglucose-positron emission tomography (FDG-PET) showed avid uptake in the anterior mediastinum, perivascular, paratracheal, subcarinal and pleural lymph nodes with large FDG uptake in the bilateral pleural effusion.Mediastinoscopy was done and biopsies showed an overtly malignant, epithelioid neoplasm with foci of vaso-formation; Keeping with high-grade epithelioid angiosarcoma of the pericardium.She received six cycles of weekly paclitaxel, but imaging for abdominal pain incidentally showed evidence of metastasis to the liver and spine so she was switched to Adriamycin-Ifosfamide for which she received one cycle so far.Her hospital course was complicated by high-output pleural effusions, chylothorax, left atrial thrombus formation and an intensive care unit stay for septic shock.

CONCLUSION: Pericardial epithelioid angiosarcoma has been reported rarely in the literature. The authors aim to report a case of extensive metastatic pericardial epithelioid angiosarcoma in a young patient; which we believe can be an addition to the literature of a malignancy associated with poor prognosis and no definitive proven treatment regimen.

PMID:39359772 | PMC:PMC11444636 | DOI:10.1097/MS9.0000000000002521
Radioembolization of Primary Splenic Angiosarcoma Complicated by Delayed Gastric Hemorrhage

Fetched: October 16th, 2024, 2:00am UTC by Aditya Garg

J Vasc Interv Radiol. 2024 Sep 30:S1051-0443(24)00608-0. doi: 10.1016/j.jvir.2024.09.014. Online ahead of print.

NO ABSTRACT

PMID:39357601 | DOI:10.1016/j.jvir.2024.09.014
Ruptured aortic aneurysm with previous endovascular aneurysm repair in a patient with high-grade angiosarcoma

Fetched: October 16th, 2024, 2:00am UTC by Ievgen Gegiia

J Vasc Surg Cases Innov Tech. 2024 Aug 22;10(6):101610. doi: 10.1016/j.jvscit.2024.101610. eCollection 2024 Dec.

ABSTRACT

Ruptured aortic aneurysms after endovascular repair is rare, particularly in the absence of type I or type III endoleaks. In such cases, a thorough investigation into the causes is imperative, including the consideration of an underlying malignancy. We report a case involving a 78-year-old woman who experienced abdominal aortic aneurysm rupture 4 years after aortic endograft treatment. We explanted the endograft and performed aortobi-iliac bypass. Initial aortic thrombus pathological analysis revealed atherosclerosis. However, the patient returned 4 months later with multiple lesions suggestive of metastases, and a reevaluation of the pathology slides uncovered a diagnosis of angiosarcoma.

PMID:39351206 | PMC:PMC11439839 | DOI:10.1016/j.jvscit.2024.101610
Pseudoangiosarcoma and cutaneous collagenous vasculopathy in a patient on aÂ Bruton's tyrosine kinase inhibitor

Fetched: October 16th, 2024, 2:00am UTC by Alison H Kucharik

JAAD Case Rep. 2024 Jul 1;51:17-21. doi: 10.1016/j.jdcr.2024.06.011. eCollection 2024 Sep.

NO ABSTRACT

PMID:39345278 | PMC:PMC11437239 | DOI:10.1016/j.jdcr.2024.06.011
Primary cardiac angiosarcoma: an unexpected presentation

Fetched: October 16th, 2024, 2:00am UTC by Marco Martinelli

G Ital Cardiol (Rome). 2024 Oct;25(10):752-755. doi: 10.1714/4336.43218.

ABSTRACT

Among cardiac tumors, angiosarcoma is the most common primary malignancy, with a relatively higher prevalence in young male adults and a weak dominance in the right atrium as a primary site of growth. It is characterized by rapid infiltration of cardiac structures and possible metastasis to mediastinal and distant organs. The patient may be asymptomatic until advanced phases. It therefore has a poor prognosis. Diagnosis requires the use of multimodality imaging, including echocardiography, computed tomography (CT), cardiac magnetic resonance and positron emission tomography. The definitive diagnosis is based on histological examination. We report the case of a young male adult who was referred to the cardiology department for a syncopal event 5 h after cocaine assumption. During the diagnostic work-up, a chest X-ray was performed, showing multiple pulmonary lesions, which were evaluated with a chest CT highlighting the presence of a cardiac mass in the right atrium and ventricle. For this reason, a complete cardiological evaluation was performed. The clinical and instrumental suspicion of a malignant cardiac tumor was confirmed by multimodality imaging and finally by histological examination.

PMID:39342560 | DOI:10.1714/4336.43218
Surgical planning aided with 3D technologies for management of complex paracardiac tumors

Fetched: October 16th, 2024, 2:00am UTC by Camilo E PÃ©rez-CualtÃ¡n

J Cardiothorac Surg. 2024 Sep 28;19(1):548. doi: 10.1186/s13019-024-03096-w.

ABSTRACT

BACKGROUND: Accurate diagnosis and treatment of complex cardiac tumors poses challenges, particularly when surgical resection is considered. 3D reconstruction and printing appear as a novel approach to allow heart teams for optimal surgical and post operative care.

METHODS: We report two patients with uncommon masses including a cardiac angiosarcoma (CAS) and a IgG4-related disease (IgG4-RD) with exclusive cardiac involvement. In both cases, three-dimensional (3D) reconstruction and 3D-printed models were utilized to aid the surgical team achieve optimal pre-operative planning. Both patients underwent ECG-gated cardiac computed tomography angiography (CCTA) imaging and, due to the complex anatomy of the masses, their large dimensions, proximity to vital cardiac and vascular structures, and unclear etiology, computational and 3D-printed models were created for surgical planning. An exploratory literature review of studies using 3D-printed models in surgical planning was performed.

RESULTS: In case 1 (CAS), due to the size and extension of the mass to the right ventricular free wall, surgical intervention was not considered curative and, during thoracotomy, an open biopsy confirmed the imaging suspicion of CAS which guided the initiation of optimal medical treatment with chemotherapy and, after clear tumor retraction, the patient underwent a second surgical intervention, and during the 18 months of follow-up showed no signs of recurrence. In Case 2 (IgG4-RD), the patient underwent uncomplicated total surgical resection; this allowed directed treatment and, at 12 months follow-up, there are no signs of recurrence. Computational and 3D-printed models were used to plan the surgery and to confirm the findings. Limited studies have explored the use of 3D printing in the surgical planning of tumors.

CONCLUSIONS: We present two patients with uncommon cardiac tumors, highlighting the significant value of 3D models in the anatomical characterization and assessment of their extension. These models may be essential in surgical planning for complex cardiovascular cases and could provide more information than conventional imaging modalities. Further studies are needed to demonstrate the impact of 3D technologies in studying cardiac tumors.

PMID:39342312 | PMC:PMC11438039 | DOI:10.1186/s13019-024-03096-w
Imaging features of hepatic angiosarcoma: retrospective analysis of two centers

Fetched: October 16th, 2024, 2:00am UTC by Lei Jiang

BMC Cancer. 2024 Sep 27;24(1):1191. doi: 10.1186/s12885-024-12817-2.

ABSTRACT

PURPOSE: Identifying primary hepatic angiosarcoma (PHA) preoperatively is challenging, often relying on postoperative pathology. Invasive biopsy increases bleeding risk, emphasizing the importance of early PHA diagnosis through imaging. However, comprehensive summaries of ultrasound, abdominal computed tomography (CT), magnetic resonance imaging (MRI), and whole- body positron emission tomography-CT (PET-CT) in this context are lacking. This study aimed to investigate the comprehensive imaging characteristics of PHA.

PATIENTS AND METHODS: Imaging data were collected from 7 patients diagnosed with PHA via pathology between January 2000 and December 2019 in two provincial grade III hospitals. All patients underwent routine color ultrasound examinations before surgery, with 3 patients receiving contrast-enhanced ultrasound (CEUS).CT scans, both plain and enhanced, were performed on 5 patients, and whole-body PET-CT examinations were conducted on 2 patients.

RESULTS: Among the 7 patients with PHA, 4 presented with a single solid intrahepatic mass (2 of which were large), 1 with a single exophytic macroblock type, 1 with a mixed type featuring multiple masses and nodules, and 1 with a multiple nodule type. Conventional ultrasound of PHA showed uneven echoes within the tumor, potentially accompanied by septal zone echoes, and a blood flow grade of 0-I. CEUS displayed early-stage circular high enhancement, a central non-enhancement area, and a "vascular sign" around the tumor. CT scans revealed low-density shadows in the plain scan stage, high peripheral ring enhancement, and punctate nodular enhancement in the arterial phase, with varying intensities and the presence of a "vascular sign." During the portal vein stage, the interior of the tumor was consistently unfilled and exhibited structural disorder. PET-CT showed low-density lesions in the liver and low fluorodeoxyglucose metabolism.

CONCLUSIONS: Imaging diagnosis plays a crucial role in PHA diagnosis. When liver tumor imaging matches the above characteristics, consider PHA.

PMID:39333917 | PMC:PMC11437663 | DOI:10.1186/s12885-024-12817-2
Acute respiratory distress syndrome as the initial symptom of hepatic angiosarcoma with Kasabach-Merritt syndrome: A case report

Fetched: October 16th, 2024, 2:00am UTC by Xiaoqian Cui

Medicine (Baltimore). 2024 Sep 27;103(39):e39800. doi: 10.1097/MD.0000000000039800.

ABSTRACT

RATIONALE: Hepatic angiosarcoma (HSA) has a poor prognosis. Our understanding of its clinical features, diagnosis, treatment, and prognosis remains limited. In certain cases, vascular tumors such as HSA can induce bleeding complications due to thrombocytopenia, known as Kasabach-Merritt syndrome (KMS). When KMS symptoms occur in the lungs, its clinical manifestations closely resemble those of ARDS, leading to misdiagnosis and poor outcomes. Unfortunately, this condition is extremely rare and there is a lack of relevant case reports, which further adds to the difficulty of its diagnosis and treatment.

PATIENT CONCERNS: This case report describes a patient who initially presented with symptoms of ARDS. Due to the unique nature of these symptoms, the patient underwent a complex diagnostic and treatment process before finally being diagnosed with HSA complicated by KMS through pathological examination.

DIAGNOSES: The patient was eventually diagnosed with HSA by pathology and KMS with multiorgan hemorrhage.

INTERVENTIONS: Highly misleading clinical manifestations were recorded during the diagnosis and treatment, which, to our knowledge, have not been previously reported.

OUTCOMES: The patient died from a massive pulmonary hemorrhage.

LESSONS: Dysfunction of a single organ or system may be the external manifestation of a multi-system clinical disease. Therefore, in the clinical diagnosis and treatment process, especially during early diagnosis, while it is important to focus on the primary or typical clinical symptoms, it is equally crucial not to underestimate or ignore accompanying symptoms that lack specificity. When diagnosis and treatment reach an impasse, these "atypical" symptoms often prove to be key in solving the puzzle.

PMID:39331928 | PMC:PMC11441907 | DOI:10.1097/MD.0000000000039800
Cardiac angiosarcoma with lung metastasis presented with multiple halo signs: One case report and literature review

Fetched: October 16th, 2024, 2:00am UTC by Yan Liu

Radiol Case Rep. 2024 Sep 17;19(12):5932-5935. doi: 10.1016/j.radcr.2024.09.026. eCollection 2024 Dec.

ABSTRACT

Cardiac angiosarcoma is a rare aggressive malignancy with rapid progress and poor prognosis. Here we report 1 patient with cardiac angiosarcoma with lung metastasis, which presented as multiple halo signs and ground glass opacities. The patient underwent computed tomography (CT) guided lung biopsy and postoperative tissue histology confirmed the diagnosis of angiosarcoma. Transthoracic echocardiography (TTE) and cardiac Magnetic Resonance Imaging (MRI) identified the main tumor in the right atrium. Positron emission tomography/computed tomography (PET/CT) excluded intraabdominal lesions. The patient was given chemotherapy with nab-paclitaxel, cardiac radiation therapy and remained follow-up. Considering the rapid disease progression and poor prognosis, the present case report is intended to provide diagnostic insight into cardiac angiosarcoma with lung metastasis, especially with lung CT scans of multiple halo signs and ground glass opacities.

PMID:39328938 | PMC:PMC11424893 | DOI:10.1016/j.radcr.2024.09.026
Timeline of surgery in localized angiosarcoma of the breast: Improving outcome following multidisciplinary treatment optimization

Fetched: October 16th, 2024, 2:00am UTC by Massimiliano Gennaro

Eur J Surg Oncol. 2024 Sep 17;50(12):108699. doi: 10.1016/j.ejso.2024.108699. Online ahead of print.

ABSTRACT

INTRODUCTION: Primary (PAS) and radiation-associated angiosarcomas (RAAS) of the breast are rare tumors of vascular origin with poor survival. In this retrospective cohort study, we aimed to assess the impact of multidisciplinary treatment optimization on the prognosis of patients who underwent surgery at a national referral center.

MATERIALS AND METHODS: Cases of operable angiosarcoma of the breast evaluated by a multidisciplinary team including surgeons, medical oncologists and radiation oncologists expert in the field and treated from January 2012 to January 2023 were retrieved from a prospectively maintained database. The outcomes of three treatment groups, defined by the timing of surgery in relation to adjuvant and neoadjuvant therapies, were compared.

RESULTS: Fifty-nine patients with operable angiosarcomas of the breast (49 RAAS and 10 PAS) were retrospectively identified. The five-year overall survival was 85.2 % (95 % CI 73.9-98.2) and event-free survival was significantly better in patients with grade 1 than those with grade 2 or 3 tumors. Patients receiving neoadjuvant chemotherapy had significantly better outcomes than those treated with primary surgery. Pathological complete response was significantly higher in patients receiving neoadjuvant radiotherapy after neoadjuvant chemotherapy, and a trend towards better distant-disease-free survival was found for patients with complete response at time of surgery.

CONCLUSIONS: Optimization of angiosarcoma treatment based on specialized, multidisciplinary assessment regarding the type and timing of surgery and the use of neoadjuvant chemoradiotherapy can improve outcomes. The findings of this study support the use of neoadjuvant chemotherapy as well as adjuvant and neoadjuvant radiotherapy in clinical practice.

PMID:39326303 | DOI:10.1016/j.ejso.2024.108699
Magnetic resonance imaging and clinicopathological findings of primary hepatic angiosarcoma

Fetched: October 16th, 2024, 2:00am UTC by Jingwen Zhang

Abdom Radiol (NY). 2024 Sep 21. doi: 10.1007/s00261-024-04592-2. Online ahead of print.

ABSTRACT

PURPOSE: To investigate the magnetic resonance imaging (MRI) and clinicopathological features of primary hepatic angiosarcoma (PHA) and enhance preoperative diagnosis.

METHODS: MRI and clinicopathological information of 12 cases proved PHA were reviewed. Summarize the MRI characteristics of PHA combined with literature reviews.

RESULTS: Among 12 cases (6 males and 6 females; age range, 23-70 years; mean, 53.3 years), one presented as a large mass, two as a diffuse infiltrating tumor, and nine as a mixed pattern of large masses with multiple nodules, all involving both lobes of the liver and ranging from 0.1 cm to 11 cm in diameter. A total of 63 lesions were analyzed, including 21 masses and 42 nodules. 13 masses (61.9%) demonstrated intratumoral hemorrhage. 18 masses (85.7%) demonstrated heterogeneous patchy, ring-shaped, septate, or irregular shaped enhancing foci on late arterial phase (LAP). On dynamic contrast-enhanced MRI (DCE-MRI), 14 masses (66.7%) showed a centripetal or centrifugal pattern of incomplete progressive enhancement. 6 nodules (14.3%) appeared intratumoral hemorrhage. 31 nodules (73.8%) showed no enhancing foci on LAP images and 27 nodules (64.3%) showed enhancement pattern of complete filling, either centripetal or centrifugal pattern. Moreover, 12 cases (100%) exhibited prominent vessels within or adjacent to at least one lesion.

CONCLUSION: PHA exhibits diverse appearances on MRI. Typical MRI signs include multifoci with intratumoral hemorrhage, prominent vessels within or adjacent to the foci, as well as varied degrees of progressive enhancement with incomplete filling in dominant masses of PHA.

PMID:39320496 | DOI:10.1007/s00261-024-04592-2
Effects of Lipophilic Statins on Cell Viability and Tissue Factor Expression in Canine Haemangiosarcoma Cells

Fetched: October 16th, 2024, 2:00am UTC by Kosuke Kobayashi

Vet Comp Oncol. 2024 Sep 25. doi: 10.1111/vco.13012. Online ahead of print.

ABSTRACT

Canine haemangiosarcoma (HSA) is a highly aggressive cancer often associated with coagulation abnormalities. Statins, inhibitors of 3-hydroxy-3-methyl-glutaryl-CoA reductase (HMGCR) clinically prescribed for hypercholesterolemia, are also believed to possess antitumour and anticoagulant properties by inhibiting downstream Akt activation. Akt phosphorylation is involved in the mechanism of the antitumour and tissue factor (TF)-lowering effects of statins. In the present study, we aimed to investigate whether statins could inhibit cell viability while concurrently inducing anticoagulant properties by regulating the expression of TFs in canine HSA cells. Using reverse transcription-quantitative polymerase chain reaction (RT-qPCR), we initially exclusively detected HMGCR mRNA expression in canine HSA tissues and cell lines but not in normal cephalic vein and spleen tissues. Moreover, treatment with lipophilic statins, including atorvastatin, fluvastatin, and simvastatin, inhibited cell viability in a concentration-dependent manner and decreased TF expression both at the mRNA and protein levels, as evidenced by cell viability assays, RT-qPCR, and immunoblotting, respectively. Further investigation using cell viability assays and flow cytometry revealed that simvastatin decreased Akt phosphorylation, and MK-2206, a specific Akt inhibitor, mirrored the effect of simvastatin on cell viability and cell cycle arrest. However, MK-2206 exhibited different effects on TF expression depending on the cell type, indicating that Akt phosphorylation may not consistently regulate TF expression. Overall, this study provides insights into the potential therapeutic use of statins in targeting tumour growth and coagulation abnormalities in canine HSA. Further research is warranted to fully elucidate the underlying mechanisms and clinical applications of statins in canine HSA treatment.

PMID:39319370 | DOI:10.1111/vco.13012
What is your diagnosis? Fine-needle aspirate from a neck lesion in a dog

Fetched: October 16th, 2024, 2:00am UTC by Cynthia Robveille

Vet Clin Pathol. 2024 Sep 24. doi: 10.1111/vcp.13389. Online ahead of print.

NO ABSTRACT

PMID:39317995 | DOI:10.1111/vcp.13389
Synchronous primary breast angiosarcoma with invasive ductal carcinoma

Fetched: October 16th, 2024, 2:00am UTC by Wala Ben Kridis

Breast Dis. 2024;43(1):271-274. doi: 10.3233/BD-240003.

