Cardiac angiosarcoma with metastatic to lung, brain, and bone

Fetched: December 5th, 2023, 5:00am GMT by Hiroshi Yamashita

Radiol Case Rep. 2023 Nov 17;19(1):473-478. doi: 10.1016/j.radcr.2023.10.056. eCollection 2024 Jan.

ABSTRACT

Cardiac angiosarcoma is a malignant tumor derived from vascular endothelium with a dismal prognosis. The imaging findings of cardiac angiosarcoma are nonspecific and endomyocardial and pericardial biopsies have insufficient accuracy. For these reasons, the diagnosis is sometimes difficult. Primary and metastatic tumors tend to bleed easily, causing hemoptysis and neurological symptoms. Brain metastases are not often known to be fatal when they cause hemorrhage. We report a 27-year-old man diagnosed with right atrium angiosarcoma, with metastases in the lung, brain, and bone. The patient had only respiratory symptoms at the first visit and did not show any symptoms derived from brain metastases yet died after 27 days due to hemorrhage from brain metastases. If brain metastasis from angiosarcoma is suspected based on imaging findings, urgent radiotherapy should be considered before histological examination for a definitive diagnosis.

PMID:38046925 | PMC:PMC10692493 | DOI:10.1016/j.radcr.2023.10.056
Paranasal sinus angiosarcoma with facial paralysis as a novel manifestation: a case report and literature review

Fetched: December 3rd, 2023, 5:00am GMT by Chengcheng Chai

BMC Neurol. 2023 Dec 2;23(1):428. doi: 10.1186/s12883-023-03482-2.

ABSTRACT

BACKGROUND: Paranasal sinus angiosarcoma is an uncommon malignancy, with only a few reported cases worldwide. Although it exhibits multiple symptoms, facial paralysis has not been previously documented as a noticeable presentation.

CASE PRESENTATION: In this case, we report a 40-year-old male who presented with facial numbness and pain for one month, weakness of his facial muscles for 15 days, and recurrent right epistaxis for 1 year. He had a history of nasal inflammatory polyps with chronic sinusitis. Computed tomography and magnetic resonance imaging showed space-occupying lesions in the right nasal cavity and maxillary sinus, with bone destruction occurring in the sinus wall and turbinate. This patient then underwent endoscopic surgery. According to the histopathological and immunohistochemical results, he was eventually diagnosed with paranasal sinus angiosarcoma in April 2021. To date, this patient has not initiated any radiotherapy or chemotherapy and has survived with lymphatic metastasis for at least 3 years.

CONCLUSIONS: This manuscript suggests that paranasal sinus angiosarcoma can present with facial paralysis. Moreover, pathological and immunohistochemical tests are still vital for diagnosing paranasal sinus angiosarcoma and differential diagnosis. Additionally, regular follow-up is crucial for patients with paranasal sinus angiosarcoma, enabling monitoring of recurrence, metastasis, and recovery while contributing valuable clinical data to understanding this rare disease and associated research endeavours.

PMID:38042771 | PMC:PMC10693057 | DOI:10.1186/s12883-023-03482-2
Recurrent, Tumor Mutation Burden-High, Cutaneous Angiosarcoma of the Scalp Treated with Pembrolizumab

Fetched: December 1st, 2023, 5:01am GMT by Ryo Amagai

Case Rep Oncol. 2023 Nov 28;16(1):1490-1493. doi: 10.1159/000534657. eCollection 2023 Jan-Dec.

ABSTRACT

INTRODUCTION: Chemoradiotherapy with taxanes is well-recognized as a first-line therapy for cutaneous angiosarcoma (CAS), but second-line therapy for CAS is still controversial.

CASE PRESENTATION: In this report, we described a 75-year-old Japanese case of recurrent, tumor mutation burden-high CAS on the scalp treated with pembrolizumab. Our present case survived for 1 year despite of taxane refractory CAS with mediastinal lymph node metastasis, though the administration of anti-PD-1 Abs alone could not fully suppress the tumor progression of CAS.

CONCLUSION: Since various factors such as pro-angiogenic molecules are correlated with the tumor progression in CAS, the administration of anti-PD-1 Abs alone could not fully suppress the tumor progression of CAS. Further novel anticancer drugs are needed in the future for the treatment of CAS.

PMID:38033417 | PMC:PMC10684255 | DOI:10.1159/000534657
Epithelioid angiosarcoma of the stomach

Fetched: December 1st, 2023, 5:01am GMT by Zhijun Lin

Rev Esp Enferm Dig. 2023 Nov 30. doi: 10.17235/reed.2023.10061/2023. Online ahead of print.

ABSTRACT

A 86-year-old man presented with a 2-month history of epigastric pain. The patient denied nausea, vomiting, hematochezia, or hematemesis. Physical examination on admission showed tenderness beneath the xiphoid process, with a palpable hard mass. Abdominal CT revealed a huge irregular inhomogeneous low density mass between liver and stomach, with moderate enhancement.

PMID:38031916 | DOI:10.17235/reed.2023.10061/2023
Case Report: Successful surgical management of a challenging primary cardiac angiosarcoma

Fetched: November 30th, 2023, 5:00am GMT by Yichen Li

Front Cardiovasc Med. 2023 Nov 3;10:1279177. doi: 10.3389/fcvm.2023.1279177. eCollection 2023.

ABSTRACT

Primary cardiac tumors are exceptionally rare, with malignant tumor occurrences ranging from 0.0017% to 0.28%. Among these, primary cardiac angiosarcoma (PCA) stands as the most prevalent malignancy, primarily impacting the right cardiac system. In this case report, we present the instance of a 44-year-old woman who recently exhibited acute chest discomfort and was subsequently diagnosed with a microangiosarcoma within the right atrium and superior vena cava. Diagnostic modalities including chest x-rays, CT, MRI, and PET-CT were instrumental in pinpointing the tumor's location and nature. Surgical excision followed by pathological and immunological examinations confirmed the diagnosis. The patient's recovery post-surgery has been encouraging, with successful follow-up chemoradiotherapy administered. Despite advancements, devising optimal strategies for enhancing patient survival and quality of life in angiosarcoma cases remains a pressing research challenge.

PMID:38028439 | PMC:PMC10655095 | DOI:10.3389/fcvm.2023.1279177
Characteristic findings of primary perirenal angiosarcoma on chemical shift subtraction magnetic resonance imaging (CSS-MRI): A case report

Fetched: November 30th, 2023, 5:00am GMT by Tetsuo Yamasaki

Radiol Case Rep. 2023 Nov 3;19(1):285-289. doi: 10.1016/j.radcr.2023.10.037. eCollection 2024 Jan.

ABSTRACT

A 70-year-old man with supraglottic carcinoma underwent computed tomography (CT) for staging purposes. A tumor measuring approximately 7 × 10 cm was found incidentally in the left perirenal space. The tumor showed homogeneous high signal intensity on chemical shift subtraction magnetic resonance imaging (CSS-MRI) suggesting the presence of minimal amounts of fat. Five months later, the tumor had grown to approximately 10 × 12 cm with indistinct margins. CSS-MRI showed high signal intensity in the tumor periphery only. The tumor was resected and the pathological diagnosis was angiosarcoma. Angiosarcomas are malignant endothelial vascular neoplasms that are highly invasive to their surroundings. Here we report a case of primary perirenal angiosarcoma that was difficult to differentiate from a dedifferentiated liposarcoma. On CSS-MRI, high signal intensity within a tumor may be a characteristic feature of primary perirenal angiosarcoma.

PMID:38028291 | PMC:PMC10652099 | DOI:10.1016/j.radcr.2023.10.037
Spinal cord haemangiosarcoma in one dog - Case report

Fetched: November 30th, 2023, 5:00am GMT by Maria Kuricova

Vet Med (Praha). 2023 Oct 30;68(10):412-418. doi: 10.17221/60/2023-VETMED. eCollection 2023 Oct.

ABSTRACT

A 5-year-old intact female Shih Tzu was presented with acute onset of hind leg paralysis. The neurologic examination revealed severe T3-L3 myelopathy. The differential diagnoses included degenerative, anomalous, traumatic, inflammatory, vascular, metabolic, and neoplastic changes. The results of the paraclinical examinations and diagnostic imaging narrowed the list of differential diagnoses and, along with the patient's deteriorating condition, led to the owner's decision to euthanise the dog. The histologic findings of the spinal cord specimens indicated a tumour of the blood vessels formed by the proliferation of endothelial cells, which may present as either capillary or cavernous structures. In this case, the tumour was a capillary-type haemangiosarcoma. The primary site of proliferation could not be determined in this case because no mass formation was noted while performing the necropsy.

PMID:38028208 | PMC:PMC10666659 | DOI:10.17221/60/2023-VETMED
Equine conjunctival haemangiosarcoma: Clinical presentation, management, and outcome of seven cases in the United Kingdom

Fetched: November 30th, 2023, 5:00am GMT by Andrea Kashani-Carver

Open Vet J. 2023 Oct;13(10):1366-1378. doi: 10.5455/OVJ.2023.v13.i10.17. Epub 2023 Oct 31.

ABSTRACT

BACKGROUND: Only 27 cases of equine conjunctival haemangiosarcoma have been reported in the literature over the past 37 years. Out of these, 22% of cases were lost to follow-up, 52% were euthanized, and 26% survived. A scarcity of cases and information is available for this rarely seen conjunctival tumour.

AIM: To describe the clinical features, management, and outcome of conjunctival hemangiosarcoma in seven horses in the UK.

METHODS: Optivet medical records were reviewed for equine cases seen or advised on with a histopathological diagnosis of conjunctival haemangiosarcoma between January 2013 and March 2023. Medical records were accessed for details of signalment, history, management, and follow-up. Histopathology was used to confirm the diagnosis of haemangiosarcoma and assess the surgical margins. Immunohistochemistry was performed in a minority of cases with poorly differentiated solid tumours to support vascular lineage.

RESULTS: Seven eyes from seven horses (five geldings and two mares) with a mean age of 16 years and median of 18 years (range 10-21 years) met the criteria. Serosanguinous discharge was seen in six eyes. All eyes were managed surgically; 4 by exenteration and 3 by conjunctivectomy/keratectomy. Adjunctive cryotherapy was performed in two eyes. Metastatic disease in the ipsilateral parotid salivary gland, confirmed with histopathology, was seen in one horse. Surgical margins were clear in all but one eye. Solar elastosis was noted in five eyes. All horses were healthy at the last follow-up (0.2-5 years, mean 2.9 years, and median 2 years).

CONCLUSION: Equine conjunctival haemangiosarcoma is rare. Serosanguinous ocular discharge is a common clinical sign. Early surgical excision is highly effective. Solar elastosis is a common histopathological feature, suggesting a role for UV-light in the pathogenesis.

PMID:38027397 | PMC:PMC10658016 | DOI:10.5455/OVJ.2023.v13.i10.17
In silico approaches in carcinogenicity hazard assessment: case study of pregabalin, a nongenotoxic mouse carcinogen

Fetched: November 30th, 2023, 5:00am GMT by Douglas A Keller

Front Toxicol. 2023 Nov 13;5:1234498. doi: 10.3389/ftox.2023.1234498. eCollection 2023.

ABSTRACT

In silico toxicology protocols are meant to support computationally-based assessments using principles that ensure that results can be generated, recorded, communicated, archived, and then evaluated in a uniform, consistent, and reproducible manner. We investigated the availability of in silico models to predict the carcinogenic potential of pregabalin using the ten key characteristics of carcinogens as a framework for organizing mechanistic studies. Pregabalin is a single-species carcinogen producing only one type of tumor, hemangiosarcomas in mice via a nongenotoxic mechanism. The overall goal of this exercise is to test the ability of in silico models to predict nongenotoxic carcinogenicity with pregabalin as a case study. The established mode of action (MOA) of pregabalin is triggered by tissue hypoxia, leading to oxidative stress (KC5), chronic inflammation (KC6), and increased cell proliferation (KC10) of endothelial cells. Of these KCs, in silico models are available only for selected endpoints in KC5, limiting the usefulness of computational tools in prediction of pregabalin carcinogenicity. KC1 (electrophilicity), KC2 (genotoxicity), and KC8 (receptor-mediated effects), for which predictive in silico models exist, do not play a role in this mode of action. Confidence in the overall assessments is considered to be medium to high for KCs 1, 2, 5, 6, 7 (immune system effects), 8, and 10 (cell proliferation), largely due to the high-quality experimental data. In order to move away from dependence on animal data, development of reliable in silico models for prediction of oxidative stress, chronic inflammation, immunosuppression, and cell proliferation will be critical for the ability to predict nongenotoxic compound carcinogenicity.

PMID:38026843 | PMC:PMC10679394 | DOI:10.3389/ftox.2023.1234498
Paediatric and Adolescent Breast Cancer: A Narrative Review

Fetched: November 30th, 2023, 5:00am GMT by Natalie Hassan

Cureus. 2023 Nov 18;15(11):e48983. doi: 10.7759/cureus.48983. eCollection 2023 Nov.

ABSTRACT

Breast cancer is the most prevalent form of cancer worldwide. Every year, it affects a significant number of women in the UK and is considered one of the leading causes of cancer-related deaths globally. While breast cancer is primarily linked to adult women, its occurrence in children and adolescents is exceedingly rare. This study conducted a narrative review spanning from 1999 to 2023, examining 32 case reports to investigate the characteristics of breast cancer in the paediatric age group. These reports focused on patients under 18 years old who were diagnosed with primary glandular breast cancer, excluding cases originating from other tissues like angiosarcoma, leukaemia, and metastatic cancer. The data analysis encompassed various parameters, including gender, age, histology, receptor status, lymph node involvement, treatment methods, and genetic characteristics. From the published case reports, it was concluded that the most common type of breast cancer affecting children and adolescents is secretory breast carcinoma and predominantly occurs in females. It is typically hormone receptors negative, and the preferred treatment approach involves mastectomy as breast conservation surgery to preserve the developing breast tissue is a real challenge due to limited breast tissue volume in this age group.

PMID:38024017 | PMC:PMC10656636 | DOI:10.7759/cureus.48983
Rapid Progression of Angiosarcoma in a Man with a Left Psoas Muscle Hematoma and a Recent EVAR of Abdominal Aorta

Fetched: November 30th, 2023, 5:00am GMT by Dimitra Alexiadou

Maedica (Bucur). 2023 Sep;18(3):519-522. doi: 10.26574/maedica.2023.18.3.519.