ABSTRACT

Primary angiosarcoma (PAS) of the breast is an extremely uncommon variant of breast malignancies. Highly aggressiveness and dismal prognosis characterize this endothelial neoplasm. We report here an unusual case of PAS of the breast occurring in a 46-year-old woman associated with concurrent bilateral invasive ductal carcinoma and ovarian metastases.

PMID:39302353 | DOI:10.3233/BD-240003
Epithelioid Hemangioendothelioma With Extensive Metastasis Masquerading as Epithelioid Angiosarcoma: Answer

Fetched: October 16th, 2024, 2:00am UTC by Nada Shaker

Am J Dermatopathol. 2024 Oct 1;46(10):715-716. doi: 10.1097/DAD.0000000000002690.

NO ABSTRACT

PMID:39293668 | DOI:10.1097/DAD.0000000000002690
Bilateral pneumothorax unveiling cystic pulmonary metastases from facial angiosarcoma: A case report

Fetched: October 16th, 2024, 2:00am UTC by Anas Orgi

Radiol Case Rep. 2024 Sep 3;19(11):5471-5473. doi: 10.1016/j.radcr.2024.08.023. eCollection 2024 Nov.

ABSTRACT

Cutaneous angiosarcoma is a rare malignant vascular tumor that most often affects the scalp and face of elderly males. It is most frequently associated with lung metastases, with the appearance of thin-walled cyst. We report the case of a 78-year-old male who presented with acute chest pain and dyspnea, subsequently revealing bilateral pneumothorax caused by cystic pulmonary metastases from a neglected facial angiosarcoma. The diagnosis was confirmed through comprehensive radiological evaluations, emphasizing the critical role of CT imaging. This case underscores the critical need to not underestimate pneumothorax complicating cystic lung lesions and to consider angiosarcoma metastases, particularly in elderly men, as a potential diagnosis.

PMID:39285964 | PMC:PMC11403414 | DOI:10.1016/j.radcr.2024.08.023
Bilateral primary angiosarcoma of the breast after reduction mammoplasty: An extremely rare case and literature review

Fetched: October 16th, 2024, 2:00am UTC by Cansu SÃ¶nmez

Pathol Int. 2024 Sep 13. doi: 10.1111/pin.13483. Online ahead of print.

NO ABSTRACT

PMID:39267459 | DOI:10.1111/pin.13483
Epithelioid angiosarcoma arising from pleomorphic xanthoastrocytoma, CNS WHO grade 3

Fetched: October 16th, 2024, 2:00am UTC by Austin J Helmink

J Neuropathol Exp Neurol. 2024 Sep 13:nlae101. doi: 10.1093/jnen/nlae101. Online ahead of print.

NO ABSTRACT

PMID:39271179 | DOI:10.1093/jnen/nlae101
Pediatric Angiosarcoma with Novel Phenotypic and Genotypic Profile in Chinese Children

Fetched: October 16th, 2024, 2:00am UTC by Bo Shao

Fetal Pediatr Pathol. 2024 Sep 13:1-11. doi: 10.1080/15513815.2024.2402395. Online ahead of print.

ABSTRACT

Introduction: Angiosarcoma is an exceedingly rare entity in pediatric population. Herein, we report two pediatric angiosarcoma with novel phenotypic and genotypic profile. Methods: The two patients' information was summarized by clinical data, histopathology, immunohistochemistry, genetic, treatment, and prognosis. Results: Two Chinese children presented with abdominal mass or consumptive hypothyroidism at 2 and 6 years. A patient presented with a unique histopathology of epithelioid AS with smooth muscle hyperplasia, and carried a novel somatic mutation in FAT1 (c.3929C > T/p. Ser1310Leu) along with germ-line variants in CDK8 (c.895A > C/p. Lys299Gln), FANCI (c.3906-07inv/p. Glu1303Lys), and MST1R (c.3581-83delinsACG/p. Arg1194-Ser1195delinsHisGly). The other patient presented with a novel -clinical phenotype of consumptive hypothyroidism. They received postoperative treatment and were monitored for 20 and 26 months, showing good recovery. Conclusion: The phenotypic and genotypic spectrum of AS in pediatric population was expanded by these two patients, which requires the accumulating more cases to gain a deeper understanding.

PMID:39269732 | DOI:10.1080/15513815.2024.2402395
Recognising angiosarcoma presenting as an enlarging ecchymotic plaque

Fetched: October 16th, 2024, 2:00am UTC by Michelle Wu

BMJ Case Rep. 2024 Sep 12;17(9):e258751. doi: 10.1136/bcr-2023-258751.

ABSTRACT

Cutaneous angiosarcoma (cAS) is a rare malignant neoplasm of vascular endothelial origin with an unfavourable prognosis. Its diagnosis often faces delays due to its manifestation as an inconspicuous 'bruise-like' lesion in an otherwise asymptomatic individual, leading to a generally low index of suspicion for angiosarcoma. Here, we present a case of a man who presented to his general practitioner with an ecchymotic plaque on his forehead, initially thought to be benign. Over the subsequent 6 weeks, the lesion progressively enlarged and became ulcerated, prompting the patient to represent to his general practitioner. He was urgently referred to a dermatologist and a subsequent biopsy confirmed the diagnosis of cAS. Our presentation of this case serves as a reminder for physicians to maintain a high index of suspicion and low threshold for biopsy for patients with atraumatic ecchymotic lesions.

PMID:39266028 | DOI:10.1136/bcr-2023-258751
Primary angiosarcoma of the breast: a literature review

Fetched: October 16th, 2024, 2:00am UTC by Yidan Zhu

Chin Clin Oncol. 2024 Sep 5:cco-24-16. doi: 10.21037/cco-24-16. Online ahead of print.

ABSTRACT

BACKGROUND AND OBJECTIVE: Primary angiosarcoma of the breast (PBA) is an extremely rare and heterogeneous disease. PBA is difficult to diagnose and has a poor prognosis. In order to better understand the disease and provide evidence-based treatment for PBA patients, a review of the published literature in the English language was conducted.

METHODS: A literature review in agreement with the PRISMA protocol was conducted. Medline and Cochrane databases were searched for English articles on PBA patients in September 2023 with a predetermined strategy. The articles were categorized and assessed based on hierarchical levels of scientific evidence.

KEY CONTENT AND FINDINGS: A total of 255 articles were identified, among these 137 publications which included 1,888 patients met the criteria for inclusion in the final analysis. No prospective, randomized trials exclusive to PBA have been recognized. This article provides an overview of the most current and comprehensive evidence concerning the epidemiology, etiology, genomic features, clinical presentations, diagnosis, treatment, and prognosis of PBA.

CONCLUSIONS: Despite the fact that current evidence is largely derived from retrospective studies, database analyses, and case reports, we utilized this information to tackle important clinical questions concerning optimal patient management practices for PBA. Complete surgical excision continues to be the mainstay treatment for PBA. However, the effectiveness of adjuvant therapies is still unclear. This narrative review highlights the urgent need for more rigorously designed research to enhance the management and treatment strategies for PBA.

PMID:39260429 | DOI:10.21037/cco-24-16
Radiation-induced angiosarcoma of the breast: A case report

Fetched: October 16th, 2024, 2:00am UTC by Emin Gurleyik

Int J Surg Case Rep. 2024 Oct;123:110253. doi: 10.1016/j.ijscr.2024.110253. Epub 2024 Sep 10.

ABSTRACT

INTRODUCTION AND IMPORTANCE: Today, breast-conserving surgery (BCS) and adjuvant radiotherapy are preferred treatments for patients with early invasive breast cancer. Radiation-induced angiosarcoma (RIAS) of the breast is a rare but serious complication of radiotherapy.

CASE PRESENTATION: Seventy-one-year-old woman is presented to our department with a locally advanced dark red polypoid lesion on her left breast. She had left BCS, axillary dissection, and adjuvant radiotherapy for invasive breast cancer 8 years before presentation. A small tissue sample from the breast lesions was sent for histopathologic examination that the diagnosis was angiosarcoma of the breast. She had neoadjuvant chemotherapy. Following the completion of chemotherapy, a total mastectomy was performed as surgical treatment. The final histopathologic diagnosis was well-differentiated angiosarcoma.

CLINICAL DISCUSSION: RIAS of the breast is rare disease that develops after a several-year latency period. Locally advanced disease was initially treated with neoadjuvant chemotherapy which appears to be effective for significant disease regression. Patients who respond well to chemotherapy in vivo may have higher disease-specific survival rates. After chemotherapy-induced regression of locally advanced sarcoma, total mastectomy was performed for radical treatment.

CONCLUSION: RIAS of the breast is defined as the histological diagnosis of angiosarcoma in an irradiated region after a long latency period in a patient who has previously received radiotherapy for breast carcinoma. Based on clinical and nuclear imaging data, we may conclude that neoadjuvant chemotherapy can result in significant disease regression, and following neoadjuvant chemotherapy the treatment of angiosarcoma is completed by radical breast surgery.

PMID:39260344 | PMC:PMC11413748 | DOI:10.1016/j.ijscr.2024.110253
Malignant transformation of an intraparenchymal hemangioma in the cervical spinal cord of a German shepherd dog

Fetched: October 16th, 2024, 2:00am UTC by Courtney P Korff

J Vet Intern Med. 2024 Sep-Oct;38(5):2681-2685. doi: 10.1111/jvim.17190. Epub 2024 Sep 11.

ABSTRACT

An 8-year-old female spayed German shepherd dog was presented for evaluation of a 1-week history of right thoracic limb monoparesis. Magnetic resonance imaging (MRI) identified an intraparenchymal, T2 hypointense and T1 isointense, strongly heterogeneously contrast-enhancing mass with moderate internal susceptibility artifact on T2* images at the level of the cranial extent of the C5 vertebral body. Euthanasia was elected after a rapid neurologic decline in the 24 hours after MRI. Necropsy and histopathology identified an intraparenchymal hemangiosarcoma arising from a hemangioma in the cervical spinal cord, with no evidence of neoplastic disease in any other examined organs. The spectrum of vasoproliferative disorders in the central nervous system in veterinary species has been codified recently, but hemangiosarcoma is considered metastatic to the central nervous system. Herein we describe the clinical, imaging, and histologic findings in a dog with a novel primary location of hemangiosarcoma in the cervical spinal cord.

PMID:39258518 | PMC:PMC11423490 | DOI:10.1111/jvim.17190
Statistics of Visceral Sarcoma in Japan: Report From the Population-Based National Cancer Registry (NCR) in Japan

Fetched: October 16th, 2024, 2:00am UTC by Shudai Muramatsu

J Surg Oncol. 2024 Sep 10. doi: 10.1002/jso.27867. Online ahead of print.

ABSTRACT

BACKGROUND AND OBJECTIVES: Sarcomas developing in the visceral organs are extremely rare, with no previous reports to describe their national epidemiology. We analyzed Japanese domestic statistics for visceral sarcoma, using the National Cancer Registry (NCR) in Japan, a population-based database launched in 2016.

METHODS: We identified 3245 cases of visceral sarcomas in the NCR dated 2016-2019 to analyze demographic and disease information, initial diagnostic process, volume and type of the hospitals, treatment, and prognosis.

RESULTS: Visceral sarcoma shows a higher prevalence in the older generation (60+ years), with a significant male predominance (p = 0.006). Leiomyosarcomas occurred frequently in the gastrointestinal tract (N = 240; 39.5%), and angiosarcomas in the liver, gall bladder, pancreas, and spleen (N = 244; 43.9%). Visceral sarcomas were often treated in facilities of lower volume without specific adjuvant treatments (p < 0.001). The cumulative 3-year overall survival was 44.8%, and several factors such as surgery or absence of chemotherapy positively affected survival.

CONCLUSIONS: This is the first nationwide study in Japan to analyze the inclusive epidemiology of visceral sarcomas. Visceral sarcomas are characterized by senior and male predominance with relatively poor prognosis, often managed in nonspecialized facilities and rarely with adjuvant therapies. Several histologic subtypes had the propensity to develop in specific organs.

PMID:39257202 | DOI:10.1002/jso.27867
HEMANGIOSARCOMA IN RED WOLVES (CANIS RUFUS) AND GRAY WOLVES (CANIS LUPUS) IN HUMAN CARE: SIX CASES

Fetched: October 16th, 2024, 2:00am UTC by Jasmine Y Sarvi

J Zoo Wildl Med. 2024 Sep;55(3):819-826. doi: 10.1638/2024-0007.

ABSTRACT

Wolves are commonly housed in zoological institutions and captive breeding facilities that are essential for maintaining genetic diversity and for the recovery of declining populations. Neoplasia is a common cause of mortality in wolves, but hemangiosarcoma has not previously been described. This condition was diagnosed in four red wolves (Canis rufus) and two gray wolves (Canis lupus) housed at five different institutions between 2008 and 2018. Animals were 11-16 yr of age at the time of presentation. Clinical signs included loss of body condition, abdominal distension, lethargy, weakness, ataxia, and hyporexia. Three animals were mildly anemic. All animals were humanely euthanized within an average of 3 d from onset of clinical signs. Two animals had primary splenic tumors, two had pelvic tumors with one originating from the aorta, and one had a cranial mediastinal mass. Diagnosis was made on postmortem histologic examination in all cases. Four wolves had evidence of metastases with foci in the lungs, lymph nodes, mesentery, liver, subcutis/skeletal muscle, kidney, adrenal, and thyroid gland. Hemangiosarcoma should be considered in geriatric wolves presenting with nonspecific signs, particularly if abdominal distension, free peritoneal fluid, or anemia is present.

PMID:39255227 | DOI:10.1638/2024-0007
Outcomes following extended resection of radiation-induced angiosarcoma of the breast: a sarcoma unit experience and systematic review

Fetched: October 16th, 2024, 2:00am UTC by Eyal Mor

ANZ J Surg. 2024 Sep 10. doi: 10.1111/ans.19231. Online ahead of print.

ABSTRACT

PURPOSE: Radiation-induced angiosarcoma (RIAS) of the breast is a rare tumour with high rate of local recurrence. The aim of this study is to evaluate the outcome of radical resections.

METHODS: A retrospective analysis of all patients who underwent extended surgical resection for RIAS of the breast between 2013 and 2022. Included were patients who underwent radical resection, including complete resection of previously irradiated skin and underlying fascia of pectoralis major. Post-operative and long-term oncological outcomes were than analysed. A systematic review was performed using the MEDLINE database in the last 20 years.

RESULTS: Twenty-two (n = 22) patients met the inclusion criteria. The median length of the specimen was 220 mm (range, 120-377 mm). At a median follow-up of 33.5 months (range, 7.9-102.4), 3 (13.6%) patients had both local and metastatic lung disease and 1 (4%) patient with only lung metastasis. The estimated 3- and 5-year OS was 81.1% and 57.9%, respectively. The estimated 3- and 5-year DSS was 91.7% and 65.5%, respectively. The estimated 3- and 5-year DFS rate were both 75.2%. The systematic review identified 17 studies with a recurrence rate ranging from 33% to 100%.

CONCLUSIONS: Treatment of RIAS of the breast with an up-front locally extended approach is associated with a low rate of local recurrence compared with the reported literature.

PMID:39253994 | DOI:10.1111/ans.19231
Radiation-induced angiosarcoma-An unusual cause of recurrent pleural effusion

Fetched: October 16th, 2024, 2:00am UTC by Jenny Sw Yun

Respirol Case Rep. 2024 Sep 9;12(9):e70013. doi: 10.1002/rcr2.70013. eCollection 2024 Sep.

ABSTRACT

Although rare, radiotherapy can induce secondary malignancies, such as radiation-induced angiosarcoma (RIAS), which is associated with a poor prognosis. Early detection is crucial for improving outcomes. The modified Cahan criteria are instrumental in diagnosing RIAS, which is ultimately confirmed through histological examination. We present a case of a middle-aged woman who developed RIAS after undergoing radiotherapy post-surgery and adjuvant chemotherapy for right-sided breast cancer. The patient presented with a rapidly reaccumulating right-sided pleural effusion, and RIAS was confirmed through pleural biopsy and aspirate. This case report highlights the pathway for establishing a diagnosis of RIAS and the need for early detection through clinical examination and surveillance imaging for patients following radiotherapy.

PMID:39253318 | PMC:PMC11381914 | DOI:10.1002/rcr2.70013
Molecular Analysis of Renal/Adrenal Angiosarcomas Reveals High Frequency of Recurrent Genetic Alterations

Fetched: October 16th, 2024, 2:00am UTC by Pedram Argani

Genes Chromosomes Cancer. 2024 Sep;63(9):e23268. doi: 10.1002/gcc.23268.

ABSTRACT

Angiosarcomas of the kidney and adrenal gland are rare, highly aggressive vascular neoplasms. Their genomic profile has not been systematically studied to date. We report the clinicopathologic and molecular features of six angiosarcomas centered in the kidney/adrenal gland. All patients were male adults, ranging from 58 to 77 years of age. Tumor sizes ranged from 2.5 to 22.5 cm. Half of the cases demonstrated hot spot mutations in the KDR gene, while one-third demonstrated mutations in the PIK3CA gene; both of these gene alterations being previously described, preferentially in breast angiosarcomas. In addition, two cases each demonstrated BRIP1 gene amplification, CTNNB1 and ETV6 mutations, which have not been previously reported in angiosarcoma. Notably, molecular studies were critical in establishing the correct diagnoses in three cases: one was an epithelioid angiosarcoma originally misdiagnosed as metastatic adenocarcinoma to the adrenal gland, the second was a vasoformative angiosarcoma that mimicked hemangioma, and the third was a collision tumor between a high-grade angiosarcoma and a chromophobe renal cell carcinoma which was originally diagnosed as a sarcomatoid renal cell carcinoma. In summary, angiosarcomas of the kidney and adrenal gland have a high frequency of recurrent genetic alterations, some of them being shared with other angiosarcoma subtypes, while other appear to be novel. In particular, activating hot spot KDR and PIK3CA mutations represent potential therapeutic targets for these highly aggressive cancers.

PMID:39248552 | DOI:10.1002/gcc.23268
A Novel TEK::GAB2 Gene Fusion in Pediatric Angiosarcoma of Pelvic soft Tissue: A Case Report and Literature Review

Fetched: October 16th, 2024, 2:00am UTC by Zachary Emmanuel Sandoval

Pediatr Dev Pathol. 2024 Sep 9:10935266241279073. doi: 10.1177/10935266241279073. Online ahead of print.