ABSTRACT

Angiosarcoma is a rare type of soft tissue cancer with several clinical presentations and a poor prognosis. We present a case of a 75-year-old man who was admitted due to anemia and fatigue. The patient had undergone an endovascular repair (EVAR) of a 9 cm infrarenal aneurysm of the abdominal aorta two months ago. A computed tomography (CT) scan of the abdomen on admission indicated a Type-II endoleak and a large hematoma of the left psoas muscle with multiple sites of intramuscular extravasation. Osseous metastases were found at the head of the left femoral head and at the iliac bones. A CT guided biopsy of the femoral head revealed an angiosarcoma of unknown primary site a few days after the patient had died from intra-alveolar hemorrhage caused by lung metastases.

PMID:38023756 | PMC:PMC10674126 | DOI:10.26574/maedica.2023.18.3.519
An unusually indolent well-differentiated Wilson-Jones angiosarcoma: A case report

Fetched: November 30th, 2023, 5:00am GMT by Eliana Rohr

SAGE Open Med Case Rep. 2023 Nov 16;11:2050313X231212988. doi: 10.1177/2050313X231212988. eCollection 2023.

ABSTRACT

Cutaneous angiosarcomas are rare soft tissue tumours originating from hematogenous vasculature that are aggressive and carry a poor prognosis. We describe the case of a 73-year-old man with a low-grade well-differentiated angiosarcoma. Our case distinguishes itself from those previously reported in the slow progression and important delay to the presentation of 30 months and survival time of 5.5 years. Additionally, its severe clinical appearance (T2 stage) but milder pathological picture (T1 stage) is very uncommon. A repeat biopsy is warranted when results are inconclusive and there is a high clinical suspicion of angiosarcoma.

PMID:38022853 | PMC:PMC10656791 | DOI:10.1177/2050313X231212988
Massive Localized Lymphedema: Two Case Studies and Diagnostic Challenges

Fetched: November 30th, 2023, 5:00am GMT by Saleh A Ba-Shammakh

Cureus. 2023 Oct 16;15(10):e47092. doi: 10.7759/cureus.47092. eCollection 2023 Oct.

ABSTRACT

Massive localized lymphedema (MLL) is an emerging clinical phenomenon predominantly observed in morbidly obese individuals. It presents both diagnostic and therapeutic challenges to clinicians due to its characterization by large, pendulous masses in the abdomen or thigh. MLL may resemble malignant conditions, such as liposarcoma, leading to unnecessary invasive interventions. This study presents two case studies: a 74-year-old male who succumbed to postoperative complications and a 56-year-old female who experienced successful recovery. These cases highlight the urgent need for robust diagnostic criteria and evidence-based management approaches for MLL. In addition, further research exploring the pathogenesis, risk factors, and potential connections among MLL, hypothyroidism, and angiosarcoma is essential.

PMID:38021692 | PMC:PMC10646702 | DOI:10.7759/cureus.47092
Recurrence of scalp angiosarcoma after multiple surgeries: A case report and literature review

Fetched: November 30th, 2023, 5:00am GMT by Dongjian Li

Oncol Lett. 2023 Oct 31;26(6):536. doi: 10.3892/ol.2023.14122. eCollection 2023 Dec.

ABSTRACT

Scalp angiosarcoma (SA) is rare, accounting for <1% of soft tissue sarcomas, with a high degree of malignancy, a high recurrence rate and a poor prognosis. The best treatment strategy is uncertain. Therefore, it is essential to continuously refine treatment strategies and improve the prognosis of patients. Curative-intent surgery increases overall survival in patients with primary cutaneous angiosarcoma of the scalp and face, and radiation therapy combined with chemotherapy is now recommended for the curative treatment of patients who both can or cannot undergo surgery. The present case report is of an 87-year-old man hospitalised for the fifth time with SA. He had experienced four recurrences and previously underwent curative-intent surgery four times. However, the patient did not undergo radiotherapy or chemotherapy after any of the surgeries. A detailed report of the management of this case is presented along with a review of the relevant literature. It is hypothesised that patients with SA should receive a combination of radiotherapy and chemotherapy after surgery whenever possible, which may improve patient prognosis.

PMID:38020301 | PMC:PMC10655065 | DOI:10.3892/ol.2023.14122
Multiple angiosarcomas of both breasts: a case report

Fetched: November 29th, 2023, 5:00am GMT by Ryota Matsuda

Surg Case Rep. 2023 Nov 28;9(1):205. doi: 10.1186/s40792-023-01782-w.

ABSTRACT

BACKGROUND: Primary angiosarcomas of the breast are rare and highly aggressive. We herein report a rare case of multiple angiosarcomas detected concurrently in both breasts.

CASE PRESENTATION: A 49-year-old woman visited a doctor after noticing a lump in her right breast. At that time, mammography and ultrasonography revealed no abnormal findings in either breast. She was referred to our hospital 5 months later, because screening mammography had revealed a focal asymmetric density in her right breast. Ultrasonography showed ill-defined hyper- and hypo-echoic lesions in both breasts. Magnetic resonance imaging disclosed five heterogeneously enhanced masses (5.8 cm in maximum diameter) in the right breast and six enhanced masses (approximately 1-3 cm in diameter) in the left breast. Histological examination of core needle biopsies revealed proliferation of irregularly shaped vascular channels lined by atypical endothelial cells throughout the adipose tissue and lobules of the breasts, leading to a diagnosis of well-differentiated angiosarcoma. The lesions were assumed to be primary angiosarcomas, because she had neither a history of breast surgery nor of radiation therapy. She underwent bilateral mastectomies and postoperative chest wall irradiation. Computed tomography 11 weeks after the surgery revealed multiple, small, subcutaneous nodules in the chest wall that were suspected of being angiosarcoma metastases. We started chemotherapy (weekly paclitaxel 80 mg/m²), which achieved shrinkage of these nodules within 2 months.

CONCLUSIONS: Early diagnosis, immediate initiation of local and systemic therapies, and intensive follow-up are important in improving the prognosis of angiosarcomas.

PMID:38015377 | PMC:PMC10684844 | DOI:10.1186/s40792-023-01782-w
Suppression or Inhibition of VEGFs on Splenic Angiosarcoma Cell with Traditional Chinese Medicine Zhi Gan Cao

Fetched: November 27th, 2023, 5:01am GMT by Tianyang Zhao

Stud Health Technol Inform. 2023 Nov 23;308:396-403. doi: 10.3233/SHTI230865.

ABSTRACT

Primary splenic angiosarcoma is a very rare disease that causes the development of malignant tumors in the vascular endothelium of the splenic sinuses. Moreover, the disease maintains a very low survival rate for patients to live over 5 years, which is relatively low when compared to another splenic cancer, splenic lymphomas. The treatment options for splenic angiosarcoma narrow down to surgical removal or radiation combined with chemotherapy, but both cost a lot, so discovering potential alternative treatments may eventually increase the possible survival rate. Ginseng and Zhi Gan Cao are both common herbs in Traditional Chinese Medicine (TCM); however, the price of Ginseng is much higher than that of Zhi Gan Cao. A possible reason could be the frequent studies and researches over Ginseng's active ingredient, ginsenoside rh2 or rg3 as they are both potent cancer treatments. The reason to study Zhi Gan Cao and predict its possible potential in cancer treatment is due to the similarity between its active ingredient and the active ingredient in Ginseng, namely, ginsenoside rh2 and licorice saponins. Both TCM contain the active ingredient, triterpenoid saponin, as their main composition, and the further text will predict the possible research and results that may be taken in vitro to reveal the question of whether licorice saponin has the potential to become a major treatment for splenic angiosarcoma or not.