ABSTRACT

Pediatric angiosarcoma of soft tissue, an extremely rare entity, remains poorly understood from a genetic standpoint. Herein, we present the case of a previously healthy 17-year-old girl with acute left hip pain. Subsequent magnetic resonance imaging revealed a 21.8 cm left pelvic sidewall mass with heterogeneous enhancement and multiple lung nodules. Biopsy of the tumor showed an infiltrative, hemorrhagic neoplasm composed primarily of atypical spindle to epithelioid cells. Focal vasoformative architecture was appreciated. Immunohistochemically, the tumor cells were strongly positive for CD31, ERG, and FLI-1, supporting the diagnosis of angiosarcoma. Genetic analysis identified a novel TEK::GAB2 gene fusion. TEK belongs to the angiopoietin receptor family, and its fusion with GAB2 is predicted to mediate tumorigenesis. This report expands the current knowledge on the spectrum of gene rearrangements of angiosarcoma.

PMID:39248342 | DOI:10.1177/10935266241279073
MRI and PET/CT of multiple primary hepatic angiosarcomas in 25-year-old man: A case report

Fetched: October 16th, 2024, 2:00am UTC by Murat Jakipov

J Clin Imaging Sci. 2024 Aug 30;14:32. doi: 10.25259/JCIS_69_2024. eCollection 2024.

ABSTRACT

This study aims to provide a comprehensive understanding of primary hepatic angiosarcoma, a rare and aggressive malignancy, focusing on its diagnostic challenges and unique imaging characteristics. The objective is to delineate the distinctive features of angiosarcoma through computed tomography and magnetic resonance imaging modalities, contributing to improved diagnostic precision and adding valuable insights to the scientific literature. We present the case of a 25-year-old male with primary hepatic angiosarcoma, emphasizing the challenges in distinguishing it from other vascular tumors.

PMID:39246734 | PMC:PMC11380813 | DOI:10.25259/JCIS_69_2024
Primary non-radiation induced angiosarcoma of the parotid with epithelioid morphology

Fetched: October 16th, 2024, 2:00am UTC by Julian Leow

Natl J Maxillofac Surg. 2024 May-Aug;15(2):313-315. doi: 10.4103/njms.njms_29_23. Epub 2024 Jul 24.

ABSTRACT

Angiosarcomas are rare, accounting for only 1-2% of sarcomas in the head and neck region. We present an extremely rare case of primary non-radiation-induced angiosarcoma of the parotid with epithelioid morphology which required a tertiary center second opinion for its histological characteristics. We discuss the presentation, imaging, diagnosis, characteristics, and pathway of this case. As parotid lumps are common, a surgical sieve is required to rule out other possible conditions. Early correct diagnosis is crucial in conditions such as angiosarcomas due to their quick metastatic potential.

PMID:39234126 | PMC:PMC11371295 | DOI:10.4103/njms.njms_29_23
Angiomatosis of the breast: a clinicopathological analysis of six cases

Fetched: October 16th, 2024, 2:00am UTC by J Cheng

Zhonghua Bing Li Xue Za Zhi. 2024 Sep 8;53(9):905-909. doi: 10.3760/cma.j.cn112151-20240227-00123.

ABSTRACT

Objective: To investigate the clinicopathological features and differential diagnosis of breast angiomatosis. Methods: Six cases of breast angiomatosis diagnosed at the Department of Pathology, the Seventh Medical Center, People's Liberation Army General Hospital and the Department of Pathology, Dongzhimen Hospital, Beijing University of Chinese Medicine from January 2011 to December 2023 were evaluated and reviewed. Results: All patients were female with an average age of 46 years at presentation, ranging from 25 to 62 years. The most common clinical presentation was a palpable unilateral breast mass with diameter ranging from 7 to 14 cm, and the average size was 11 cm. Histologically, all cases were composed of variably-sized ectatic, thin-walled blood vessels with minimal to no apparent smooth muscle, lined by flat normochromic endothelium without atypia, and diffusely infiltrating the breast stroma. Where present, the lesional vessels infiltrated between and around terminal duct lobular units but not into individual intralobular stroma. Immunohistochemical staining for CD31, CD34, Factor â…§, Fli-1 and D2-40 revealed positive expression in vascular and/or lymphatic endothelial cells. Additionally, the Ki-67 proliferation index was found to be less than 1%. Conclusions: Angiomatosis of the breast is a rare benign vascular lesion. Distinguishing it from low-grade angiosarcoma requires careful consideration of the growth pattern, atypical features, and Ki-67 proliferation index.

PMID:39231742 | DOI:10.3760/cma.j.cn112151-20240227-00123
Thoracic Angiosarcoma Arising from Chronic Tuberculous Pyothorax: A Case Report

Fetched: October 16th, 2024, 2:00am UTC by Takayuki Horii

Intern Med. 2024 Sep 4. doi: 10.2169/internalmedicine.4088-24. Online ahead of print.

ABSTRACT

Angiosarcoma is a rare malignancy that can arise from chronic pyothorax. We herein report a 75-year-old Japanese man with a history of tuberculosis who presented with left-sided chest pain that had persisted for 4 months. Chest computed tomography revealed an encapsulated left-sided pleural effusion with chest wall invasion, and histopathology confirmed angiosarcoma arising from a chronic tuberculous pyothorax. Chemotherapy with paclitaxel (80 mg/m² weekly) was ineffective and was discontinued after 3 months. Our findings emphasize that physicians should inform patients with chronic tuberculous pyothorax about malignant complications for which chest pain is the initial symptom, in addition to highlighting the need for careful follow-up.

PMID:39231669 | DOI:10.2169/internalmedicine.4088-24
Growth Rate and Outcomes in Locally Recurrent Extremity and Truncal Soft Tissue Sarcoma

Fetched: October 16th, 2024, 2:00am UTC by George Z Li

JAMA Netw Open. 2024 Sep 3;7(9):e2431530. doi: 10.1001/jamanetworkopen.2024.31530.

ABSTRACT

IMPORTANCE: Improved prognostic tools are needed for patients with locally recurrent extremity or truncal soft tissue sarcoma (STS).

OBJECTIVE: To examine the association between average local recurrence (LR) growth rate and outcomes following resection of locally recurrent extremity or truncal STS.

DESIGN, SETTING, AND PARTICIPANTS: This retrospective cohort study used a prospectively maintained database from a single high-volume tertiary sarcoma referral center in the US to identify patients 16 years of age or older who underwent repeat resection of a locally recurrent extremity or truncal STS between July 1, 1982, and December 31, 2021. Patients with atypical lipomatous tumors, desmoid tumors, dermatofibrosarcoma protuberans, angiosarcomas, and prior or synchronous distant recurrence were excluded. Data were analyzed from November 1, 2022, to June 17, 2024.

EXPOSURE: Average LR growth rate, defined as the sum of recurrent tumor maximal diameters divided by the disease-free interval after index operation.

MAIN OUTCOMES AND MEASURES: The primary outcomes were cumulative incidences of disease-specific death (DSD), with death from other causes as a competing risk, and second LR, with death from any cause as a competing risk.

RESULTS: The study cohort included 253 patients (median [IQR] age, 64 [51-73] years; 140 [55.3%] male). The 5-year cumulative incidence of DSD after repeat resection was 29%. Multivariable analysis indicated that LR growth rate (hazard ratio [HR], 1.12 [95% CI, 1.08-1.18]; P < .001), younger age (HR, 0.98 [95% CI, 0.97-0.99]; P = .002), R1 or R2 margins (HR, 1.71 [95% CI, 1.03-2.84]; P = .04), high LR grade (HR, 2.90 [95% CI, 1.17-7.20]; P = .02), and multifocality (HR, 2.92 [95% CI, 1.70-5.00]; P < .001) were independently associated with higher incidence of DSD. Using the minimum P value method, the optimal cutoff for growth rate was found to be 0.68 cm/mo. Patients with values above this cutoff had higher 5-year incidences of DSD following repeat resection (63% vs 19%; permutation test P < .001) and higher amputation rates (19% vs 7%; P = .008). Only R1 margins were independently associated with higher incidence of second LR (HR, 1.81 [95% CI, 1.19-2.78]; P = .006).

CONCLUSIONS AND RELEVANCE: In this cohort study of patients undergoing resection of a locally recurrent extremity or truncal STS, LR growth rate was independently associated with DSD. These findings suggest that patients with growth rates higher than 0.68 cm/mo who undergo LR resection may have high disease-specific mortality and amputation rates and should be considered for perioperative systemic therapy.

PMID:39230901 | PMC:PMC11375480 | DOI:10.1001/jamanetworkopen.2024.31530
Radiation-Induced Angiosarcoma in a Patient With Klippel-Trenaunay Syndrome: A Case Report

Fetched: September 4th, 2024, 2:01am UTC by Timothy Nehila

Eplasty. 2024 Aug 16;24:e46. eCollection 2024.

ABSTRACT

BACKGROUND: Klippel-Trenaunay syndrome (KTS) is a rare vascular disorder that typically presents in the lower limb with unilateral port-wine discoloration of the skin due to capillary malformations, varicose veins, and soft tissue and bone overgrowth. Radiation-induced angiosarcoma (RIAS) is a rare vascular malignancy that develops as a long-term complication secondary to radiation therapy. While there is no well-established direct relationship between KTS and development of angiosarcoma, there is literature that suggests a logical association between the 2 disorders. The present case report highlights the importance of further research into a potential connection between KTS and RIAS.

METHODS: A 51-year-old female with a history of KTS was referred to the plastic surgery clinic for delayed breast reconstruction following RIAS of the left breast. It was decided that a 2-stage left latissimus dorsi musculocutaneous flap reconstruction with silicone implant would best achieve her goal of symmetry.

RESULTS: The patient reported here had an unremarkable postoperative course. Of note, this patient experienced severe body dysmorphia after her mastectomy and RIAS excision due to the absence of her left breast juxtaposed with her prior large right breast augmentation. The choice of 2-stage combined autologous/implant reconstruction likely contributed to her satisfactory cosmetic outcome.

CONCLUSIONS: While RIAS and KTS are distinct conditions, a link may exist between the two. More research is needed to investigate this possible relationship. Aggressive treatment of RIAS is crucial for patient recovery, and a 2-stage combined autologous/implant reconstruction is an optimal choice for post-RIAS reconstruction, especially in patients hoping to achieve a large breast size.

PMID:39224409 | PMC:PMC11367161
Circulating nucleosomes as a potential cancer biomarker in dogs with splenic nodular lesions

Fetched: September 4th, 2024, 2:01am UTC by Meazzi Sara

Vet Q. 2024 Dec;44(1):1-7. doi: 10.1080/01652176.2024.2399648. Epub 2024 Sep 3.

ABSTRACT

Splenic nodular lesions in dogs can be either benign or malignant. They might be discovered incidentally or, in case of rupture, they may lead to hemoabdomen. Nevertheless, splenectomy followed by histopathology is essential for diagnosis and to prevent rupture. Yet, this invasive procedure might be postponed for dogs with benign splenic nodular lesions. Conversely, owners may opt for euthanasia over surgery for malignancies with poor prognosis like hemangiosarcoma. Thus, anticipating diagnosis with non-invasive biomarkers is crucial for proper patient management. In this prospective study, plasma samples were collected from 66 dogs with histologically confirmed splenic nodular lesions. A canine-specific ELISA kit was applied to assess nucleosome concentration, with histopathology of the spleen serving as the gold standard. Nucleosome concentration was found to be significantly higher in dogs with malignant splenic nodular lesions, particularly in those with hemangiosarcoma and other malignancies. The presence of hemoabdomen, more prevalent in dogs with splenic malignancy, also resulted in increased plasmatic nucleosome concentrations. Plasma nucleosomes could serve as a biomarker for detecting malignant splenic nodular lesions in dogs. More research is needed to understand how nucleosome concentration relate to disease stage and prognosis in dogs with hemangiosarcoma.

PMID:39224020 | PMC:PMC11373370 | DOI:10.1080/01652176.2024.2399648
Imaging features of pediatric angiosarcomas: clinical, pathologic, and radiological review

Fetched: September 3rd, 2024, 2:01am UTC by Musab Almushayqih

Pediatr Radiol. 2024 Oct;54(11):1873-1883. doi: 10.1007/s00247-024-06041-0. Epub 2024 Sep 3.

ABSTRACT

BACKGROUND: Angiosarcomas are rare malignant vascular tumors and there is scarcity of data on their imaging features.

OBJECTIVE: To review and illustrate the imaging, clinical, and pathologic features of angiosarcoma in children.

MATERIALS AND METHODS: A list of pathologically proven angiosarcoma seen between Nov 1992 and Jan 2023 was obtained from a pathology database and picture archiving and communication system. Those with pre-treatment imaging available on our PACS were included in the study. Imaging studies were reviewed by two readers in consensus.

RESULTS: A total of six children (two males and four females; median age of 8.8 years; range 2.9 years to 15.5 years) had angiosarcoma during the study period. Organ of origin included breast (n = 2), liver (n = 2), spleen (n = 1), and paranasal sinuses (n = 1). The patient with splenic angiosarcoma had Li-Fraumeni syndrome. Five patients had a single lesion while one had multifocal lesions. The tumors were large with a median diameter of 12.9 cm (range 2.7 cm to 24 cm). Most tumors were heterogeneous on T2-weighted imaging with hemorrhage and necrosis and showed heterogeneous enhancement. Three had well-defined borders and three had infiltrative borders. None of the tumors showed calcifications. Two tumors in the liver showed gradual non-centripetal progressive diffuse enhancement on dynamic imaging. One patient had metastases at presentation and four patients subsequently developed metastases on follow-up. Five patients underwent surgical resection and chemotherapy; one patient with a liver lesion underwent arterial embolization followed by liver transplant. Three patients died at the last follow-up.

CONCLUSION: The imaging features of angiosarcomas are nonspecific, but the tumors are large heterogeneously enhancing masses with hemorrhage and necrosis. Hepatic angiosarcomas may show non-centripetal progressive and heterogeneous enhancement on dynamic imaging.

PMID:39223383 | DOI:10.1007/s00247-024-06041-0
The utility of DNA methylation profiling in the diagnosis of un-, de- and trans-differentiated melanoma: a series of 11 cases

Fetched: September 3rd, 2024, 2:01am UTC by Zeynep Betul Erdem

Histopathology. 2024 Sep 2. doi: 10.1111/his.15309. Online ahead of print.

ABSTRACT

AIMS: Melanomas are recognised for their remarkable morphological plasticity. Some tumours may lose conventional features and/or acquire non-melanocytic characteristics, referred to as undifferentiated, dedifferentiated and transdifferentiated melanoma. Despite this phenotypical variability, melanomas typically maintain their cancer driver aberrations, affecting genes such as BRAF, NRAS and NF1. Currently, little is known about whether the DNA methylation profile follows the loss or change of differentiation or is retained despite extensive morphological transformation.

METHODS AND RESULTS: In this study we analysed 11 melanoma cases, comprising six males and five females, with a median age of 67 years, including five undifferentiated, four trans-differentiated and two de-differentiated melanomas. Undifferentiated and trans-differentiated tumours either arose in a patient with known melanoma and/or presented in the groin/axilla with molecular alterations consistent with melanoma. Cases with heterologous differentiation resembled chondrosarcoma, osteosarcoma, angiosarcoma and rhabdomyosarcoma both morphologically and immunohistochemically, while undifferentiated tumours resembled undifferentiated pleomorphic sarcoma. Methylome profiling was performed, and unsupervised clustering analysis revealed nine cases (five undifferentiated, three trans-differentiated and one de-differentiated) to cluster closely together with conventional melanomas from a reference set. Two cases clustered separately with a distinct group of conventional melanomas exhibiting H3K27me3 loss.

CONCLUSIONS: Despite loss of differentiation and phenotypical plasticity, methylation patterns seem to be retained in undifferentiated, de-differentiated and trans-differentiated melanomas and represent useful diagnostic tools to enhance diagnostic precision in these diagnostically challenging cases.

PMID:39223066 | DOI:10.1111/his.15309
Synchronous multiple primary malignancies or transdifferentiation?-A diagnostic challenge in a case of Xeroderma pigmentosum

Fetched: September 3rd, 2024, 2:01am UTC by Sandhyarani M Kanna

Indian J Pathol Microbiol. 2024 Sep 2. doi: 10.4103/ijpm.ijpm_324_24. Online ahead of print.

ABSTRACT

A diagnosis of synchronous tumors is always questionable when they show similar histology. We present a case of synchronous tumors, i.e. melanoma (MM) arising from the scalp and angiosarcoma (AS) from the cheek in a patient of Xeroderma pigmentosum (XP). The presence of melanin in AS of the cheek led to a differential diagnosis and a possibility of transdifferentiation of malignant melanoma in the first instance. Immunohistochemistry (IHC) was negative for melanoma markers (Melan A, SOX-10 & S 100) and positive for vascular markers (ERG, CD31 & CD34) ruling out transdifferentiation and concluding the cheek swelling as angiosarcoma. The presence of non-cutaneous malignancies is rare in XP and the presence of MM in a nearby site and the melanin pigment in the cutaneous malignancy mislead the diagnosis. The morphology of the tumor was the key which led to the further workup of the case.

PMID:39221877 | DOI:10.4103/ijpm.ijpm_324_24
Case report: Malignant epithelioid angiosarcoma in a Chinese female patient

Fetched: September 3rd, 2024, 2:01am UTC by Xiaohong Li

Front Oncol. 2024 Aug 16;14:1398656. doi: 10.3389/fonc.2024.1398656. eCollection 2024.

ABSTRACT

Perivascular epithelioid cell tumors (PEComas) are mesenchymal tumors that exhibit characteristic epithelioid or spindle cell morphology and typically grow around blood vessels. These tumors are characterized by the expression of melanocytic and smooth muscle markers, such as HMB-45, Melan-A, and smooth muscle actin, indicating a dual differentiation phenotype. PEComas are extremely rare diseases, and patients typically have a very poor prognosis. Here, we report a case of malignant cutaneous PEComa with pulmonary metastasis in a Chinese female and review relevant literature. The patient underwent surgical resection of a soft tissue tumor in the left upper arm under general anesthesia, and the subsequent pathological findings suggested a tumor with perivascular epithelioid cell differentiation (PEComa). The patient received adjuvant chemotherapy and radiotherapy after surgical resection, along with monitoring through computed tomography (CT) scans. Three months later, pulmonary metastasis was detected, but both the cutaneous PEComa in the left upper limb and the pulmonary metastatic lesions were stably controlled under active management and treatment. This is a rare case worth reporting and studying, and therefore, we conducted a long-term follow-up, and we hope to provide help for the clinical treatment of PEComa.

PMID:39220642 | PMC:PMC11361990 | DOI:10.3389/fonc.2024.1398656
Ventricular cardiac hemangiosarcoma with brain metastases in a dog

Fetched: September 3rd, 2024, 2:01am UTC by J Yan

J Vet Cardiol. 2024 Aug 16;55:32-37. doi: 10.1016/j.jvc.2024.08.004. Online ahead of print.

ABSTRACT

An 11-year-old, female, spayed, soft-coated Wheaten terrier presented for acute onset of neurological signs. On presentation, neurological examination showed right thoracic and pelvic limb proprioceptive deficits, absent right menace reflex, and weak right nasal septum response. A left thalamocortical lesion was localized. On thoracic auscultation, an arrhythmia was noted, and electrocardiography showed frequent ventricular premature complexes and rare runs of ventricular tachycardia. Echocardiography identified an interventricular septal mass extending into the lumen of the left ventricle. Thalamocortical metastasis secondary to the cardiac mass was suspected to be the cause of the patient's neurological signs. Humane euthanasia was elected by the owner due to the patients clinical status and poor prognosis. A postmortem examination diagnosed hemangiosarcoma of the interventricular septum, the right ventricular free wall, and left ventricular free wall. The left ventricle adjacent to the paraconal groove showed myocardial necrosis and inflammation. Metastases to the brain and secondary intracranial hemorrhage were found which were suspected to be the cause of the antemortem neurological signs. Concurrent pulmonary and hepatic metastases were noted. This report describes a rare presentation of an intracardiac hemangiosarcoma of the interventricular septum, right ventricle, and left ventricle in a patient presenting with neurological signs.

PMID:39217731 | DOI:10.1016/j.jvc.2024.08.004
Postpolypectomy Bleeding Gone Wrong: Primary Colonic Epithelioid Angiosarcoma

Fetched: September 1st, 2024, 2:00am UTC by Rishi Bolla

ACG Case Rep J. 2024 Aug 26;11(8):e01472. doi: 10.14309/crj.0000000000001472. eCollection 2024 Aug.

ABSTRACT

Epithelioid angiosarcoma is an aggressive form of angiosarcoma, and primary colonic tumors are extremely rare. We present the case of a 60-year-old man who presented with what initially appeared to be postpolypectomy bleeding. After undergoing repeat endoscopy, he was found to have epithelioid angiosarcoma of the transverse colon, and imaging further confirmed that it was the primary tumor site. Our patient underwent segmental resection of his transverse colon. The patient's initial presentation as postpolypectomy bleeding and his continued cancer-free survival after a relatively limited surgery are unique features not previously seen.

PMID:39206141 | PMC:PMC11350336 | DOI:10.14309/crj.0000000000001472
Cardiac Angiosarcoma in the Right Atrium Treated by Surgical Resection

Fetched: September 1st, 2024, 2:00am UTC by Milica Dragicevic-Antonic

Medicina (Kaunas). 2024 Aug 15;60(8):1321. doi: 10.3390/medicina60081321.

ABSTRACT

We present the case of a 49-year-old female of Caucasian European descent with chest tightness, fatigue, and palpitations, ultimately diagnosed with primary intracardiac angiosarcoma. Initial echocardiography revealed a significant mass within the right atrium, infiltrating the free wall. Surgical intervention included tumor excision and partial resection of the superior vena cava. Histopathological examination confirmed a high-grade angiosarcoma. Postoperative imaging identified a recurrent mass in the right atrium, suggestive of thrombus, alongside Takotsubo cardiomyopathy. Considering the elevated surgical risks and the presence of cardiomyopathy, management included anticoagulation therapy with Warfarin and adjuvant chemotherapy with Paclitaxel. Follow-up cardiac magnetic resonance imaging demonstrated a recurrent angiosarcoma with superimposed thrombus. This case presents the complex diagnostic and therapeutic landscape of angiosarcoma, highlighting the critical importance of early surgical intervention, advanced imaging techniques, and vigilant postoperative monitoring.

PMID:39202602 | PMC:PMC11356060 | DOI:10.3390/medicina60081321
Radiation-Associated Angiosarcoma of the Breast: The State of the Art of a Rare and Aggressive Disease

Fetched: September 1st, 2024, 2:00am UTC by Salvatore Cozzi

J Pers Med. 2024 Aug 14;14(8):859. doi: 10.3390/jpm14080859.

ABSTRACT

INTRODUCTION: Considering the increasing number of conservative surgeries (quadrantectomies) for primary breast carcinoma, especially in the early stages, often followed by adjuvant radiotherapy, the incidence of radiation-associated angiosarcoma (RAS) is expected to rise in the coming decades, and it will represent a clinical and therapeutic challenge, as limited data are available due to the rarity and heterogeneity of the disease. Though the prognosis of these patients is poor, a number of clinical and pathological factors can be evaluated to better understand the course of RAS. The aim of this systematic review is to explore the available clinical-pathological, therapeutic, and prognostic data regarding RAS to evaluate its occurrence, diagnosis, treatment, and outcomes.

METHOD: RAS clinical data were identified by a systematic review conducted in five different databases (Medline, Web of Knowledge, Google Scholar, Scopus, and Cochrane). Only RAS works published in English, with access to the full manuscript text, and with clear results, were considered as eligible.

RESULTS: We considered 52 papers comprising 319 RAS cases. The patient age at diagnosis ranged from 37 to 93 years, with most cases occurring from 5 to 10 years after breast irradiation. The most common clinical presentation was an aggressive development of macules, papules, or erythematous-violaceous skin discoloration at the site of previous radiation treatment for primary breast carcinoma. Complete surgical excision appeared to be the first-line treatment of the disease, in some cases followed by adjuvant local and/or systemic therapies. Despite different treatments, local recurrence rates ranged from 40% to 90%, leading to negative outcomes and poor prognosis for patients.

CONCLUSION: Although the literature is limited and the data are heterogeneous and contentious, our review aims to highlight the importance of early diagnosis, multimodal treatment, and long-term follow-up of RAS in order to limit and prevent the aggressiveness of this neoplasm.

PMID:39202050 | PMC:PMC11355165 | DOI:10.3390/jpm14080859
Clinical course and vascular endothelial growth factor signaling system expression in maxillary angiosarcoma: A case report

Fetched: August 29th, 2024, 2:01am UTC by Shiho Arai

Sci Prog. 2024 Jul-Sep;107(3):368504241274022. doi: 10.1177/00368504241274022.

ABSTRACT

Maxillary angiosarcoma, an aggressive tumor derived from vascular endothelial cells, is very rare. Recently, antivascular endothelial growth factor (VEGF) therapies have attracted considerable attention. We describe the clinical course of a patient with maxillary angiosarcoma and discuss the expression of VEGF signaling molecules assessed via immunohistological analysis. An 81-year-old man presented with an aggressive tumor in the left maxillary sinus. Biopsy revealed atypical nuclear cell proliferation, and the tumor was suspected to be a sarcoma. The maxillary malignancy was treated using a multidisciplinary approach with a combination of surgery, radiotherapy, and regional chemotherapy. Examination of the specimen obtained in the first surgery revealed maxillary angiosarcoma, found to be positive for CD31, while negative for CD34, D2-40, and factor â…§. Although no pathological residual tumor was observed after the planned wide surgery, cervical lymph node and distant metastases occurred. The patient died 24 months after the first surgery. Staining revealed VEGF receptor (VEGFR) 1, VEGFR2, phosphorylated Ak strain transforming, mitogen-activated protein kinase, and signal transducer and activator of transcription 3 positivity. Although our findings do not indicate that anti-VEGF therapy is beneficial for treating maxillary angiosarcomas, we found that VEGFR signaling pathways were activated in maxillary angiosarcomas similar to angiosarcomas originating at other sites. Herein, we report a case of maxillary angiosarcoma, focused on VEGFR and signaling pathway activation. To our knowledge, this is the first report to describe VEGFR system immunostaining findings in maxillary angiosarcoma.

PMID:39196593 | PMC:PMC11363231 | DOI:10.1177/00368504241274022
A Case of Undifferentiated Sarcoma of the Liver in an Adult Treated with Right Hepatectomy Followed by Adjuvant Chemotherapy

Fetched: August 28th, 2024, 2:00am UTC by Masaki Ikeda

Gan To Kagaku Ryoho. 2024 Jul;51(7):771-773.

ABSTRACT

Undifferentiated sarcoma of the liver is rare, especially in adults, and is an aggressive malignancy that originates from the primary mesenchymal tissues. A 53-year-old man was referred to our hospital for further evaluation of a low-grade fever. Contrast-enhanced CT revealed an 18-cm tumor in the right lobe of the liver. The tumor was characterized by low-density areas suspected of cystic components, a high-density area suspected of hemorrhage, and contrast enhancement in the thickened marginal and internal septa. MRI revealed a high-intensity tumor with a heterogeneous structure on T2-weighted images. Angiosarcoma of the liver with intratumoral hemorrhage was suspected, and right hepatectomy was performed. The pathological diagnosis was an undifferentiated sarcoma based on the presence of undifferentiated mesenchymal tumor cells with a stellate to spindle-shaped pleomorphism. Following a multidisciplinary discussion, 4 courses of the AI regimen (doxorubicin and ifosfamide)were administered as adjuvant chemotherapy, and no recurrence was confirmed at 2 years and 6 months follow-up. Our case suggests that radical resection followed by adjuvant chemotherapy may contribute to a favorable prognosis for undifferentiated sarcoma of the liver.

PMID:39191698
Primary adrenal sarcomas: A national analysis of epidemiological trends, treatment patterns, and outcomes

Fetched: August 28th, 2024, 2:00am UTC by Bibek Aryal

J Surg Oncol. 2024 Aug 27. doi: 10.1002/jso.27836. Online ahead of print.

ABSTRACT

BACKGROUND AND OBJECTIVES: Primary adrenal sarcoma (PAS) is an exceedingly rare malignancy with limited data available on its epidemiology, management, and outcomes. This study aimed to characterize the national incidence, treatment patterns, and survival of PAS utilizing a National Cancer Database.

METHODS: The National Cancer Database was queried for patients diagnosed with primary adrenal tumors from 2004 to 2019. Cases with sarcoma histology were identified as PAS. Annual incidence trends, histological distribution, treatment modalities (surgery, chemotherapy, radiation therapy), perioperative outcomes, and overall survival (OS) were analyzed.

RESULTS: Of 7213 primary adrenal tumor cases, 332 (4.6%) were PAS. The most common histological subtypes were leiomyosarcoma (37.3%), hemangiosarcoma (27.1%), and sarcoma not otherwise specified (6.0%). Most cases (71.7%) presented as locoregional disease. Treatment included surgery alone (47.8%), surgery plus chemotherapy and/or radiation (27.1%), chemotherapy/radiation alone (13.3%), or no treatment (13.9%). For surgical cases, the median length of stay was 5 days, the 30-day readmission rate was 3.36%, and the 30/90-day mortality rates were 3.65% and 9.90%, respectively. The 5-year OS rate for surgery alone was 43%, with a median OS of 34.6 months. For surgery with radiation/chemotherapy, the 5-year OS rate was 37.3%, with a median OS of 35.4 months.

CONCLUSIONS: This largest analysis of PAS to date demonstrates that most cases present as locoregional disease amenable to surgical resection, with favorable outcomes. The role of adjuvant therapy remains unclear, as no significant survival difference was observed between surgery alone and multimodal treatment.

PMID:39190497 | DOI:10.1002/jso.27836
An uncommon case of postradiation atypical vascular lesions extending beyond the radiation field in a breast cancer patient

Fetched: August 28th, 2024, 2:00am UTC by Ying Sun

JAAD Case Rep. 2024 Jul 6;51:69-71. doi: 10.1016/j.jdcr.2024.06.021. eCollection 2024 Sep.

NO ABSTRACT

PMID:39188333 | PMC:PMC11347040 | DOI:10.1016/j.jdcr.2024.06.021
Novel case of anastomosing hemangioma of the extradural space: illustrative case

Fetched: August 27th, 2024, 2:01am UTC by Shohei Fujita

J Neurosurg Case Lessons. 2024 Aug 26;8(9):CASE24265. doi: 10.3171/CASE24265. Print 2024 Aug 26.

ABSTRACT

BACKGROUND: Anastomosing hemangiomas are benign vascular neoplasms occurring mainly in the genitourinary tract and paraspinal soft tissues. There have been no reported cases of anastomosing hemangiomas occurring in the spinal epidural space; therefore, their clinical and radiological presentations remain unclear.

OBSERVATIONS: A 55-year-old man presented with progressive back pain and motor and sensory disturbances in both lower extremities. Magnetic resonance imaging (MRI) revealed an extradural lesion at the T4-6 level that extended into the extraspinal region through the left T5-6 intervertebral foramen. Axial MRI revealed that the tumor encircled the spinal cord, with sharp horn-like ends in the intraspinal canal. The patient underwent complete tumor resection with T4-6 laminectomy and left T5-6 foraminotomy. The histopathological diagnosis was an anastomo-sing hemangioma exhibiting anastomosing proliferation of capillary vessels with hobnailed endothelial cells. His symptoms improved, and tumor control was achieved 4 months after surgery without adjuvant therapy.

LESSONS: Although rare, anastomosing hemangiomas can occur in the spinal epidural space. Radiologically, the tumors show horn-like projections encircling the spinal cord, which can be diagnostic cues. Anastomosing hemangiomas should be included in the differential diagnosis of spinal epidural tumors, especially when characteristic horn-like projections are present. [https:]

PMID:39186826 | DOI:10.3171/CASE24265
MR Imaging of Typical Ovarian Hemangioma: A Case Report

Fetched: August 27th, 2024, 2:01am UTC by Xiaoli Yu

Curr Med Imaging. 2024;20:e15734056293540. doi: 10.2174/0115734056293540240325042225.

ABSTRACT

BACKGROUND: Ovarian hemangioma is an extremely rare tumor with atypical clinical manifestations, often discovered incidentally during autopsy or surgery. Approximately 60 cases have been reported in the past, but no more than 10 cases have been investigated by MRI and ultrasound (US).

CASE PRESENTATION: ln this paper, we reported a 51-year-old female patient with Ovarian Hemangioma who had no symptoms of abdominal pain, abnormal vaginal bleeding or discharge, or any other discomfort. Laboratory tests revealed an elevated serum carbohydrate antigen (CA125) of 48.99U/ml (reference range: 0-35U/ml). Multiparametric 3.0T magnetic resonance imaging (MRI) showed a cystic solid mass with a clear boundary and regular shape in the left ovarian area and minimal ascites in the abdominal cavity. The histological examination of the mass confirmed an ovarian hemangioma.

CONCLUSION: The MRI findings of ovarian hemangiomas are highly similar to those observed in hepatic hemangiomas, emphasizing the distinctive radiological characteristics specific to this condition in the ovary. This paper presents an overview of the typical MRI findings associated with ovarian hemangioma, which holds great importance for accurate diagnosis and effective treatment.

PMID:39185656 | DOI:10.2174/0115734056293540240325042225
Mohs Micrographic Surgery of Uncommon Tumors (Angiosarcoma, Eccrine, Paget, and Merkel Cell)

Fetched: August 22nd, 2024, 2:00am UTC by Tatiana V. Camayo

2024 Jul 13. In: StatPearls [Internet]. Treasure Island (FL): StatPearls Publishing; 2024 Janâ€“.

ABSTRACT

Mohs micrographic surgery (MMS) is a surgical technique commonly used to treat cutaneous malignancies such as squamous cell carcinoma (SCC) and basal cell carcinoma (BCC). MMS is performed in several stages, progressively removing malignant tissue while preserving healthy tissue and achieving high cure rates. The main advantage of MMS is the ability to assess all margins through the removal of tumor layers, which are processed in frozen sections. This histological assessment of the entire margin is crucial to determine tumor clearance before defect reconstruction, usually occurring in a single surgical session.

MMS is currently the mainstay treatment for non-melanoma skin cancer, including SCC and BCC. MMS has shown benefits in treating these cancers, significantly reducing recurrence rates in both tumors. Uncommon cutaneous malignancies (UCMs) are skin tumors that, although infrequent, are usually associated with poor outcomes and worse prognosis compared to their more common counterparts. MMS has proven useful in treating UCMs, such as dermatofibrosarcoma protuberans. Recently, several studies have highlighted different UCMs treated with MMS, including angiosarcoma, eccrine malignant tumors, Paget disease, and Merkel cell carcinoma (MCC).

Due to their high metastatic potential, MMS's role in treating UCMs has been questioned, especially in angiosarcoma and MCC. Usually, UCMs benefit from treatment with wide local excisions (WLEs). However, MMS has been used alternative option for UCMs over the past decade, supported by several case reports and series demonstrating its beneficial effects on UCM management. In recent years, study groups worldwide have assessed the efficacy, impact on prognosis, and relevance of MMS beyond its common indications. Nonetheless, the value of MMS for some UCMs remains to be fully defined. The following discussion reviews the available evidence on the role of MMS in treating specific UCMs.

PMID:39163450 | Bookshelf:NBK606085
Cytokeratin AE1/AE3 immunolabeling in epithelioid hemangiosarcoma

Fetched: August 22nd, 2024, 2:00am UTC by Daniel Felipe Barrantes Murillo

Vet Pathol. 2024 Aug 20:3009858241273268. doi: 10.1177/03009858241273268. Online ahead of print.

ABSTRACT

Epithelioid hemangiosarcoma (EH), a rare histological variant of hemangiosarcoma, is reported in various animal species, including humans, dogs, cows, horses, and cats. Epithelioid hemangiosarcomas are composed of highly pleomorphic epithelioid cells arranged in cords, islands, nests, or solid cellular areas, similar to epithelial neoplasms. Moreover, in humans, approximately 50% of EHs have cytoplasmic immunolabeling for cytokeratin AE1/AE3 (CK AE1/AE3), making it challenging to distinguish them from carcinomas. This retrospective study assessed the CK AE1/AE3 immunolabeling in canine EH cases from 5 veterinary institutions. Immunohistochemistry for CD31 and CK AE1/AE3 was performed on 30 cases. CK AE1/AE3 immunolabeling was detected in 43% (13/30) of cases, with cytoplasmic labeling ranging from 5% to 100% of neoplastic cells. All tumors consistently had membranous immunolabeling for CD31. The CK AE1/AE3 immunolabeling pattern in canine EHs closely resembled those documented in humans, indicating a similar diagnostic challenge. Therefore, it is recommended to include a vascular immunohistochemistry marker, such as CD31, whenever EH is suspected, particularly in small incisional cutaneous and subcutaneous biopsies.

PMID:39165004 | DOI:10.1177/03009858241273268
Angiosarcoma: an unexpected cause of fever of unknown origin

Fetched: August 21st, 2024, 2:00am UTC by Piotr Å GoÅºdzik

Pol Arch Intern Med. 2024 Aug 19:16828. doi: 10.20452/pamw.16828. Online ahead of print.