PMID:38007765 | DOI:10.3233/SHTI230865
Clinical outcomes of scalp or face angiosarcoma treatment with intensity-modulated radiotherapy: a multicenter study

Fetched: November 24th, 2023, 5:00am GMT by Takahiro Iwai

J Radiat Res. 2023 Nov 22:rrad089. doi: 10.1093/jrr/rrad089. Online ahead of print.

ABSTRACT

Combined modality therapy, including radiotherapy (RT), is a common treatment for scalp or face angiosarcoma. Although intensity-modulated radiotherapy (IMRT) can deliver homogeneous doses to the scalp or face, clinical data are limited. This multicenter study aimed to evaluate scalp or face angiosarcoma treated with definitive or post-operative IMRT. We retrospectively analyzed data from patients who received IMRT for scalp or face angiosarcoma at three institutions between January 2015 and March 2020. Local control (LC) rate, overall survival (OS), progression-free survival (PFS), recurrence patterns and toxicity were evaluated. Fifteen patients underwent IMRT during the study period. Definitive RT was performed on 10 patients and post-operative RT was performed on 5 patients. The 1-year LC rate was 85.7% (95% confidence interval [CI], 53.9-96.2%). The 1-year OS and PFS rates were 66.7% (95% CI, 37.5-84.6%) and 53.3% (95% CI, 26.3%-74.4%), respectively. Univariate analysis revealed that a clinical target volume over 500 cm3 was associated with poor LC. Distant metastasis was the most common recurrence pattern. All patients experienced Grade 2 or 3 radiation dermatitis, and five patients experienced grade ≥ 3 skin ulceration. One patient who underwent maintenance therapy with pazopanib developed Grade 5 skin ulceration. Fisher's exact test showed that post-operative RT was significantly associated with an increased risk of skin ulceration of grade ≥ 3. These results demonstrate that IMRT is a feasible and effective treatment for scalp or face angiosarcoma, although skin ulceration of grade ≥ 3 is a common adverse event in patients who receive post-operative RT.

PMID:37996084 | DOI:10.1093/jrr/rrad089
Right Atrial Angiosarcoma with Embolic Lung Metastases Masquerading as Atypical Pneumonitis

Fetched: November 23rd, 2023, 5:01am GMT by Chung-Fu Lin

Am J Respir Crit Care Med. 2023 Nov 22. doi: 10.1164/rccm.202307-1207IM. Online ahead of print.

NO ABSTRACT

PMID:37991406 | DOI:10.1164/rccm.202307-1207IM
Uterine haemangiosarcoma in a Netherland Dwarf rabbit (Oryctolagus cuniculus Netherland dwarf)

Fetched: November 23rd, 2023, 5:01am GMT by Mami Murakami

Vet Med Sci. 2023 Nov 21. doi: 10.1002/vms3.1311. Online ahead of print.

ABSTRACT

A 7-year-old intact Netherlands Dwarf rabbit with bloody discharge from the vulva underwent ovariohysterectomy. Grossly, both sides of the uterus were enlarged. Histologically, the tumour had formed protruded from the myometrial wall toward the serosa and was composed of irregular small capillaries with irregularly shaped structures and bundled proliferation of spindle-shaped cells. No tumour cells infiltrated the endometrium. The tumour cells were positive for CD31, and histological and immunohistochemical staining confirmed the diagnosis of haemangiosarcoma. Vascular tumours in the uterus of animals are uncommon, and only one case has been reported in the uterus of rabbits.

PMID:37990488 | DOI:10.1002/vms3.1311
Malignant pleural effusion due to angiosarcoma: A rare entity

Fetched: November 22nd, 2023, 5:01am GMT by Ashley N Meerschaert

JAAPA. 2023 Dec 1;36(12):1-4. doi: 10.1097/01.JAA.0000977676.98481.5e.

ABSTRACT

Angiosarcoma is a rare and aggressive vascular malignancy that typically originates in the skin or soft tissue of the body. It is known to have a propensity for metastasis to the lung parenchyma in the form of pulmonary nodules and cavitary lesions; however, a less commonly described entity is in the form of a malignant pleural effusion. Management of a malignant pleural effusion due to angiosarcoma presents a unique challenge. This article describes the challenges faced during one patient's diagnostic and treatment course, and the anticipated future complications of his aggressive disease.

PMID:37989195 | DOI:10.1097/01.JAA.0000977676.98481.5e
Cardiac angiosarcoma: a case report

Fetched: November 22nd, 2023, 5:01am GMT by Alireza Omidi Farzin

J Int Med Res. 2023 Nov;51(11):3000605231211772. doi: 10.1177/03000605231211772.

ABSTRACT

Angiosarcoma is the most invasive and malignant cardiac tumor and most commonly originates from the right atrium. Early diagnosis is essential, and echocardiography has an important role in diagnosis. This tumor grows aggressively, and metastases to other sites makes it difficult to control. Surgical treatment remains the best option for patients who do not respond to chemoradiotherapy. We herein report a case of a 17-year-old patient with cardiac angiosarcoma who presented with dyspnea, chest pain, dry cough, and fever. Although we considered the most probable diagnosis to be constrictive pericarditis, pathologic examination revealed a primary angiosarcoma originating from the pericardium. The patient underwent total pericardiectomy. However, despite receiving chemotherapy for 2 weeks postoperatively, she developed complications including leukopenia and eventually died of respiratory failure. Late diagnosis of angiosarcoma often occurs, resulting in progression to end-stage disease and a very poor prognosis. Therefore, a thorough understanding of this entity, knowledge of its pitfalls in management, and establishment of an accurate treatment guideline would help to develop a reliable and life-saving treatment approach for these patients.

PMID:37987637 | PMC:PMC10664435 | DOI:10.1177/03000605231211772
Familial Cutaneous Angiosarcoma of the Head

Fetched: November 22nd, 2023, 5:01am GMT by Masakazu Kakurai

Acta Derm Venereol. 2023 Nov 21;103:adv15314. doi: 10.2340/actadv.v103.15314.

NO ABSTRACT

PMID:37987628 | PMC:PMC10680977 | DOI:10.2340/actadv.v103.15314
Malignant tumors of the external auditory canal: diagnosis, treatment, genetic landscape, biomarkers, and clinical outcome

Fetched: November 17th, 2023, 5:01am GMT by Pinelopi Samara

Explor Target Antitumor Ther. 2023;4(5):801-811. doi: 10.37349/etat.2023.00169. Epub 2023 Sep 21.