NO ABSTRACT

PMID:39162712 | DOI:10.20452/pamw.16828
Ex vivo MRI and histological comparison of the canine adrenal glands

Fetched: August 21st, 2024, 2:00am UTC by Olga AmorÃ³s CarafÃ

Vet Radiol Ultrasound. 2024 Aug 19. doi: 10.1111/vru.13425. Online ahead of print.

ABSTRACT

Cross-sectional imaging is widely used to characterize adrenal gland tumors in humans. In small animal veterinary medicine, while some studies have attempted to distinguish between types of adrenal gland neoplasia using CT, peer-reviewed studies investigating canine adrenal glands on MRI are scant. This prospective, pilot, single-center, method comparison, cadaveric study aimed to assess the agreement between ex vivo MRI findings and analogous histopathological findings of the adrenal glands in dogs. The adrenal glands of randomly selected dogs presented for necropsy were examined by MRI (n = 31). Additionally, five adrenal masses in dogs who underwent invasive adrenalectomy (including three adrenocortical carcinomas, one pheochromocytoma, and one adenoma) were imaged. Subsequently, gross pathology and histopathology of all the specimens were performed and correlated with the imaging findings. Adrenal gland lesions were identified on MRI with a sensitivity of 24%, a specificity of 100%, a positive predictive value of 100%, a negative predictive value of 31%, and an accuracy of 45%. The present study provides MRI features of multiple adrenal gland lesions that had never been described in dogs, including cortical hyperplasia, nodular fibrosis, hemorrhage, or multiple tumors, such as adenoma, carcinoma, and hemangiosarcoma. While MRI identified numerous adrenal gland lesions, a significant portion of those went undetected. Therefore, the absence of adrenal gland lesions on MRI does not exclude the possibility of histological lesions being present.

PMID:39160654 | DOI:10.1111/vru.13425
Rapidly progressive cystic lung disease in a patient with a scalp lesion

Fetched: August 20th, 2024, 2:01am UTC by Uddalak Majumdar

BMJ Case Rep. 2024 Aug 19;17(8):e258333. doi: 10.1136/bcr-2023-258333.

ABSTRACT

We describe an elderly patient presenting with pneumothorax, cystic lung disease and a scalp lesion. The pneumothorax resolved after placing a chest tube and suction but recurred within a week. Progression of cystic features was also seen, and biopsies of the lung and scalp lesions were performed. Immunohistochemistry was positive for markers of endothelial cells (CD31 and ERG) and negative for markers expected to be positive in alveolar cells (keratin AE1/AE3 and TTF-1), supporting the diagnosis of metastatic angiosarcoma. Palliative chemotherapy did not prevent progression and the patient expired soon after. In describing the clinico-radiological correlation of metastatic angiosarcoma, we also briefly describe the approach to cystic lung disease. Understanding the pathophysiology of cyst formation in metastatic angiosarcoma may help clinicians to better appreciate and manage the full spectrum of cystic lung disease, especially with atypical features.

PMID:39159987 | DOI:10.1136/bcr-2023-258333
Penile hemangiosarcoma as a cause of stranguria in a dog: clinical presentation, imaging findings, treatment and outcome

Fetched: August 20th, 2024, 2:01am UTC by A Pierini

Iran J Vet Res. 2024;25(1):74-78. doi: 10.22099/IJVR.2024.48685.7113.

ABSTRACT

BACKGROUND: Penile tumors are rare in dogs and only single case reports or small case series have been reported.

CASE DESCRIPTION: An 11-year-old, cross-breed dog was presented for a two-week history of stranguria. At physical examination, a subcutaneous swelling of the penis was detected. Abdominal radiographs, ultrasonography, and CT showed a subcutaneous penile mass involving the penile urethra and bulbus glandis associated with marked lysis of the os penis. Histological features along with the neoplastic cell positivity to CD31 and FVIII immunohistochemical markers warranted a final diagnosis of penile hemangiosarcoma.

FINDINGS/TREATMENT AND OUTCOME: The dog was treated with amputation of the penis, scrotal urethrostomy, and five adjuvant doses of doxorubicin along with thalidomide. Cutaneous and omental metastases were found 235 days after surgery. The dog was euthanized at 296 days due to bone and pulmonary metastasis.

CONCLUSION: Penile hemangiosarcoma seems to share the same aggressive behavior with other hemangiosarcomas seen in other anatomical locations. Therefore, surgery and chemotherapy may improve survival time in dogs with penile hemangiosarcoma as well.

PMID:39156797 | PMC:PMC11327644 | DOI:10.22099/IJVR.2024.48685.7113
Case report: Germline POT1 mutation in a patient with GIST and lung adenocarcinoma

Fetched: August 20th, 2024, 2:01am UTC by Stefania Martino

Front Oncol. 2024 Aug 2;14:1419739. doi: 10.3389/fonc.2024.1419739. eCollection 2024.

ABSTRACT

The gene protection of telomere 1 (POT1) is involved in telomere maintenance and stability and plays a crucial role in the preservation of genomic stability. POT1 is considered a high-penetrance melanoma susceptibility gene; however, the number of cancer types associated with the pathogenic germline variants of POT1 is gradually increasing, including chronic lymphocytic leukemia (CLL), angiosarcomas, and gliomas, even though many associations are still elusive. Here, we reported a case of a 60-year-old man who showed early-onset multiple neoplasms, including multiple melanomas, gastrointestinal stromal tumor (GIST), and lung adenocarcinoma. Next-generation sequencing (NGS) analyses revealed a germline heterozygous pathogenic variant in the POT1 gene. Notably, GIST and lung adenocarcinoma were not previously reported in association with the POT1 germline variant. Lung cancer susceptibility syndrome is very rare and the actual knowledge is limited to a few genes although major genetic factors are unidentified. Recently, genome-wide association studies (GWAS) have pointed out an association between POT1 variants and lung cancer. This case report highlights the clinical relevance of POT1 alterations, particularly their potential involvement in lung cancer. It also suggests that POT1 testing may be warranted in patients with familial cancer syndrome, particularly those with a history of melanoma and other solid tumors.

PMID:39156708 | PMC:PMC11327130 | DOI:10.3389/fonc.2024.1419739
Cutaneous angiosarcoma presenting as erythematous plaques and diffuse facial swelling in an elderly man

Fetched: August 18th, 2024, 2:00am UTC by Apoorva Sharma

Indian J Dermatol Venereol Leprol. 2024 Aug 1:1-3. doi: 10.25259/IJDVL_439_2024. Online ahead of print.

NO ABSTRACT

PMID:39152808 | DOI:10.25259/IJDVL_439_2024
Evaluation of PRAME immunohistochemistry in cutaneous vascular neoplasms reveals frequent expression in primary and post-irradiation cutaneous angiosarcomas

Fetched: August 18th, 2024, 2:00am UTC by Andrea Krajisnik

J Cutan Pathol. 2024 Aug 17. doi: 10.1111/cup.14707. Online ahead of print.

ABSTRACT

BACKGROUND: Preferentially expressed antigen in melanoma (PRAME) has been extensively studied in cutaneous melanocytic tumors and has proven valuable as a diagnostic adjunct in routine dermatopathology practice. However, its expression in cutaneous vascular neoplasms, particularly angiosarcomas (AS), remains largely unexplored.

METHODS: To further explore PRAME expression in cutaneous AS, 18 cases of post-irradiation and 13 cases of primary cutaneous AS were evaluated for PRAME. For comparison, sections from 11 deep soft tissue/visceral AS, 10 Kaposi sarcomas, 8 microvenular hemangiomas, 7 infantile hemangiomas, 8 atypical vascular lesions, 6 epithelioid hemangioendotheliomas, 6 pyogenic granulomas, 6 papillary endothelial hyperplasias, 6 epithelioid hemangiomas, 3 capillovenous malformations, 3 hobnail hemangiomas, 2 spindle cell hemangiomas, 2 pseudomyogenic hemangioendotheliomas, and 2 composite hemangioendotheliomas were also retrieved.

RESULTS: Overall, 22 of 31 (70.9%; 12 post-irradiation and 10 primary) cutaneous AS were positive for PRAME. In contrast, only 1 of 11 (9.1%) deep soft tissue/visceral AS showed diffuse and strong PRAME nuclear staining. All other tumor types were negative for PRAME, except for 5 of 7 (71.4%) infantile hemangiomas, which demonstrated rare (<5%; four cases) and 1+ (5-25%; one case) nuclear staining.

CONCLUSIONS: In this study, we have demonstrated frequent nuclear PRAME expression in cutaneous AS. PRAME immunohistochemistry may serve as a valuable additional marker in selected clinical settings.

PMID:39152799 | DOI:10.1111/cup.14707
Evaluating preoperative coagulation panels in dogs undergoing liver lobectomy for primary liver tumors: A multi-institutional retrospective study

Fetched: August 17th, 2024, 2:00am UTC by Samuel J Burkhardt

Vet Surg. 2024 Aug 16. doi: 10.1111/vsu.14155. Online ahead of print.

ABSTRACT

BACKGROUND: The objectives of this study were to: (i) Determine whether operable primary liver tumors were associated with prolongations in prothrombin time (PT) and activated partial thromboplastin time (aPTT) and (ii) determine if these secondary hemostatic abnormalities were more prevalent with specific liver tumors.

STUDY DESIGN: Multi-institutional retrospective study.

ANIMAL POPULATION: Dogs (n = 359) undergoing liver lobectomy for a primary liver tumor with a preoperative coagulation panel.

METHODS: Data was identified via electronic medical record review at eight veterinary teaching hospitals. Baseline dog characteristics, coagulation panel values, platelet count, emergency versus non-emergency procedure, whether the dogs received transfusion(s) of a blood product, liver lobe removed, and histopathological diagnosis were extracted from the medical record. Chi-square analysis was used to compare categorical variables between groups. Continuous variables were assessed for normality using the Shapiro-Wilk test.

RESULTS: A total of 74 of 359 dogs (20.6%) had a prolongation in either PT or aPTT preoperatively. A total of 20 of 359 dogs (5.6%) were found to have prolongation of both PT and aPTT. Hemangiosarcoma was the only histopathological diagnosis associated with concurrent prolongations of both PT and aPTT (p < .001) in 6/16 (37.5%) dogs.

CONCLUSION: Coagulation panels including PT and aPTT are unlikely to detect substantial deficiencies in secondary hemostasis in most dogs with primary liver tumors except in dogs with a histopathological diagnosis of hemangiosarcoma.

CLINICAL SIGNIFICANCE: PT and aPTT testing is low yield as an elective preoperative screening test in dogs with primary liver tumors except in dogs where there is a hemoabdomen or high suspicion for hepatic hemangiosarcoma.

PMID:39149870 | DOI:10.1111/vsu.14155
Right atrial dumbbell-like angiosarcoma in an advanced age patient

Fetched: August 16th, 2024, 2:00am UTC by Xiao-Jun Xie

Acta Cardiol. 2024 Aug 15:1-2. doi: 10.1080/00015385.2024.2391138. Online ahead of print.

NO ABSTRACT

PMID:39145571 | DOI:10.1080/00015385.2024.2391138
Hepatic Sinusoidal Disorders

Fetched: August 16th, 2024, 2:00am UTC by Sudhakar K Venkatesh

Radiographics. 2024 Sep;44(9):e240006. doi: 10.1148/rg.240006.

ABSTRACT

Hepatic sinusoids are highly specialized microcirculatory conduits within the hepatic lobules that facilitate liver functions. The sinusoids can be affected by various disorders, including sinusoidal dilatation, sinusoidal obstruction syndrome (SOS), sinusoidal cellular infiltration, perisinusoidal infiltration, and endothelial neoplasms, such as hemangioendothelioma and angiosarcoma. While these disorders, particularly SOS and neoplasms, can be life threatening, their clinical manifestation is often nonspecific. Patients may present with right upper quadrant pain, jaundice, hepatomegaly, ascites, splenomegaly, and unexplained weight gain, although the exact manifestation depends on the cause, severity, and duration of the disease. Ultimately, invasive tests may be necessary to establish the diagnosis. A comprehensive understanding of imaging manifestations of various sinusoidal disorders contributes to early diagnosis and can help radiologists detect subclinical disease. Additionally, specific imaging features may assist in identifying the cause of the disorder, leading to a more focused and quicker workup. For example, a mosaic pattern of enhancement of the liver parenchyma is suggestive of sinusoidal dilatation; peripheral and patchy reticular hypointensity of the liver parenchyma on hepatobiliary MR images is characteristic of SOS; and associated diffuse multiple hyperintensities on diffusion-weighted images may be specific for malignant sinusoidal cellular infiltration. The authors provide an overview of the pathogenesis, clinical features, and imaging appearances of various hepatic sinusoidal disorders, with a special emphasis on SOS. ^Â©RSNA, 2024 Supplemental material is available for this article.

PMID:39146204 | DOI:10.1148/rg.240006
Angiosarcoma in the femoral bone: A case report of a rare bone tumor

Fetched: August 15th, 2024, 2:00am UTC by Yuni Artha Prabowo Putro

Int J Surg Case Rep. 2024 Sep;122:110124. doi: 10.1016/j.ijscr.2024.110124. Epub 2024 Aug 13.

ABSTRACT

INTRODUCTION AND IMPORTANCE: Angiosarcoma is a high-grade endothelial sarcoma rarely found in the bones. Challenges lie in its rareness, high mortality, metastasis, and recurrence. The role of radiotherapy and chemotherapy in managing angiosarcoma remains debatable, while surgery stands out as the optimal treatment for local control. We present a patient with angiosarcoma of the bone, managed through a combination of surgery and radiotherapy.

CASE PRESENTATION: A 66-year-old male presented with pain on the right distal femur and was unable to weight bearing after lifting a box. The X-ray showed a fracture and lytic lesion. The patient has lost 5 kg of body weight in the last 3 months. The Clinicopathological Conference (CPC) suspects a malignancy process, prompting us for wide resection, biopsy, and ORIF with cement augmentation. Histopathology and immunohistochemistry results confirmed the diagnosis of angiosarcoma. We decide to manage patients with radiotherapy without chemotherapy and regular monthly monitoring. The patient exhibited clinical improvement.

DISCUSSION: Angiosarcoma of the bone tends to have a bad prognosis. Diagnosis might be challenging as it is a rare condition and could be easily confused with another disease. The routine CPC we held was beneficial for the diagnosis and treatment of the patient. The combination of surgery and radiotherapy is yielding positive outcomes.

CONCLUSION: Angiosarcoma should be considered in patients with osteolytic lesions in the bone. Surgery and radiotherapy are important to local control in patients with angiosarcoma.

PMID:39142183 | DOI:10.1016/j.ijscr.2024.110124
Primary cardiac angiosarcoma: an extremely rare cause of chest discomfort

Fetched: August 15th, 2024, 2:00am UTC by Torcuato Garrido-Arroquia Jurado

Eur Heart J Cardiovasc Imaging. 2024 Aug 14:jeae206. doi: 10.1093/ehjci/jeae206. Online ahead of print.

NO ABSTRACT

PMID:39138850 | DOI:10.1093/ehjci/jeae206
Comparison of clinical and MRI features of hepatic angiosarcoma and epithelioid hemangioendothelioma

Fetched: August 14th, 2024, 2:00am UTC by Hae Young Kim

Abdom Radiol (NY). 2024 Aug 13. doi: 10.1007/s00261-024-04513-3. Online ahead of print.

ABSTRACT

OBJECTIVE: Differentiating hepatic epithelioid hemangioendothelioma (EHE) and angiosarcoma (AS), the two most common vascular tumors in the liver, is important due to disparities in their prognosis and treatment. We aimed to compare clinical and MRI features of the two tumors.

METHODS: This retrospective study included patients with pathologically-confirmed AS or EHE who underwent MRI using gadoxetate disodium between 2008 and 2023. Two radiologists independently reviewed MR images. Wilcoxon rank sum and Fisher's exact tests were used to compare clinical and imaging features. Overall survival was compared using restricted mean survival time at 3 years.

RESULTS: 32 patients with AS (18 women [56.3%]; median age, 68 years) and 38 with EHE (24 women [63.2%]; 51 years) were included. Patients with AS were generally older (81.3% â‰¥ 60 years; P < 0.001), had more frequent laboratory abnormalities (P â‰¤ 0.018), and poorer overall survival (11.2 vs. 31.8 months; P < 0.001) than those with EHE. On MRI, a large dominant mass accompanied by smaller nodules (14/32, 43.8%), often with ill-defined margins (15/32, 46.9%) was prevalent in AS; compared with nodules of similar sizes (24/38, 63.2%; P = 0.015) with well-defined margin (30/38, 78.9%; P = 0.002) in EHE. Cirrhotic appearance of the liver was more frequent in AS (62.5%, P < 0.001), along with decreased parenchymal enhancement on hepatobiliary phase (31.3%, P < 0.001) and ascites (37.5%, P = 0.010). AS frequently presented with avid enhancement of bizarrely-shaped foci, with a centrifugal enhancement pattern. In comparison, targetoid appearance was characteristic of EHE (78.9% on T2-weighted, 54.1% on diffusion-weighted, 65.8% on multiphase images) (P â‰¤ 0.002), with enhancement degree typically lower than that of the aorta. On hepatobiliary phase, all the AS exhibited hypointensity, while 39.5% of EHE showed targetoid appearance (P < 0.001).

CONCLUSION: Patients aged â‰¥ 60 years presenting with laboratory abnormalities, typically with a large dominant mass accompanied by smaller nodules, exhibiting avid, bizarre, and centrifugal enhancement-particularly in the cirrhotic-appearing liver-suggests the likelihood of AS over EHE.

PMID:39136718 | DOI:10.1007/s00261-024-04513-3
Analysis of VEGFR-2 and PDGFR-beta expression in canine splenic hemangiosarcoma to identify drug repositioning candidates

Fetched: August 13th, 2024, 2:00am UTC by Igor SimÃµes Tiagua Vicente

Braz J Vet Med. 2024 Aug 6;46:e001524. doi: 10.29374/2527-2179.bjvm001524. eCollection 2024.

ABSTRACT

Splenic tumors are very common in dogs, and canine hemangiosarcoma (HSA) is one of the most important malignant splenic tumors. Surgery followed by chemotherapy (anthracycline-based protocols) is recommended for treating canine HSA; however, patients still do not achieve long-term survival. Therefore, this research aimed to assess vascular endothelial growth factor receptor-2 (VEGFR-2) and platelet-derived growth factor receptor-Î² (PDGFR-Î²) gene expression in formalin-fixed tissues, evaluate the quality of mRNA for quantitative polymerase chain reaction (qPCR) analysis and identify drug repositioning candidates based on VEGFR-2 and PDGFR-Î². qPCR analysis identified the relative expression of heterogeneous VEGFR-2 and PDGFR-Î², with samples showing no transcripts or very low expression and those with higher relative quantification for both genes. We then used immunohistochemistry to correlate the relative quantification of VEGFR-2 and PDGFR-Î² transcripts with respective higher protein expression to validate our results. In the next step, we evaluated drug repositioning candidates and identified small molecule inhibitors (i.e. sorafenib) and natural compounds (curcumin and resveratrol) with the ability to block VEGFR-2 and PDGFR-Î² genes. Overall, our results indicated that VEGFR-2 and PDGFR-Î² expression is highly variable among canine HSA samples and different drugs can block the expression of both genes. Therefore, a personalized approach could be useful for selecting anti-VEGFR-2 and PDGFR-Î² therapies and both genes are potential candidates for future oncological panels.