ABSTRACT

Malignant tumors of the external auditory canal (EAC) are rare neoplasms that appear in the head and neck area. A common feature of these malignancies is their rarity, as well as their delayed diagnosis due to the appearance of non-specific symptoms that mimic various benign otologic conditions. The reported histological types of cancer of the external ear are: squamous cell carcinoma, basal cell carcinoma, malignant melanoma, Merkel cell carcinoma, angiosarcoma, adnexal carcinoma (including ceruminous adenocarcinoma and adenoid cystic carcinoma), and lymphoma (Lancet Oncol. 2005;6:411-20. doi: 10.1016/S1470-2045(05)70208-4). Several therapeutic interventions have been proposed, primarily orientated towards the cure of the patient, placing the surgical excision of the lesions at the tip of the spear. Subsequently and depending on the clinical stage and the pathological characteristics of the tumor, radiation, chemotherapy, a combination thereof, or some form of palliative treatment for particularly advanced cases, may be recommended. The aim of all the above-mentioned approaches is the complete resection of the mass with negative surgical margins along with lymph node dissection, the elimination of any residual disease or metastasis, and the improvement of survival. The anatomical complexity of the region will always remain a demanding challenge. Nevertheless, advances in the fields of ear microsurgery, imaging, radiation, molecular biology, and genomics have led to remarkable outcomes compared to the past, with a view to the patient's quality of life. Large, well-organized, and prospective studies with the participation of multiple centers in contrast to existing retrospective studies with a limited number of patients will help to establish universally accepted guidelines. The exploration of the molecular and genetic background of these cancers in conjunction with the search for new biomarkers and target molecules seems promising for providing upgraded and more personalized treatment modalities for the future.

PMID:37970205 | PMC:PMC10645464 | DOI:10.37349/etat.2023.00169
Oral mucosa involvement in pediatric patients with xeroderma pigmentosum: a comprehensive review

Fetched: November 16th, 2023, 5:00am GMT by Defne Baskurt

Int J Dermatol. 2023 Nov 14. doi: 10.1111/ijd.16890. Online ahead of print.

ABSTRACT

BACKGROUND: Xeroderma pigmentosum (XP) is a rare autosomal recessive disorder presenting with an inability to repair UV-induced DNA damage. This can lead to the development of neoplasms affecting multiple organ systems, with onset often in childhood. Unfortunately, no cure currently exists for XP, and management strategies focus on sun protection and early intervention for malignancies. Although most skin problems in XP patients are UV induced, various oral lesions are also described. However, the literature has not extensively characterized the oral manifestations and their prognostic significance.

METHODS: We conducted a comprehensive review to evaluate the prevalence and nature of oral mucosal lesions in pediatric XP patients.

RESULTS: Our literature search yielded 130 pediatric XP patients with oral involvement and 210 associated tumoral or non-tumoral lesions. Squamous cell carcinoma was the most common type of oral mucosal tumor reported, with other malignancies including basal cell carcinoma, melanoma, angiosarcoma, fibrosarcoma, and trichilemmal carcinoma.

CONCLUSION: Given the potential morbidity and mortality associated with oral mucosal tumors in XP patients, our study aims to raise awareness of these manifestations. Early diagnosis and treatment are crucial for managing these lesions effectively, and routine oral exams should be considered a critical component of dermatological evaluations for XP patients, especially in the pediatric age group.

PMID:37964400 | DOI:10.1111/ijd.16890
Masson's Tumor as an Uncommon Cause of Neck Mass: A Case Presentation

Fetched: November 15th, 2023, 5:01am GMT by Ehab M Abdo

Vasc Endovascular Surg. 2023 Nov 14:15385744231215102. doi: 10.1177/15385744231215102. Online ahead of print.

ABSTRACT

BACKGROUND: Masson's tumor, commonly referred to as intravascular papillary endothelial hyperplasia (IPEH), is an uncommon growth of endothelial cells within a vessel wall that is frequently assumed to indicate an abnormal resolution of thrombosis. IPEH is most typically found in the extremities however it is rare for IPEH to appear as a neck tumor. The issue with IPEH is that it could clinically, radiologically, and pathologically imitate some malignant neoplasms such as angiosarcomas creating a diagnostic challenge.

CASE REPORT: We describe a 21-year-old male patient who presented with right anterolateral neck swelling for 12 months. Ultrasound revealed a 9.0 × 8.0 cm well-defined echogenic hyper-vascular lesion. The contrast computed tomography (CT) scan of the neck revealed an oval, well-defined subcutaneous mass, measuring 9 × 4.5 cm, situated over and separable from the right sternocleidomastoid muscle with no significant enhancement in the post-contract study. T1-weighted and T2-weighted MRI revealed a 10 × 9 × 7 cm well-defined subcutaneous lobulated lesion superficial to the sternocleidomastoid expanding upward to the Rt. side of the cheek and below to the suprasternal region, eliciting an intermediate signal in T1 and a heterogenous bright signal (mostly fluid) in T2 with low signal foci within the mass. The decision had been reached to entirely excise the lesion surgically with safety margins for histological evaluation. Histological examination indicated thrombosed variable-sized ectatic vascular spaces with papillary formations related to the thrombus, covered with a single layer of flat endothelium, and no features suggestive of malignancy. There was no recurrence at 18 months follow-up post-surgery.

CONCLUSION: Masson's tumor is a benign intravascular disease with an unclear origin and no confirmed inheritance pattern. Presentation of Masson's tumor as a neck mass is incredibly uncommon. Masson's tumor lacks a distinct or distinguishing clinical and radiological appearance. Histopathologic examination is the sole definitive way for diagnosing the disease and the only tool for distinguishing it from angiosarcoma. Surgical excision is the best treatment for IPEH. Recurrence is extremely rare.

PMID:37962479 | DOI:10.1177/15385744231215102
Primary epithelioid angiosarcoma of endometrium

Fetched: November 14th, 2023, 5:00am GMT by Selim Sevim

Pol J Pathol. 2023;74(3):219-222. doi: 10.5114/pjp.2023.132302.

ABSTRACT

Angiosarcoma is a poor prognostic tumor observed less than 1% in soft tissue, while it is rarely detected in the endometrium and has been described in few case reports. In this report, we present a case of primary epithelioid angiosarcoma of endometrium to raise awareness and emphasize for pathologists and clinicians.

PMID:37955542 | DOI:10.5114/pjp.2023.132302
Multimodal imaging features of retroperitoneal anastomosing hemangioma: a case report and literature review

Fetched: November 14th, 2023, 5:00am GMT by Liqing Zhang

Front Oncol. 2023 Oct 25;13:1269631. doi: 10.3389/fonc.2023.1269631. eCollection 2023.

ABSTRACT

BACKGROUND: Anastomotic hemangioma is a rare subtype of capillary hemangioma primarily found in the genitourinary tract. We present a case of a patient with an anastomotic hemangioma located in the retroperitoneal space; then, we explore and summarize the imaging features from previously reported cases for accurate diagnosis.

CASE PRESENTATION: A 57-year-old woman complained of left lower back pain. Contrast-enhanced ultrasound revealed a hypoechoic mass with "slow-in and slow-out" enhancement. Abdominal CT scan displayed a well-defined, round soft tissue mass in the right retroperitoneal region with obvious enhancement. MRI indicated low signal on T1-weighted imaging, high signal on T2-weighted imaging and diffusion-weighted imaging, and progressive enhancement after enhancement. Surgical removal of the tumor was performed. Histopathological examination exhibited a distinct tumor border with interconnected blood vessels and a cavity lined by a single layer of cubic endothelial cells. Immunohistochemistry confirmed the presence of CD31[+] and CD34[+]. The final pathological diagnosis was anastomotic hemangioma. No recurrence was observed during a 40-month follow-up.

CONCLUSION: Retroperitoneal anastomotic hemangioma is a rare and benign neoplasm that may be misdiagnosed as ectopic pheochromocytoma or angiosarcoma. This case report presents and analyzes the imaging characteristics of a series of retroperitoneal anastomotic hemangiomas, which can be valuable for future diagnoses and help prevent unnecessary surgeries.

PMID:37954079 | PMC:PMC10634416 | DOI:10.3389/fonc.2023.1269631
Vascular tumors of the liver: A brief review

Fetched: November 14th, 2023, 5:00am GMT by Sujata Sarangi

Ann Hepatobiliary Pancreat Surg. 2023 Nov 30;27(4):329-341. doi: 10.14701/ahbps.23-046. Epub 2023 Nov 13.

ABSTRACT

Vascular tumors of the liver are mesenchymal lesions from endothelial cells. They range from common benign lesions such as haemangioma, intermediate tumors like Kaposi sarcoma, and perivascular epithelioid cell tumor to malignant tumors such as hepatic epithelioid hemangioendothelioma and hepatic angiosarcoma in adults. Pediatric vascular tumors of the liver also include benign, locally aggressive, borderline, and malignant masses with haemangiomas being the most common benign tumors and epithelioid hemangioendothelioma being an uncommon pediatric malignancy. The list of these lesions is completed by nodular regenerative hyperplasia, solitary fibrous tumour, and hepatic small vessel neoplasms (HSVN). Some of these tumors are uncommon and rare. This review article aimed to enumerate hepatic vascular tumors along with their imaging, histopathology, molecular findings for accurate diagnosis that can result in better management.

PMID:37953709 | DOI:10.14701/ahbps.23-046
What is your diagnosis? Peritoneal fluid from a Hanoverian horse

Fetched: November 11th, 2023, 5:00am GMT by Cornelius J Withers

Vet Clin Pathol. 2023 Nov 10. doi: 10.1111/vcp.13300. Online ahead of print.

NO ABSTRACT

PMID:37947167 | DOI:10.1111/vcp.13300
Efficacy and safety of TM5614 in combination with paclitaxel in the treatment of paclitaxel-resistant cutaneous angiosarcoma: Phase II study protocol

Fetched: November 11th, 2023, 5:00am GMT by Taku Fujimura

Exp Dermatol. 2023 Nov 9. doi: 10.1111/exd.14976. Online ahead of print.

ABSTRACT

Cutaneous angiosarcoma (CAS) is an endothelial cell-derived, highly aggressive type of vascular tumour. Although chemoradiotherapy with paclitaxel (PTX) is recognized as a first-line therapy for CAS, second-line therapy for CAS remains controversial, and there is no standard therapy for taxane-resistant CAS. Plasminogen activator inhibitor-1 (PAI-1) is associated with poor clinical outcomes, and elevated levels of PAI-1 in both tissue and serum are correlated with poor response to therapy in various cancers, including skin cancers. Since PAI-1 protects endothelial cells from Fas ligand-mediated apoptosis, PAI-1 inhibition might induce apoptosis of endothelial cell-derived tumours such as CAS. This is a single-arm, open-label, multi-institutional, Phase 2 clinical trial to assess the efficacy and safety of PTX in combination with TM5614 (PAI-1 inhibitor) in patients with PTX-resistant CAS. PTX will be administered for 28 weeks, with oral administration of TM5614. The primary endpoint of this study will be the overall response rate (ORR) at 28 weeks after starting treatment (central image evaluation). The secondary endpoint will include the ORR at 28 weeks after starting treatment (investigator evaluation), ORR at 8 weeks and 16 weeks after initiation of treatment (central and investigator evaluation), progression-free survival, overall survival, disease control rate and safety profiles. Assuming the null hypothesis of a response rate of 13.6% and an alternative hypothesis of 45%, a minimum of 15 patients are required to achieve a two-sided, Type I error of 5% and power of 70% based on the exact binomial distribution. Data quality control will be conducted by a combination of centralized (remote) and on-site monitoring. This study will contribute to the development of novel combination therapy for PTX-resistant CAS patients, which remains an unmet clinical need.

PMID:37946551 | DOI:10.1111/exd.14976
Surgery for primary splenic tumors and metastases of the spleen

Fetched: November 10th, 2023, 5:00am GMT by F Huettl

Chirurgie (Heidelb). 2023 Dec;94(12):994-999. doi: 10.1007/s00104-023-01978-8. Epub 2023 Nov 9.

ABSTRACT

The correct indications for surgical treatment of primary splenic tumors as well as metastases of the spleen are challenging due to the rarity of the various entities. Primary solid splenic tumors include benign lesions, such as hemangiomas, hamartomas and sclerosing angiomatous nodular transformation (SANT) of the spleen. In these cases, surgical treatment is indicated only in the case of inconclusive imaging and after careful consideration of the risk-benefit ratio, even in the case of pronounced symptoms. In contrast, primary angiosarcoma or undifferentiated pleomorphic sarcoma as highly malignant tumors represent an urgent indication for surgery. Although more frequent than primary splenic malignancies, secondary splenic tumors are also not that frequent. Solitary splenic metastases are rare; however, from an oncological point of view they can be treated by resection. In the case of oligometastasis with splenic involvement, splenectomy is used only as part of a palliative concept in cases of pronounced symptoms or in the context of cytoreductive surgery. In general, the laparoscopic approach is to be preferred when the operation is technically feasible as it is associated with fewer pulmonary and infectious complications and a shorter hospital stay. In addition, to reduce the risk of severe infections after splenectomy, the option of partial splenectomy should be considered, especially for benign lesions. A thorough informing of the patient regarding both intraoperative and perioperative risks as well as potential long-term sequelae, especially severe infectious diseases, is an essential component of informed consent before surgery.

PMID:37946024 | DOI:10.1007/s00104-023-01978-8
Radiation-Associated Breast Angiosarcoma

Fetched: November 10th, 2023, 5:00am GMT by Ryusei Yoshino

Cureus. 2023 Oct 8;15(10):e46673. doi: 10.7759/cureus.46673. eCollection 2023 Oct.

ABSTRACT

Radiation-associated breast angiosarcomas are rare following breast-conserving surgery. These angiosarcomas are considered adverse events associated with radiation therapy and are characterized by a high risk of both local and distant recurrence, even after complete resection. Despite this, there is currently no established standard treatment for them. The patient was a 70-year-old woman who had breast-conserving surgery for right breast cancer 10 years before presentation. She was followed up for 10 years after receiving 50 Gy of residual breast irradiation and an aromatase inhibitor for 5 years. During follow-up, a painless purplish area with induration, measuring 10 cm by 5.4 cm, was noted on the right nipple. A skin biopsy confirmed hemangiosarcoma. Treatment included surgery with a 2.0 cm margin from the area, followed by skin excision and total mastectomy. A final diagnosis of radiation-associated breast angiosarcoma was made. Radiation-associated breast angiosarcoma is a rare disease with a poor prognosis that lacks standard treatment. An aggressive skin biopsy should be considered when skin findings such as purpura are seen after breast cancer radiotherapy, as in this case.