PMID:39131208 | PMC:PMC11315467 | DOI:10.29374/2527-2179.bjvm001524
Rapid Development of Primary Right Atrial Angiosarcoma

Fetched: August 13th, 2024, 2:00am UTC by Sophie Talbot

Cureus. 2024 Jul 10;16(7):e64273. doi: 10.7759/cureus.64273. eCollection 2024 Jul.

ABSTRACT

Cardiac angiosarcomas are rare and generally followed by a high level of metastasis with poor median survival outcomes. Echocardiograms, CT scans, and MRIs are the standard methods for finding sites of cardiac tumors; however, immunohistochemical confirmation is necessary for a definitive diagnosis of angiosarcoma. A 58-year-old male presented to the emergency room with one week of dyspnea on moderate exertion accompanied by chest pain and alleviated with rest. A workup done to evaluate mass found a single 5 x 3.5 x 4.8 cm mass heavily vascularized by the right coronary artery and left circumflex involving the free wall of the right atrium with no extension to the tricuspid valve. Surgical resection was performed, and immunohistochemistry was consistent with a primary cardiac angiosarcoma. An exudative fluid analysis on pericardial and pleural fluid analysis may warrant screening for malignancy more frequently in concurrence with a patient's history and presentation. Although the time from onset of symptoms to diagnosis of cardiac angiosarcoma is not well established, further investigation of such correlation may offer insight into survival post-treatment.

PMID:39131000 | PMC:PMC11315593 | DOI:10.7759/cureus.64273
Guselkumab - In Psoriasis and Beyond

Fetched: August 11th, 2024, 2:00am UTC by Aditya Kumar Bubna

Dermatol Pract Concept. 2024 Jul 1;14(3):e2024181. doi: 10.5826/dpc.1403a181.

ABSTRACT

INTRODUCTION: Guselkumab is an interleukin 23p19 inhibitor, and the first in this group, to be approved by the US Food and Drug Administration for the management of moderate to severe psoriasis. Apart from its utility in psoriasis, there are a number of other dermatologic conditions where guselkumab has demonstrated value.

OBJECTIVES: The aim of this narrative review is to describe the utility of guselkumab in psoriasis as well as its implication in off-label dermatologic disorders.

METHODS: Pubmed, Google Scholar, Scopus and ResearchGate were searched for scholarly articles related to guselkumab and its utility in dermatology using the search terms "Guselkumab" AND "Psoriasis" AND "other dermatological disorders".

RESULTS: Guselkumab is a valuable biologic agent for the management of psoriasis and psoriatic arthropathy. It has also been used successfully for other dermatologic disorders like hidradenitis suppurativa, lichen planus, pityriasis rubra pilaris and pyoderma gangrenosum. Recently, its utility in Stewart-Treves angiosarcoma (STA) has been exemplified.

CONCLUSION: Guselkumab usage is not limited to psoriasis. Its benefit extends to many more dermatologic conditions. Its utility in STA could open an avenue for its application in the field of oncology. Furthermore, it has an acceptable safety profile.

PMID:39122539 | PMC:PMC11314551 | DOI:10.5826/dpc.1403a181
Assessing spatial distribution of bioindicator elements in various cutaneous tumors using correlative imaging with laser-ablation-based analytical methods

Fetched: August 11th, 2024, 2:00am UTC by KateÅ™ina Kiss

Talanta. 2024 Nov 1;279:126651. doi: 10.1016/j.talanta.2024.126651. Epub 2024 Aug 5.

ABSTRACT

Correlative imaging of cutaneous tumors provides additional information to the standard histopathologic examination. However, the joint progress in the establishment of analytical techniques, such as Laser-Induced Breakdown Spectroscopy (LIBS) and Laser Ablation Inductively Coupled Plasma Mass Spectrometry (LA-ICP-MS) in clinical practice is still limited. Their combination provides complementary information as it is also shown in our study in terms of major biotic (Ca, Mg, and P) and trace (Cu and Zn) elements. To elucidate changes in the elemental composition in tumors, we have compiled a set of malignant tumors (Squamous Cell Carcinoma, Basal Cell Carcinoma, Malignant Melanoma, and Epithelioid Angiosarcoma), one benign tumor (Pigmented Nevus) and one healthy-skin sample. The data processing was based on a methodological pipeline involving binary image registration and affine transformation. Thus, our paper brings a feasibility study of a practical methodological concept that enables us to compare LIBS and LA-ICP-MS results despite the mutual spatial distortion of original elemental images. Moreover, we also show that LIBS could be a sufficient pre-screening method even for a larger number of samples according to the speed and reproducibility of the analyses. Whereas LA-ICP-MS could serve as a ground truth and reference technique for preselected samples.

PMID:39121552 | DOI:10.1016/j.talanta.2024.126651
Surgical treatment and outcome of primary rib tumours in cats: eight cases (2016-2023)

Fetched: August 8th, 2024, 2:00am UTC by F Cinti

J Small Anim Pract. 2024 Aug 7. doi: 10.1111/jsap.13770. Online ahead of print.

ABSTRACT

OBJECTIVES: To describe the clinical features and oncologic outcome for cats with primary rib tumours.

MATERIALS AND METHODS: Medical records for cats with surgically treated primary rib tumours from six veterinary referral centres were reviewed. Signalment, preoperative clinical signs, reconstruction technique, and surgical and oncologic outcome were retrieved from medical records or by telephone interview with owners and/or referring veterinarians.

RESULTS: Of the eight cats with primary rib tumours, three had hemangiosarcoma, two had osteosarcoma and one cat each had chondrosarcoma, osteochondroma and osteoma. The size of the primary rib mass ranged from 2 Ã— 2 Ã— 1.6 cm to 9 Ã— 7 Ã— 7.5 cm. Three minor and one major complication developed during the immediate post-operative period. Surgery consisted of thoracic wall resection in all cats. All animals survived the procedure and the median time to discharge was 3 days. The survival time for benign tumours was 150 (case 5) and 466 (case 4) days, while for malignant tumours ranged from 105 to 550 days (cases 1 to 3, cases 6 to 8).

CLINICAL SIGNIFICANCE: Hemangiosarcoma and osteosarcoma were the most represented primary rib tumours in this cohort of cats. Wide surgical excision and adjuvant chemotherapy is recommended for cats with hemangiosarcoma and osteosarcoma, but the prognosis remains guarded. Prognosis appears to be fair for the other tumour types.

PMID:39113158 | DOI:10.1111/jsap.13770
Ultrasonographic liver nodules are more often benign lesions in dogs with hemoperitoneum secondary to splenic tumor rupture

Fetched: August 8th, 2024, 2:00am UTC by Alba R Ramirez

J Am Vet Med Assoc. 2024 Aug 7:1-5. doi: 10.2460/javma.24.04.0254. Online ahead of print.

ABSTRACT

OBJECTIVE: To evaluate the reliability of preoperative abdominal ultrasonography as a staging tool for dogs with hemoperitoneum due to presumed splenic tumor rupture, focusing on the detection of metastatic lesions in the liver.

ANIMALS: 99 dogs from 20 emergency and specialty hospitals across the US.

METHODS: Dogs with nontraumatic hemoperitoneum secondary to splenic tumor rupture were included. A post hoc analysis was conducted on data from a nationwide prospective trial investigating novel treatments for canine hemangiosarcoma. The accuracy of preoperative staging was assessed by comparing ultrasonographic findings with intraoperative observations and histologic findings.

RESULTS: On preoperative ultrasonography, there was a 20% incidence of liver lesions identified, with no association to liver lesions seen during operation. Notably, 22% of liver lesions observed during operation were missed on preoperative ultrasonography. The presence of liver lesions on preoperative ultrasonography was associated with a higher likelihood of a benign splenic tumor diagnosis. There was no association between the identification of liver lesions on preoperative ultrasonography and the presence of metastatic disease on liver biopsy, with a sensitivity and specificity of 19% and 82%, respectively. Additionally, ultrasound had low sensitivity in detecting intra-abdominal lesions beyond the liver and spleen, with 82% of these lesions missed preoperatively.

CLINICAL RELEVANCE: This study challenges conventional perceptions around the approach to staging in dogs with hemoperitoneum. These findings advocate for a reevaluation of the staging approach, with more comprehensive modalities like whole-body CT or MRI potentially being more warranted.

PMID:39111340 | DOI:10.2460/javma.24.04.0254
Association of IL-8 and CXCR2 with AST/ALT Ratio in Liver Abnormalities Screening during Oxidative Stress Injury Caused by VCM

Fetched: August 8th, 2024, 2:00am UTC by Yiwen Dong

J Toxicol. 2024 Jul 30;2024:1951046. doi: 10.1155/2024/1951046. eCollection 2024.

ABSTRACT

Liver impairment caused by VCM has been linked to irreversible damage such as fibrosis, necrosis, hepatocellular carcinoma, and liver angiosarcoma. However, the ability to detect abnormalities during initial phase have not been achieved so far. Thus, this study aimed to investigate the effect of interleukin 8 (IL-8) and C-X-C chemokines 2 (CXCR2) on screening for a VCM-exposed group (n = 227) from a PVC manufacturing factory compared to a control group (n = 110) in Tianjin City in 2020 with influence factors evaluation. Ambient concentrations of VCM and health archives from 2012 to 2018 were collected for establishing the dose-effect trend. A cross-sectional survey in 2020 was performed to measure TDGA, IL-8, CXCR2, 8-OHdG, SOD, GPX, CAT, MDA, and ROS levels. Results indicated a continuous increased incidence on liver abnormalities despite a fluctuated downward trend in cumulative time-weighted average (CTWA) VCM concentrations over the years. ALT, AST, and AST/ALT ratio all contributed to liver abnormalities that contained fatty liver, liver calcification, and liver cysts, IL-8 and CXCR2 correlated with each other strongly and showed significant associations with oxidative stress markers, even AST/ALT ratio. IL-8 (>1547 Âµg/m³) or CXCR2 (<139 Âµg/m³) influenced the AST/ALT ratio through reciprocal interactions under oxidative stress injury, CXCR2 (>222 Âµg/m³), working years of 21 to 30 (a) and 11 to 20 (a), TDGA (>1.52 mg/L), alcohol consumption, smoking habit, and a less sleeping duration of <4 h per day would also be potential factors affecting the AST/ALT ratio. In conclusion (1) even with decreased VCM concentrations in PVC manufacturing factories liver abnormalities that contained fatty liver, liver calcification, and liver cysts could still occur due to oxidative stress injury with involvement of IL-8 and CXCR2. The status of protective measure and appropriate mask types also play a role; (2) the AST/ALT ratio could be a specific indicator for detecting abnormalities when combined with liver B ultrasonography results before impairment altered from bad to worse; and (3) factors such as definite medication history, fully broken protective facilities, alcohol consumption, less sleeping duration, inappropriate mask types, and longer working years could also influence AST/ALT ratio alterations through complex interactions.

PMID:39108287 | PMC:PMC11303046 | DOI:10.1155/2024/1951046
Primary Pulmonary Angiosarcoma Found Incidentally in a Complicated Patient: A Rare Case Report

Fetched: August 8th, 2024, 2:00am UTC by Reza Basiri

Clin Respir J. 2024 Aug;18(8):e13818. doi: 10.1111/crj.13818.

ABSTRACT

INTRODUCTION: Primary pulmonary angiosarcoma (PPA) is a highly aggressive and rare malignancy originating from the endothelial cells of blood vessels in the lungs. PPA is an extremely rare subtype, with less than 30 cases reported to date. PPA is not only challenging to diagnose but also has a poor prognosis, often resulting in a high mortality rate within a year of diagnosis, regardless of the treatment approach.

METHOD: We present the case of a 33-year-old woman with no significant past medical history who presented with abdominal pain and was incidentally found to have a right hilar mass with pleural effusion and empyema. After undergoing surgery for a perforated gastric ulcer, her pulmonary lesions were further worked up. Despite an extensive diagnostic evaluation, including imaging, bronchoscopy, and thoracotomy, establishing a diagnosis was challenging. Ultimately, PPA was diagnosed on surgical lung biopsy, and the patient was started on pazopanib and paclitaxel chemotherapy but expired after 1 month due to multiple complications.

CONCLUSION: This case highlights the difficulty in diagnosing this rare tumor and its poor prognosis regardless of therapy. Greater awareness of PPA and more research are needed to improve early detection and treatment options for this deadly disease.

PMID:39107956 | PMC:PMC11303451 | DOI:10.1111/crj.13818
Role of ultrasound-guided vacuum-assisted breast biopsy in the management of radiologic-pathologic discordance: a retrospective single-centre study

Fetched: August 6th, 2024, 2:00am UTC by Giulia Vatteroni

Radiol Med. 2024 Aug 5. doi: 10.1007/s11547-024-01864-1. Online ahead of print.

ABSTRACT

PURPOSE: To evaluate the efficacy of US-guided vacuum-assisted biopsy (US-VAB) in radiologic-pathologic (rad-path) discordance in women with suspicious breast lesions.

METHODS: Two thousand three hundred and sixty patients with 2385 BI-RADS category 4 and 5 lesions underwent percutaneous US-guided CNB. Thirty-six lesions were classified as discordant benign and underwent second-line US-VAB. A 14-gauge needle was utilized for CNB and 10-gauge for US-VAB. Final pathology was the reference standard for women who underwent surgery, imaging follow-up in other cases. Rates of malignancy for US-VAB and subsequent surgery were evaluated. Lesions with upgrade and no upgrade to second-line VAB were compared in terms of patient's age, lesion type and characteristics, size and BI-RADS category. Positive predictive value (PPV), negative predictive value (NPV) for BI-RADS categories and diagnostic performance for second-line US-VAB were calculated. p value < 0.05 was considered statistically significant (t-test, Mann-Whitney, Ï‡²).

RESULTS: US-VAB identified 10 B2, 9 B3 and 17 B5 lesions with upgrade to malignancy of 47.2% (17/36). There were 8 invasive no special type, 7 ductal in situ, 1 invasive lobular carcinoma and 1 angiosarcoma, and their distribution among BI-RADS categories was: 2/2 in BI-RADS 5 (100%), 12/18 in BI-RADS 4C (67%) and 3/16 in BI-RADS 4B lesions (19%) (p = 0.006). Of the remaining 19 lesions, 6 underwent surgery and 2 were upgraded to ductal carcinoma in situ; 13 underwent radiological follow-up and one resulted malignant. False-negative rate for US-VAB was 15.8% (3/19) with final upgrade to malignancy of 55% (20/36). The univariate analysis revealed mass shape (p = 0.008) and BI-RADS categories (p = 0.006) to be associated with upgrade to malignancy. Sensitivity, specificity, PPV, NPV and accuracy for US-VAB were 85, 100, 100, 84 and 92%, respectively.

CONCLUSIONS: US-VAB identified almost 50% of cancers missed by CNB, avoiding surgical biopsies and validating as an effective mini-invasive approach in rad-path discordance.

PMID:39102107 | DOI:10.1007/s11547-024-01864-1
Cardiac Mass in a 78-Year-Old Patient With a History of Cancer: Diagnostic and Treatment Challenges

Fetched: August 3rd, 2024, 2:00am UTC by Bihong Zhao

Tex Heart Inst J. 2024 Aug 2;51(2):e238299. doi: 10.14503/THIJ-23-8299.

ABSTRACT

Primary cardiac angiosarcoma is a rare, aggressive malignancy that commonly metastasizes to various organs. The presenting symptoms are typically nonspecific, so a comprehensive examination is required to confirm the diagnosis promptly. This case report describes the presentation of an older patient with a history of neoplasms. Echocardiography and biopsy were performed, but despite surgical intervention to resect a large right atrial mass, the patient died. A final diagnosis of primary angiosarcoma was made based on the resected specimen.

PMID:39093814 | PMC:PMC11298980 | DOI:10.14503/THIJ-23-8299
Primary breast angiosarcoma: A case report

Fetched: August 3rd, 2024, 2:00am UTC by Yan Wang

Medicine (Baltimore). 2024 Aug 2;103(31):e39186. doi: 10.1097/MD.0000000000039186.

ABSTRACT

RATIONALE: Primary breast angiosarcoma is a rare tumor, accounting for only 0.05% of all malignant breast tumors. The primary breast angiosarcoma typically presents with nonspecific clinical manifestations, which can easily lead to misdiagnosis. Potential factors contributing to misdiagnosis include skin changes that may be erroneously attributed to breast trauma-induced bruising and breast swelling that may be mistaken for inflammatory diseases or other benign tumors.

PATIENT CONCERNS: A 19-year-old female was admitted to the hospital due to repeated lump formation in the left breast for 9 months after left breast trauma.

DIAGNOSES: The diagnosis of primary breast angiosarcoma was confirmed on hematoma biopsy.

INTERVENTIONS: Due to the patient's condition, no special treatment was given postoperatively. After then, there was a recurrence in the chest wall, and the patient received 2 cycles of chemotherapy, resulting in a reduction in the size and lightening of the recurrent chest wall mass. When chemotherapy intolerance happened, the patient chose to discontinue treatment.

OUTCOMES: After an 18-month follow-up, the recurrent chest wall mass increased and the patient died from bleeding.

LESSONS: Primary breast angiosarcoma has a low incidence but high malignancy, with a high recurrence and metastasis rate, leading to a poor prognosis. The adjuvant chemotherapy, radiotherapy, targeted therapy, and other treatments should be considered to reduce the local recurrence rate and prolong patient survival.

PMID:39093752 | PMC:PMC11296414 | DOI:10.1097/MD.0000000000039186
Primary cardiac angiosarcoma presenting as restrictive cardiomyopathy

Fetched: August 2nd, 2024, 2:00am UTC by Joshua M Peterson

Lancet Oncol. 2024 Aug;25(8):e396. doi: 10.1016/S1470-2045(24)00325-5.

NO ABSTRACT

PMID:39089314 | DOI:10.1016/S1470-2045(24)00325-5
Incidence risk of hepatobiliary malignant neoplasms in the cohort of workers chronically exposed to ionizing radiation

Fetched: July 31st, 2024, 2:00am UTC by Galina Zhuntova

Sci Rep. 2024 Jul 30;14(1):17561. doi: 10.1038/s41598-024-63503-z.