PMID:37942397 | PMC:PMC10628990 | DOI:10.7759/cureus.46673
Anastomosing haemangioma of the mediastinum: Clinicopathological series with radiological and genetic characterisation

Fetched: November 9th, 2023, 5:00am GMT by Nicholas J Caldwell

Histopathology. 2023 Nov 7. doi: 10.1111/his.15085. Online ahead of print.

ABSTRACT

AIMS: Anastomosing haemangiomas are benign tumours with anastomosing vascular channels that may mimic angiosarcoma. While anastomosing haemangiomas have been described in diverse locations, particularly the abdominal/paraspinal region, data on anastomosing haemangiomas in the mediastinum remain limited. We report the clinicopathological, radiological and molecular characteristics of the largest single-institutional series of mediastinal anastomosing haemangiomas.

METHODS AND RESULTS: In our pathology archives in 2011-23, we reviewed all vascular lesions involving the mediastinum and identified seven anastomosing haemangiomas. Clinical information was abstracted from medical charts; available radiological imaging was reviewed. Targeted DNA-based next-generation sequencing (447 genes, including GNAQ and GNA11) was performed on five cases. The seven patients included five women and two men, with an age range of 55-77 (median = 72) years. Of the six tumours with available radiology, two each were in the prevascular, visceral and paravertebral mediastinum, with lobulated peripheral enhancement in all tumours examined with contrast enhancement. Six patients underwent tumour resection; one patient received proton radiotherapy. Microscopically, each tumour was solitary and characterised by anastomosing capillary-sized vessels lined by hobnail endothelial cells. Fibrin microthrombi, hyaline globules and extramedullary haematopoiesis were common. In the five tumours analysed by next-generation sequencing, GNAQ p.Q209P was identified in one tumour; no additional reportable alterations were identified in the remaining cases. No recurrence was noted in the four patients with available follow-up of 3-58 (median = 9.5) months after resection.

CONCLUSION: While mediastinal anastomosing haemangiomas can microscopically mimic angiosarcoma, awareness of this entity and radiological correlation may help to circumvent this diagnostic pitfall.

PMID:37936489 | DOI:10.1111/his.15085
New risk factors and molecular landscapes of hepatic angiosarcoma in the Taiwanese population†

Fetched: November 7th, 2023, 5:01am GMT by Felicia Chung

J Pathol. 2023 Nov 6. doi: 10.1002/path.6224. Online ahead of print.

ABSTRACT

Hepatic angiosarcoma is a rare, highly aggressive malignancy of the liver. The tumorigenesis of hepatic angiosarcoma has been relatively understudied in terms of aetiology and molecular properties. A recent study published in The Journal of Pathology revealed a strong association between hepatic angiosarcoma incidence and chronic kidney disease, particularly in end-stage renal disease using population-based data from the National Health Insurance Research Database in Taiwan and an institutional cohort. The study also revealed enrichment in the mutational signature of aristolochic acid exposure and is the first reported observation of this mutational signature in human sarcomas. © 2023 The Pathological Society of Great Britain and Ireland.

PMID:37929656 | DOI:10.1002/path.6224
An Autopsy Case of Ruptured Hepatic Angiosarcoma Treated by Transcatheter Arterial Embolization

Fetched: November 7th, 2023, 5:01am GMT by Toshiki Entani

Case Rep Gastroenterol. 2023 Oct 16;17(1):309-315. doi: 10.1159/000533552. eCollection 2023 Jan-Dec.

ABSTRACT

An 80-year-old Japanese man presented to our hospital with intra-abdominal hemorrhage due to a ruptured liver tumor. Transcatheter arterial embolization (TAE) temporarily achieved hemostasis, but he died following re-rupture 4 days later. Based on autopsy findings, the liver tumor was diagnosed as hepatic angiosarcoma. Embolic agents used during embolization were identified within the hepatic small interlobular arteries. However, there were no findings of tumor cell necrosis or ischemic change in the angiosarcoma. In the present case, TAE alone did not induce ischemia-induced tumor necrosis, suggesting that TAE might be unsuitable to treat hepatic angiosarcoma. Treatment optimization for ruptured hepatic angiosarcoma is desired.

PMID:37928973 | PMC:PMC10624939 | DOI:10.1159/000533552
Spindle Cell Renal Neoplasms: A Pathological Case Report on Primary Renal Leiomyosarcoma and Sarcomatoid Renal Cell Carcinoma

Fetched: November 7th, 2023, 5:01am GMT by Hristo Popov

Cureus. 2023 Oct 3;15(10):e46449. doi: 10.7759/cureus.46449. eCollection 2023 Oct.

ABSTRACT

Renal oncopathology in adults, as a field of pathology, is dominated by a single entity - clear cell renal cell carcinoma (RCC) with other entries, such as urothelial carcinoma of the renal pelvis, angiosarcoma, and others being extremely rare. Herein, we report two histopathological cases with differential diagnoses of spindle cell renal neoplasms. The first patient, a 42-year-old male, presented with new-onset right-sided abdominal flank pain, and imaging showed a 12 cm renal tumor. Histopathology showed a spindle cell neoplasm, with significant mitotic activity and giant cell, with immunohistochemistry being positive for caldesmon and vimentin, focally for smooth muscle actin (SMA). No reaction was noted for pan-cytokeratin (CK AE1/AE3), epithelial membrane antigen (EMA), cytokeratin (CK) 7, cluster of differentiation (CD) 117, soluble 100 protein (S100), human melanoma black (HMB) 45, Melan A, CD10, and desmin. Due to peculiar histomorphology and the immunophenotype, the tumor was interpreted as primary renal leiomyosarcoma. Due to continuous outpatient consultations, treatment initiation was delayed, and three months later, the patient had already developed an 87 mm local recurrence and liver metastasis. The second patient, a 53-year-old male, presented to our institution for consultation of an already excised renal tumor, diagnosed as an incidental finding on a prophylactic abdominal ultrasound. The tumor presented for consultation histologically grew as intertwining bundles of spindle cells with polymorphic hyperchromic nuclei with prominent nucleoli and had extensive areas with necrosis. Immunohistochemically, the tumor diffusely expressed CK AE1/AE3 and caldesmon and had a patchy reaction for EMA and CD10. The SMA, desmin, CD117, and CK7 reactions were negative; hence, the tumor was interpreted as a spindle cell variety (sub-type) of clear RCC.

PMID:37927619 | PMC:PMC10623498 | DOI:10.7759/cureus.46449
[[Translated article]]A Rare Case of Cutaneous Angiosarcoma Secondary to Chronic Lymphedema in a Patient on Hemodialysis

Fetched: November 7th, 2023, 5:01am GMT by A M Carballido Vázquez

Actas Dermosifiliogr. 2023 Nov 1:S0001-7310(23)00858-X. doi: 10.1016/j.ad.2022.09.031. Online ahead of print.

NO ABSTRACT

PMID:37923076 | DOI:10.1016/j.ad.2022.09.031
Association of Lower Extremity Lymphedema and Nonmelanoma Skin Cancers

Fetched: November 6th, 2023, 5:01am GMT by Nimay C Anand

Mayo Clin Proc. 2023 Nov;98(11):1653-1659. doi: 10.1016/j.mayocp.2023.02.030.

ABSTRACT

OBJECTIVE: To determine whether patients with lymphedema of a lower extremity (LE) had a greater risk of skin cancer than those without lymphedema.