ABSTRACT

The increased risk of liver malignancies was found in workers of the first Russian nuclear production facility, Mayak Production Association, who had been chronically exposed to gamma rays externally and to alpha particles internally due to plutonium inhalation. In the present study, we updated the radiogenic risk estimates of the hepatobiliary malignancies using the extended follow-up period (1948-2018) of the Mayak worker cohort and the improved Â«Mayak worker dosimetry system-2013Â». The cohort comprised 22,377 workers hired at the Mayak PA between 1948 and 1982. The analysis considered 62 liver malignancies (32 hepatocellular carcinomas, 13 intrahepatic cholangiocarcinomas, 16 angiosarcomas, and 1 anaplastic cancer) and 33 gallbladder adenocarcinomas. The analysis proved the positive significant association of the liver malignancy risk (the total of histological types, hepatocellular carcinoma) with the liver absorbed alpha dose from internal exposure. The excess relative risk per Gy (95% confidence interval) of alpha dose (the linear model) was 7.56 (3.44; 17.63) for the total of histological types and 3.85 (0.95; 13.30) for hepatocellular carcinoma. Indications of non-linearity were observed in the dose-response for internal exposure to alpha radiation. No impact of external gamma-ray exposure on the liver malignancy incidence was found. In the study cohort, the number of angiosarcomas among various types of liver malignancies was very high (25.8%), and most of these tumors (73.3%) were registered in individuals internally exposed to alpha radiation at doses ranging between 6.0 and 21.0 Gy. No association with chronic occupational radiation exposure was observed for the incidence of gallbladder malignancies.

PMID:39079951 | PMC:PMC11289462 | DOI:10.1038/s41598-024-63503-z
Rare infiltrative primary hepatic angiosarcoma: A case report and review of literature

Fetched: July 30th, 2024, 2:00am UTC by Xiao-Jing Lin

World J Gastrointest Oncol. 2024 Jul 15;16(7):3341-3349. doi: 10.4251/wjgo.v16.i7.3341.

ABSTRACT

BACKGROUND: The most primary sites of angiosarcoma are the skin, breast gland, and soft tissues. Primary hepatic angiosarcoma (PHA) is a rare malignant tumor of mesothelial tissue originating from the liver. PHA often presents with multiple intrahepatic foci or metastasis at the time of presentation due to its nonspecific clinical presentation and highly aggressive nature. There are no established or effective treatment guidelines for PHA, so early detection and early treatment are of great value for patient survival. Unfortunately, there is a paucity of literature on the imaging features of PHA, making the diagnosis and treatment of this disease a considerable challenge.

CASE SUMMARY: In this case report, we present a 59-year-old man who initially presented with abdominal pain and radiating pain in the right shoulder. Magnetic resonance imaging and positron emission tomography-computed tomography revealed multiple intrahepatic nodules that needed to be differentiated from tumors of vascular epithelial origin and tumors with progressive enhancement features, and signs of tumor metastasis were assessed. The patient was then subjected to contrast-enhanced ultrasonography (CEUS) to further clarify the extent of tumor infiltration and the state of microcirculatory perfusion. The manifestations observed on CEUS were similar to the classical characteristic presentation of hepatocellular carcinoma, called "quick wash-in and quick wash-out". In addition, CEUS showed that the lesion exhibited gradual infiltration and growth along the liver pedicle structures with no invading blood vessels. Finally, based on pathological and immunohistochemical tests and the above imaging manifestations, it was confirmed that the patient had infiltrating PHA, which is a rare pathological type of PHA. The patient underwent transcatheter arterial chemoembolization and chemotherapy. Four months after the onset of symptoms, the follow-up radiological examination revealed poor treatment efficacy and rapid deterioration.

CONCLUSION: This case report complements the imaging modalities of a rare infiltrative PHA, in which CEUS and quantitative analysis are found to offer substantial advantages in characterizing the microcirculatory perfusion of the lesion, providing clinicians with diagnostic information at the earliest opportunity to make a diagnosis and develop a treatment strategy to prolong the patient survival.

PMID:39072148 | PMC:PMC11271798 | DOI:10.4251/wjgo.v16.i7.3341
Better 90 Minutes Late than Never: Differential Diagnosis on MRI Scanning in a Case of Hepatic Angiosarcoma

Fetched: July 28th, 2024, 2:00am UTC by Teodora Anca Albu

Life (Basel). 2024 Jun 28;14(7):823. doi: 10.3390/life14070823.

ABSTRACT

Primary hepatic angiosarcoma (PHA) is a rare liver malignancy with few studies describing its radiological characteristics. This article aims to assess the imaging features of each of the multiple delayed contrast-enhanced magnetic resonance imaging (MRI) scans, in addition to the conventional MRI protocol, in a patient with PHA. Standard MRI sequences and a liver protocol were used in the examination of a 71 year-old male with pathologically proven PHA after current imaging evaluation. In addition, the patient underwent transversal and coronal MRI T1-weighted scans at 10 min, 20 min and 90 min after intravenous (IV) administration of gadobenatedimeglumine (Gd-BOPTA). The PHA revealed a variable appearance on MRI, with classic imaging being insufficient in making a reliable diagnosis. Lesions have increased vascularity, which translates into increased IV contrast uptake in the MRI arterial phase, showing progressive and globular enhancement in the portal and parenchymatous phases. On delayed scans, at 10 min after IV administration, the lesions maintained no washout, but slightly began to washout at 20 min post-contrast. However, in the hepatobiliary phase (90 min post-contrast injection), on an MRI T1-weighted sequence, PHA lesions were hypointense, suggesting the absence of hepatocytes, thus indicating high-grade malignancy. This approach proved the conclusion that in a patient with PHA, an extra MRI T1-weighted scan at 90 min post-gadobenatedimeglumine injection can provide helpful information in differential diagnosis.

PMID:39063577 | PMC:PMC11278181 | DOI:10.3390/life14070823
Angiosarcoma of the Maxillary Sinus: A Case Report

Fetched: July 27th, 2024, 2:00am UTC by Gowtham Narasimhan

Cureus. 2024 Jun 25;16(6):e63131. doi: 10.7759/cureus.63131. eCollection 2024 Jun.

ABSTRACT

Angiosarcoma can be defined as a malignant neoplasm arising from the lining of the blood and lymphatic vessels, including the endothelial cells. It can occur in any body part, such as blood vessels, skin, liver, and breast. Its incidence varies based on the site. There are different underlying etiologies associated with the incidence of angiosarcoma. Clinical presentation depends on the site of origin. Angiosarcoma of the sinus or nasal openings can be observed as a tissue mass, lesions, obstructed nasal cavity, facial swelling, proptosis, anosmia, nasal discharge, and epistaxis. These are rare malignancies with very low incidence. Though it has been reported in all age groups, it is more common in adults in their sixth decade and more. Nasal angiosarcoma can be a diagnostic challenge due to its rarity. This is a case of a 56-year-old female with a major complaint of nasal obstruction and face swelling for two months. Physical examination revealed a pinkish polypoidal mass. A contrast-enhanced computed tomography scan showed a heterogeneously arterial enhancing soft-tissue lesion in the left maxillary sinus with significant erosive changes. Histopathological analysis revealed a malignant spindle cell tumor, which was confirmed by a CD34 immunohistology stain. The patient was advised surgical excision for further management, which was denied. The patient is undergoing radiation therapy and is on third cycle as per the last follow-up.

PMID:39055444 | PMC:PMC11272144 | DOI:10.7759/cureus.63131
TRPS1 Expression Is Frequently Seen in a Subset of Cutaneous Mesenchymal Neoplasms and Tumors of Uncertain Differentiation: A Potential Diagnostic Pitfall

Fetched: July 27th, 2024, 2:00am UTC by Moon Joo Kim

Dermatopathology (Basel). 2024 Jul 15;11(3):200-208. doi: 10.3390/dermatopathology11030021.

ABSTRACT

Although extensively studied in cutaneous epithelial neoplasms, the TRPS1 immunoreactivity in cutaneous mesenchymal neoplasms and tumors of uncertain differentiation (CMNTUDs), such as atypical fibroxanthoma (AFX), remains largely unexplored. We assessed TRPS1 immunoreactivity in 135 CMNTUDs, comprising 46 fibrohistiocytic/fibroblastic tumors, 28 vascular tumors, 24 peripheral nerve sheath tumors (PNSTs), 21 tumors of uncertain differentiation, and 16 smooth muscle tumors. Additionally, we included selected cases of melanoma with spindled cell morphology or desmoplastic features (n = 9) and sarcomatoid squamous cell carcinoma (SSCC) (n = 5) to compare TRPS1 expression patterns with those of AFX. TRPS1 expression was prevalent in dermatofibromas (24/24), leiomyomas (8/8), AFXs/pleomorphic dermal sarcoma (PDS) (20/21), dermatofibrosarcomas protuberans (14/22), and leiomyosarcomas (6/8). It was uncommon in angiosarcomas (3/20), Kaposi sarcomas (2/8), and neurofibromas (5/17) and absent in perineuriomas (0/2). AFXs/PDS exhibited the highest median H-score of 240, contrasting with minimal TRPS1 immunoreactivity in vascular neoplasms and PNSTs, with median H-scores consistently below 10. Significant differences in H-score were observed between AFXs/PDS and angiosarcomas (p < 0.001), melanomas (p < 0.001), and leiomyosarcomas (p = 0.029). However, no significant difference was found compared to SSCCs, suggesting limited discriminatory power of TRPS1 in this context. This study sheds light on TRPS1 expression patterns in a subset of CMNTUDs, extending beyond prior studies primarily focused on epithelial tumors, while underscoring potential pitfalls associated with TRPS1 immunohistochemistry.

PMID:39051323 | PMC:PMC11270280 | DOI:10.3390/dermatopathology11030021
Regression of cardiac angiosarcoma in a 17-year-old: a percutaneous biopsy effect

Fetched: July 24th, 2024, 2:01am UTC by Noor Sharrack

Cardiooncology. 2024 Jul 23;10(1):45. doi: 10.1186/s40959-024-00239-9.

ABSTRACT

BACKGROUND: Cardiac angiosarcoma is a very rare and aggressive primary cardiac tumor associated with poor prognosis. Diagnosis is often delayed due to non-specific symptoms, with most cases involving metastases at the time of diagnosis. We describe a unique case of apparent tumor regression of cardiac angiosarcoma post percutaneous biopsy.

CASE PRESENTATION: A young male was admitted with suspected pericarditis. Echocardiogram revealed a pericardial mass. Cardiovascular magnetic resonance (CMR) suggested primary cardiac malignancy. Percutaneous biopsy was inconclusive, with subsequent CMR demonstrating apparent tumor regression. Interval imaging revealed further tumor growth, and surgical biopsy revealed primary cardiac angiosarcoma (PCAS). Causes of tumor regression following percutaneous biopsy are discussed.

CONCLUSIONS: Cases of suspected primary cardiac malignancy require careful follow up with serial multimodality imaging. Percutaneous biopsy effects should be considered in cases of tumor regression, and serial imaging should be planned afterwards.

PMID:39044266 | PMC:PMC11264432 | DOI:10.1186/s40959-024-00239-9
Translational history and hope of immunotherapy of canine tumors

Fetched: July 24th, 2024, 2:01am UTC by Jeffrey N Bryan

Clin Cancer Res. 2024 Jul 23. doi: 10.1158/1078-0432.CCR-23-2266. Online ahead of print.

ABSTRACT

Companion dogs have served an important role in cancer immunotherapy research. Sharing similar environments and diets with humans, dogs naturally develop many of the same cancers. These shared exposures, coupled with dogs' diverse genetic makeup, makes them ideal subjects for studying cancer therapies. Tumors like osteosarcoma (cOSA), hemangiosarcoma (cHSA), soft-tissue sarcoma (cSTS), and non-Hodgkin lymphoma (cnHL) occur with greater frequency than their counterpart disease in humans. Canine brain tumors allow study of therapy strategies with imaging, surgery, and radiotherapy equipment in veterinary patients with near-human geometry. Non-specific immunostimulants, autologous and allogeneic vaccines, immune checkpoint inhibitors, and cellular therapies used treating canine cancers have been tested in veterinary clinical trials. These treatments have not only improved outcomes for dogs but have also provided valuable insights for human cancer treatment. Advancements in radiation technology and the development of tools to characterize canine immune responses have further facilitated the ability to translate veterinary clinical trial results to human applications. Advancements in immunotherapy of canine tumors have directly supported translation to human clinical trials leading to approved therapies for cancer patients around the world. The study of immunotherapy in dogs has been and will continue to be a promising avenue for advancing human cancer treatment.

PMID:39042399 | DOI:10.1158/1078-0432.CCR-23-2266
An Update on Cutaneous Angiosarcoma Diagnosis and Treatment

Fetched: July 24th, 2024, 2:01am UTC by Elizabeth Richards

Cutis. 2024 May;113(5):218-223. doi: 10.12788/cutis.1008.

ABSTRACT

Cutaneous angiosarcoma (CAS) is a rare aggressive malignancy that most commonly manifests in White men older than 60 years and often appears as an enlarging ecchymosis on the head, neck, or scalp. Surgery with negative margins is the first-line treatment. The role of Mohs micrographic surgery (MMS) is uncertain but can be used in smaller, well-circumscribed lesions on the head and neck. The greatest impact that dermatologists can have in the management of CAS is through a thorough total-body skin examination and heightened awareness resulting in a shortened time to diagnosis. Until quality evidence allows for the creation of consensus guidelines, multidisciplinary care at a cancer center that specializes in rare difficult-to-treat tumors is essential in optimizing patient outcomes.

PMID:39042137 | DOI:10.12788/cutis.1008
A commentary on 'Electrochemotherapy for advanced cutaneous angiosarcoma: A European register-based cohort study from the International Network for Sharing Practices of electrochemotherapy (InspECT)'

Fetched: July 24th, 2024, 2:01am UTC by Jinghui Sun

Int J Surg. 2024 Jul 1;110(7):4405-4406. doi: 10.1097/JS9.0000000000001039.

NO ABSTRACT

PMID:39042088 | PMC:PMC11254185 | DOI:10.1097/JS9.0000000000001039
Radiation-associated breast angiosarcoma after strut-adjusted volume implant brachytherapy

Fetched: July 24th, 2024, 2:01am UTC by Chidi T Nwachukwu

Radiol Case Rep. 2024 Jun 28;19(9):3888-3894. doi: 10.1016/j.radcr.2024.05.081. eCollection 2024 Sep.

ABSTRACT

Angiosarcoma is a rare malignancy that may classically occur in the post-treatment breast. Radiation and post-treatment edema have been identified in the literature as causative risk factors. Modern treatment innovations have provided patients with more targeted radiation therapy and more conservative surgical options, which may individually limit exposure to these risk factors. Advanced treatment options are also able to provide superior cosmetic outcomes that can positively impact patient quality of life. Despite the ability for modern treatment options to mitigate post-treatment morbidities, there is still long-term risk to the patient of developing treatment-related pathologies, such as breast angiosarcoma. Here we present a patient who underwent lumpectomy and received targeted brachytherapy through a strut-adjusted volume implant device to her lumpectomy site. Her initial post-treatment course was mildly complicated by localized breast lymphedema, which resolved and left the patient with favorable cosmetic results. She developed treatment-associated breast angiosarcoma after initial breast conservation therapy was completed approximately 6 and a half years prior. Her presenting physical exam and imaging findings are portrayed with a comprehensive discussion of the commonly described presenting clinical features and imaging findings of breast angiosarcoma. Factors related to radiation treatment planning and use of the strut-adjusted volume implant device are also discussed. Comparisons between primary and secondary breast angiosarcoma are made, and a review of treatment options is given.

PMID:39040825 | PMC:PMC11261268 | DOI:10.1016/j.radcr.2024.05.081
Molecular and immune pathobiology of human angiosarcoma

Fetched: July 22nd, 2024, 2:01am UTC by Ryan Mao Heng Lim

Biochim Biophys Acta Rev Cancer. 2024 Jul 18;1879(5):189159. doi: 10.1016/j.bbcan.2024.189159. Online ahead of print.

ABSTRACT

Angiosarcoma is a rare endothelial-derived malignancy that is extremely diverse in anatomy, aetiology, molecular and immune characteristics. While novel therapeutic approaches incorporating targeted agents and immunotherapy have yielded significant improvements in patient outcomes across several cancers, their impact on angiosarcoma remains modest. Contributed by its heterogeneous nature, there is currently a lack of novel drug targets in this disease entity and no reliable biomarkers that predict response to conventional treatment. This review aims to examine the molecular and immune landscape of angiosarcoma in association with its aetiology, anatomical sites, prognosis and therapeutic options. We summarise current efforts to characterise angiosarcoma subtypes based on molecular and immune profiling. Finally, we highlight promising technologies such as single-cell spatial "omics" that may further our understanding of angiosarcoma and propose strategies that can be similarly applied for the study of other rare cancers.

PMID:39032539 | DOI:10.1016/j.bbcan.2024.189159

Trial - Angiosarcoma

Agnostic Therapy in Rare Solid Tumors

Posted: October 14th, 2024, 11:00pm UTC

Conditions: Urachal Cancer; Parathyroid Carcinoma; Fibrolamellar Carcinoma; Angiosarcoma; Secretory Carcinoma of Breast; Anal Neoplasms; Metaplastic Breast Carcinoma; Translocation Renal Cell Carcinoma; Carcinosarcoma; Small Intestine Neoplasms; Cholangiocarcinoma; Sertoli-Leydig Cell Tumor; Adenoid Cystic Carcinoma; Mesothelioma; Neuroblastoma; Adrenal Gland Neoplasms; Penile Neoplasms; Apocrine Carcinoma; Fibrosarcoma; Cancer of Unknown Primary; Hemangioblastoma; Thyroid Neoplasms; Hepatoblastoma; Fallopian Tube Neoplasms; Leiomyosarcoma; Vaginal Neoplasms; Neurofibrosarcoma; Gallbladder Neoplasms; Osteosarcoma; Biliary Tract Neoplasms; Clear Cell Endometrial Cancer; Yolk Sac Tumor; Vulvar Neoplasms; Kaposi Sarcoma; Ovarian Epithelial Cancer; Soft Tissue Sarcoma; Urethral Neoplasms; Granulosa Cell Tumor; Primitive Neuroectodermal Tumor; Neuroendocrine Tumors; Trophoblastic Tumor
Interventions: Drug: Nivolumab
Sponsors: Instituto do Cancer do Estado de SÃ£o Paulo; Financiadora de Estudos e Projetos
Recruiting
A French Multicenter Observational Retrospective Study of Rare Primary Liver Cancers

Posted: August 7th, 2024, 11:00pm UTC

Conditions: Hepatocholangiocarcinoma; Fibrolamellar Carcinoma; Hepatic Epithelioid Hemangioendothelioma; Hepatoblastoma; Hepatic Neuroendocrine Carcinoma; Hepatic Carcinosarcomas; Hepatic Cystadenoma; Hepatic Leiomyosarcomas; Hepatic Angiosarcomas; Cholangiocarcinoma
Sponsors: Federation Francophone de Cancerologie Digestive; SociÃ©tÃ© Nationale FranÃ§aise de GastroentÃ©rologie
Recruiting
Descriptive Cohort of French Patients Treated With Carbonetherapy Since October 2010 Outside PHRC-ETOILE

Posted: August 2nd, 2024, 11:00pm UTC

Conditions: Cancer; Cystic Carcinomas, Adenoid; Soft-tissue Sarcomas; Rhabdomyosarcoma Pleomorphic; Sarcoma,Soft Tissue; Osteosarcomas; Chondrosarcoma, Grade 3; Chordoma; Angiosarcomas
Interventions: Other: Questionnaire
Sponsors: Hospices Civils de Lyon; Centre FranÃ§ois Baclesse, Centre de lutte contre le cancer (CLCC)
Not yet recruiting
Evaluation of Chest CT Versus Chest X-Ray for Lung Surveillance After Curative-Intent Resection of High-Risk Truncal-Extremity Soft Tissue Sarcoma

Posted: July 30th, 2024, 11:00pm UTC

Conditions: Adult Pleomorphic Rhabdomyosarcoma; AJCC Grade 2 Sarcoma; AJCC Grade 3 Sarcoma; Alveolar Soft Part Sarcoma; Angiosarcoma; Clear Cell Sarcoma of Soft Tissue; Dedifferentiated Liposarcoma; Extraskeletal Ewing Sarcoma; Extraskeletal Myxoid Chondrosarcoma; Fibrosarcoma; Fibrosarcomatous Dermatofibrosarcoma Protuberans; Leiomyosarcoma; Malignant Peripheral Nerve Sheath Tumor; Myxofibrosarcoma; Pleomorphic Liposarcoma; Round Cell Sarcoma With EWSR1-non-ETS Fusion; Sarcoma; Soft Tissue Sarcoma; Soft Tissue Sarcoma of the Trunk and Extremities; Spindle Cell Sarcoma; Stage III Soft Tissue Sarcoma of the Trunk and Extremities AJCC v8; Synovial Sarcoma; Undifferentiated Pleomorphic Sarcoma
Interventions: Procedure: Biopsy; Procedure: Chest Computed Tomography; Procedure: Chest Radiography; Other: Quality-of-Life Assessment; Other: Questionnaire Administration
Sponsors: ECOG-ACRIN Cancer Research Group; National Cancer Institute (NCI)
Not yet recruiting
Prospective Observational Study of Localized Angiosarcoma of Any Site: ProStars

Posted: April 19th, 2024, 11:00pm UTC

Conditions: Angiosarcoma
Interventions: Other: Observational
Sponsors: Italian Sarcoma Group
Recruiting
MASCT-I Combined With Doxorubicin and Ifosfamide for First-line Treatment of Advanced Soft Tissue Sarcoma

Posted: February 26th, 2024, 1:00am UTC

Conditions: Leiomyosarcoma; Liposarcoma; Synovial Sarcoma; Angiosarcoma; Undifferentiated Pleomorphic Sarcoma; Epithelioid Sarcoma; Malignant Peripheral Nerve Sheath Tumors; Fibrosarcoma; Pleomorphic Rhabdomyosarcoma; Endometrial Stromal Sarcoma; Desmoplastic Small Round Cell Tumor
Interventions: Biological: MASCT-I; Drug: Doxorubicin; Drug: Ifosfamide
Sponsors: HRYZ Biotech Co.
Not yet recruiting
A Study of MQ710 With and Without Pembrolizumab in People With Solid Tumor Cancer

Posted: May 15th, 2023, 11:00pm UTC

Conditions: Cutaneous Squamous Cell Carcinoma; SCC - Squamous Cell Carcinoma; Basal Cell Carcinoma; BCC; BCC - Basal Cell Carcinoma; Melanoma; Merkel Cell Carcinoma; Sebaceous Carcinoma; Extramammary Paget Disease; Kaposi Sarcoma; Head and Neck Squamous Cell Carcinoma; HNSCC; Adnexal Carcinoma; Angiosarcoma; Cutaneous Neoplasm; Advanced Cancer; Metastatic Cancer; Refractory Cancer; Solid Tumor
Interventions: Biological: MQ719; Drug: Pembrolizumab
Sponsors: Memorial Sloan Kettering Cancer Center
Active, not recruiting
A Study of OMX-0407 in Patients With Previously Treated Solid Tumours That Can't be Removed Surgically

Posted: April 24th, 2023, 11:00pm UTC

Conditions: Solid Tumor
Interventions: Drug: OMX-0407
Sponsors: iOmx Therapeutics AG
Recruiting
Phase I Study of TH1 Dendritic Cell Immunotherapy for the Treatment of Cutaneous Angiosarcoma

Posted: April 5th, 2023, 11:00pm UTC

Conditions: Angiosarcoma
Interventions: Drug: Paclitaxel; Biological: mRNA plus Lysate-loaded Dendritic Cell Vaccine; Drug: PEGYLATED-INTERFERON ALPHA-2A; Drug: Filgrastim
Sponsors: M.D. Anderson Cancer Center; Cancer Cures 4 Kids
Recruiting
Sintilimab for the Treatment of Locally Advanced, Metastatic, or Recurrent Angiosarcoma, the SiARa Cancer Study

Posted: August 30th, 2021, 11:00pm UTC

Conditions: Locally Advanced Angiosarcoma; Metastatic Angiosarcoma; Recurrent Angiosarcoma
Interventions: Other: Quality-of-Life Assessment; Biological: Sintilimab
Sponsors: M.D. Anderson Cancer Center
Active, not recruiting
A Study to Learn About the Study Medicine (Called Ontorpacept or TTI-621) Given Alone and in Combination With Doxorubicin in People With Leiomyosarcoma

Posted: August 9th, 2021, 11:00pm UTC

Conditions: Leiomyosarcoma
Interventions: Drug: Ontorpacept (TTI-621); Drug: Doxorubicin
Sponsors: Pfizer
Terminated
Albumin-bound Paclitaxel Combined With Liposomal Doxorubicin in the Treatment of Advanced or Unresectable Angiosarcoma

Posted: April 26th, 2021, 11:00pm UTC

Conditions: Angiosarcoma Metastatic
Interventions: Drug: Abraxane combined with liposomal doxorubicin
Sponsors: Sun Yat-sen University
Recruiting
Lenvatinib and Pembrolizumab in People With Advanced Soft Tissue Sarcoma

Posted: March 5th, 2021, 1:00am UTC

Conditions: Advanced Sarcoma
Interventions: Drug: Lenvatinib; Drug: Pembrolizumab
Sponsors: Memorial Sloan Kettering Cancer Center; Merck Sharp & Dohme LLC; Parker Institute for Cancer Immunotherapy
Active, not recruiting
$Permalink for 'Oleclumab and Durvalumab for the Treatment of Recurrent, Refractory, or Metastatic Sarcoma'$
Oleclumab and Durvalumab for the Treatment of Recurrent, Refractory, or Metastatic Sarcoma

Posted: December 16th, 2020, 1:00am UTC

Conditions: Metastatic Angiosarcoma; Metastatic Dedifferentiated Liposarcoma; Metastatic Osteosarcoma; Recurrent Angiosarcoma; Recurrent Dedifferentiated Liposarcoma; Recurrent Osteosarcoma; Refractory Dedifferentiated Liposarcoma; Refractory Osteosarcoma
Interventions: Biological: Durvalumab; Biological: Oleclumab
Sponsors: M.D. Anderson Cancer Center; National Cancer Institute (NCI)
Recruiting
Efficacy and Safety of Regorafenib as Maintenance Therapy After First-line Treatment in Patients With Bone Sarcomas

Posted: August 13th, 2019, 11:00pm UTC

Conditions: Bone Sarcoma; Osteosarcoma; Ewing Sarcoma; Chondrosarcoma; Undifferentiated Pleomorphic Sarcoma; Leiomyosarcoma; Angiosarcoma
Interventions: Drug: Treatment by Regorafenib; Drug: Treatment by Placebo
Sponsors: Centre Leon Berard
Recruiting
Induction Paclitaxel Followed by Concurrent Paclitaxel and Radiation Therapy for Cutaneous Angiosarcoma

Posted: April 19th, 2019, 11:00pm UTC

Conditions: Cutaneous Angiosarcoma
Interventions: Drug: Paclitaxel; Radiation: Radiation therapy; Procedure: Research blood draw
Sponsors: Washington University School of Medicine
Recruiting
Fc-Engineered Anti-CTLA-4 Monoclonal Antibody in Advanced Cancer

Posted: March 1st, 2019, 1:00am UTC

Conditions: Advanced Cancer; Angiosarcoma; Colorectal Cancer Without Liver Metastases; Endometrial Cancer; Fibrolamellar Carcinoma; Non-small-cell Lung Cancer; Ovarian Cancer; Prostate Cancer
Interventions: Drug: Botensilimab; Drug: Balstilimab
Sponsors: Agenus Inc.
Recruiting
Natural History and Biospecimen Acquisition for Children and Adults With Rare Solid Tumors

Posted: November 14th, 2018, 1:00am UTC

Conditions: Malignant Solid Tumors; Other Neoplasms Solid Tumors; Pediatric Solid Tumor; Refractory Solid Tumors; Solid Tumor
Sponsors: National Cancer Institute (NCI)
Recruiting
A Study of Epacadostat, an IDO1 Inhibitor, in Combination With Pembrolizumab in Patients With Metastatic and/or Locally Advanced Sarcoma

Posted: January 29th, 2018, 1:00am UTC

Conditions: Sarcoma
Interventions: Drug: Epacadostat; Drug: Pembrolizumab
Sponsors: Memorial Sloan Kettering Cancer Center; Incyte Corporation; Merck Sharp & Dohme LLC; M.D. Anderson Cancer Center
Active, not recruiting
A Study of NKTR-214 in Combination With Nivolumab in Patients With Metastatic and/or Locally Advanced Sarcoma

Posted: September 13th, 2017, 11:00pm UTC

Conditions: SARCOMA
Interventions: Drug: NKTR-214; Drug: Nivolumab
Sponsors: Memorial Sloan Kettering Cancer Center; M.D. Anderson Cancer Center; Stanford University; Rockefeller University
Active, not recruiting
Trial of Sunitinib and/or Nivolumab Plus Chemotherapy in Advanced Soft Tissue and Bone Sarcomas

Posted: September 11th, 2017, 11:00pm UTC

Conditions: Soft Tissue Sarcoma; Bone Sarcoma
Interventions: Drug: Sunitinib 37.5 MG, Sunitinib 25 MG [Sutent]; Drug: Nivolumab 100 MG/10 ML [Opdivo]; Drug: Epirubicin; Drug: Ifosfamide; Drug: Doxorubicin; Drug: Dacarbazine; Drug: Cisplatin; Drug: Methotrexate
Sponsors: Grupo Espanol de Investigacion en Sarcomas
Completed
Nivolumab and Ipilimumab in Treating Patients With Rare Tumors

Posted: July 15th, 2016, 11:00pm UTC

Conditions: Acinar Cell Carcinoma; Adenoid Cystic Carcinoma; Adrenal Cortical Carcinoma; Adrenal Gland Pheochromocytoma; Anal Canal Neuroendocrine Carcinoma; Anal Canal Undifferentiated Carcinoma; Angiosarcoma; Apocrine Neoplasm; Appendix Mucinous Adenocarcinoma; Bartholin Gland Transitional Cell Carcinoma; Basal Cell Carcinoma; Bladder Adenocarcinoma; Breast Metaplastic Carcinoma; Cervical Adenocarcinoma; Cholangiocarcinoma; Chordoma; Colorectal Squamous Cell Carcinoma; Desmoid Fibromatosis; Endometrial Transitional Cell Carcinoma; Endometrioid Adenocarcinoma; Esophageal Neuroendocrine Carcinoma; Esophageal Undifferentiated Carcinoma; Extrahepatic Bile Duct Carcinoma; Extramammary Paget Disease; Fallopian Tube Adenocarcinoma; Fallopian Tube Transitional Cell Carcinoma; Fibromyxoid Tumor; Gallbladder Carcinoma; Gastric Neuroendocrine Carcinoma; Gastric Squamous Cell Carcinoma; Gastric Undifferentiated Carcinoma; Gastrointestinal Stromal Tumor; Gestational Trophoblastic Tumor; Giant Cell Carcinoma; Human Papillomavirus-Independent Cervical Adenocarcinoma, Clear Cell-Type; Intestinal Neuroendocrine Carcinoma; Intrahepatic Cholangiocarcinoma; Lung Neuroendocrine Tumor; Lung Sarcomatoid Carcinoma; Major Salivary Gland Carcinoma; Malignant Odontogenic Neoplasm; Malignant Peripheral Nerve Sheath Tumor; Malignant Solid Neoplasm; Malignant Testicular Sex Cord-Stromal Tumor; Metastatic Malignant Neoplasm of Unknown Primary; Metastatic Pituitary Neuroendocrine Tumor; Minimally Invasive Lung Adenocarcinoma; Mixed Mesodermal (Mullerian) Tumor; Mucinous Adenocarcinoma; Mucinous Cystadenocarcinoma; Nasal Cavity Adenocarcinoma; Nasal Cavity Carcinoma; Nasopharyngeal Carcinoma; Nasopharyngeal Low Grade Papillary Adenocarcinoma; Nasopharyngeal Undifferentiated Carcinoma; Oral Cavity Carcinoma; Oropharyngeal Undifferentiated Carcinoma; Ovarian Adenocarcinoma; Ovarian Germ Cell Tumor; Ovarian Mucinous Adenocarcinoma; Ovarian Squamous Cell Carcinoma; Ovarian Transitional Cell Carcinoma; Pancreatic Acinar Cell Carcinoma; Pancreatic Neuroendocrine Carcinoma; Paraganglioma; Paranasal Sinus Adenocarcinoma; Paranasal Sinus Carcinoma; Parathyroid Gland Carcinoma; PEComa; Penile Squamous Cell Carcinoma; Peritoneal Mesothelial Neoplasm; Placental Choriocarcinoma; Primary Peritoneal High Grade Serous Adenocarcinoma; Pseudomyxoma Peritonei; Rare Disorder; Scrotal Squamous Cell Carcinoma; Seminal Vesicle Adenocarcinoma; Seminoma; Serous Cystadenocarcinoma; Small Intestinal Adenocarcinoma; Small Intestinal Squamous Cell Carcinoma; Spindle Cell Neoplasm; Teratoma With Somatic-Type Malignancy; Testicular Non-Seminomatous Germ Cell Tumor; Thyroid Gland Carcinoma; Tracheal Carcinoma; Transitional Cell Carcinoma; Ureter Adenocarcinoma; Ureter Squamous Cell Carcinoma; Urethral Adenocarcinoma; Urethral Squamous Cell Carcinoma; Vaginal Adenocarcinoma; Vaginal Squamous Cell Carcinoma, Not Otherwise Specified; Vulvar Carcinoma
Interventions: Procedure: Biospecimen Collection; Procedure: Computed Tomography; Procedure: Echocardiography; Biological: Ipilimumab; Procedure: Magnetic Resonance Imaging; Biological: Nivolumab
Sponsors: National Cancer Institute (NCI)
Active, not recruiting
Multi-Arm Study to Test the Efficacy of Immunotherapeutic Agents in Multiple Sarcoma Subtypes

Posted: June 28th, 2016, 11:00pm UTC

Conditions: Advanced and/or Metastatic Sarcoma
Interventions: Drug: Durvalumab; Drug: Tremelimumab
Sponsors: M.D. Anderson Cancer Center; MedImmune LLC
Completed
I-SPY TRIAL: Neoadjuvant and Personalized Adaptive Novel Agents to Treat Breast Cancer

Posted: January 5th, 2010, 1:00am UTC

Conditions: Breast Neoplasms; Breast Cancer; Breast Tumors; Angiosarcoma; TNBC - Triple-Negative Breast Cancer; HER2-positive Breast Cancer; HER2-negative Breast Cancer; Hormone Receptor Positive Tumor; Hormone Receptor Negative Tumor; Early-stage Breast Cancer; Locally Advanced Breast Cancer
Interventions: Drug: Standard Therapy; Drug: AMG 386 with or without Trastuzumab; Drug: AMG 479 (Ganitumab) plus Metformin; Drug: MK-2206 with or without Trastuzumab; Drug: AMG 386 and Trastuzumab; Drug: T-DM1 and Pertuzumab; Drug: Pertuzumab and Trastuzumab; Drug: Ganetespib; Drug: ABT-888; Drug: Neratinib; Drug: PLX3397; Drug: Pembrolizumab - 4 cycle; Drug: Talazoparib plus Irinotecan; Drug: Patritumab and Trastuzumab; Drug: Pembrolizumab - 8 cycle; Drug: SGN-LIV1A; Drug: Durvalumab plus Olaparib; Drug: SD-101 + Pembrolizumab; Drug: Tucatinib plus trastuzumab and pertuzumab; Drug: Cemiplimab; Drug: Cemiplimab plus REGN3767; Drug: Trilaciclib with or without trastuzumab + pertuzumab; Drug: SYD985 ([vic-]trastuzumab duocarmazine); Drug: Oral Paclitaxel + Encequidar + Dostarlimab (TSR-042) + Carboplatin with or without trastuzumab; Drug: Oral Paclitaxel + Encequidar + Dostarlimab (TSR-042) with or without trastuzumab; Drug: Amcenestrant; Drug: Amcenestrant + Abemaciclib; Drug: Amcenestrant + Letrozole; Drug: ARX788; Drug: ARX788 + Cemiplimab; Drug: VV1 + Cemiplimab; Drug: Datopotamab deruxtecan; Drug: Datopotamab deruxtecan + Durvalumab; Drug: Zanidatamab; Drug: Lasofoxifene; Drug: Z-endoxifen; Drug: ARV-471; Drug: ARV-471 + Letrozole
Sponsors: QuantumLeap Healthcare Collaborative
Recruiting
Role of Angiogenesis in Dermatologic Diseases: A Potential Therapeutic Target

Posted: February 12th, 2009, 1:00am UTC

Conditions: Dermatologic Diseases
Interventions: Other: skin tissue sample
Sponsors: University of California, Irvine
Recruiting

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Rare Cancer News & Clinical Trials » Angiosarcoma

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Angiosarcoma (225 unread)

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