PATIENTS AND METHODS: This retrospective cohort study included patients with LE lymphedema examined at Mayo Clinic in Rochester, MN, USA, from January 1, 2000, through December 31, 2020. All patients with the phrase "lower extremity lymphedema" and a diagnostic code for lymphedema present in their electronic health record, as well as their age-, race-, and sex-matched controls without lymphedema, were included in the study. A Kaplan-Meier curve was constructed to examine the time to development of the first skin cancer for the lymphedema cohort and the controls. A Cox proportional hazards regression model was used to calculate hazard ratios.

RESULTS: In total, 4437 patients had lymphedema within the study period. Compared with the matched control group, the lymphedema group had a significantly increased risk of skin cancer. For the subset of patients with unilateral lymphedema, the lymphedematous extremity was 2.65 times as likely as the nonlymphedematous LE to have skin cancer, particularly basal cell carcinoma.

CONCLUSION: Lower extremity lymphedema appears to be a risk factor for squamous cell carcinoma, basal call carcinoma, and as expected, angiosarcoma. Clinicians caring for patients with LE lymphedema should be aware of this increased risk and monitor at-risk patients accordingly.

PMID:37923522 | DOI:10.1016/j.mayocp.2023.02.030
Well-differentiated primary adrenal angiosarcoma: A case report

Fetched: November 5th, 2023, 5:00am GMT by Bing Zhou

Asian J Surg. 2023 Nov 2:S1015-9584(23)01707-4. doi: 10.1016/j.asjsur.2023.10.094. Online ahead of print.

NO ABSTRACT

PMID:37925290 | DOI:10.1016/j.asjsur.2023.10.094
Current understanding of angiosarcoma: disease biology and evolving treatment

Fetched: November 4th, 2023, 5:00am GMT by Woo Ju Kim

Arch Craniofac Surg. 2023 Oct;24(5):203-210. doi: 10.7181/acfs.2023.00409. Epub 2023 Oct 20.

ABSTRACT

Angiosarcoma is a very rare soft tissue sarcoma that originates from endothelial cells and typically has a poor prognosis. It is most commonly found in elderly white men and can occur anywhere in the body, particularly in the head, neck, and scalp. Patients who have undergone previous radiation treatment or who have chronic lymphedema also face an elevated risk of this condition. Various genetic changes are suspected to contribute to the development of angiosarcoma, and these changes have been identified as potential targets for treatment. For localized disease, wide surgical resection is often the prudent course of action. A multidisciplinary approach, which may include surgery, radiotherapy, systemic chemotherapy, or immunotherapy, is typically the most effective way to achieve favorable outcomes. In this review, we discuss the general understanding of angiosarcoma and its management, with a particular focus on the current evolving treatments for the disease.

PMID:37919906 | PMC:PMC10622948 | DOI:10.7181/acfs.2023.00409
Primary Cardiac Epithelioid Angiosarcoma with Sustained Remission following Surgical Resection: A Case Report

Fetched: October 31st, 2023, 5:01am GMT by May Myint Thanda Kyaw

Case Rep Oncol. 2023 Sep 12;16(1):878-885. doi: 10.1159/000530113. eCollection 2023 Jan-Dec.

ABSTRACT

Epithelioid angiosarcoma is an extremely rare subtype of cardiac angiosarcoma that is highly aggressive and associated with poor prognosis. Due to its rare nature, the epidemiology and pathogenesis of this disease are not well-known. Thus, effective diagnostic and treatment modalities are limited. Here, we report a case of a primary epithelioid angiosarcoma in a patient who was treated successfully with surgical resection. A 45-year-old woman who initially presented with chronic systemic symptoms and severe anemia and subsequently developed new-onset atrial fibrillation with rapid ventricular rate was found to have a right-sided cardiac mass with a large pericardial effusion. Several years prior to presentation, she was treated for localized papillary thyroid cancer with Gc protein-derived macrophage-activating factor (Gc-MAF) therapy after declining thyroidectomy. After initial workup of her cardiac mass, including a transthoracic echocardiogram and cardiac MRI, she was transferred to an outside hospital where her mass was surgically resected. She was found to have stage IIIA high-grade epithelioid angiosarcoma involving the inferior vena cava, right atrium, and pericardium. She subsequently had complete resolution of her pericardial effusion and anemia and continues to have good performance status 16 months after her excellent surgical outcome without evidence of recurrence. This unique case contributes to our knowledge of epithelioid cardiac angiosarcoma, of which limited number of cases has been reported. It highlights a favorable outcome following surgical resection of a rare, life-threatening primary cardiac tumor.

PMID:37900857 | PMC:PMC10601732 | DOI:10.1159/000530113
Cutaneous Radiation-Associated Angiosarcoma After Treatment of Carcinoma Penis: First Report from Tertiary Cancer Centre of North India

Fetched: October 31st, 2023, 5:01am GMT by Sarbartha Kumar Pratihar

Indian J Surg Oncol. 2023 Sep;14(3):556-560. doi: 10.1007/s13193-023-01766-0. Epub 2023 May 17.

ABSTRACT

Cutaneous radiation-associated angiosarcoma (cRAA) is a rare and aggressive secondary cutaneous angiosarcoma (cAS) with poor survival. cRAA has been mostly reported in breast carcinoma patients. Owing to its rarity, there is scanty literature available and no treatment guidelines. To the best of our knowledge, this is the first report of cRAA after multimodality treatment of carcinoma penis. A sixty-eight-year-old gentleman, a known case of carcinoma penis, underwent total penectomy with perineal urethrostomy and bilateral radical inguinopelvic lymph node dissection 6 years ago. He received adjuvant radiotherapy to the pelvis and bilateral groin. He presented with a bleeding plaque-like lesion with ulceration over the left lower abdomen (within previous radiation field) which rapidly progressed in size over the past 2 months. On examination, the lesion bled profusely on touch. Contrast MRI was suggestive of lobulated exophytic enhancing cutaneous lesion free from underlying muscle. Wedge biopsy was suggestive of cutaneous angiosarcoma. He underwent wide local excision with local perforator flap reconstruction from the right lower abdomen. Histopathology was suggestive of cutaneous angiosarcoma which showed immunoexpression of CD31, ERG1, cMYC suggestive of cRAA. cRAA is a very aggressive disease with 5-year survival of 15-34%. To the best of our knowledge, this is the first ever reported case of cRAA of lower abdomen after multimodality management of carcinoma penis. It masquerades with other benign and less aggressive radiation-induced skin lesions. cMYC immunoexpression is specific for secondary cAS. Wide local resection with negative margin provides the best outcome.

PMID:37900658 | PMC:PMC10611641 | DOI:10.1007/s13193-023-01766-0
Primary hepatic angiosarcoma: a clinicopathological analysis of nine cases

Fetched: October 30th, 2023, 5:00am GMT by S Zhao

Zhonghua Bing Li Xue Za Zhi. 2023 Nov 8;52(11):1132-1137. doi: 10.3760/cma.j.cn112151-20230328-00225.

ABSTRACT

Objective: To investigate the clinical manifestations, histomorphology, and differential diagnosis of primary hepatic angiosarcoma. Methods: Nine cases of primary hepatic angiosarcoma diagnosed in the Department of Pathology, the First Affiliated Hospital of Nanjing Medical University from January 2014 to December 2021 were collected, including biopsy and surgical specimens. The histomorphology, clinical, and radiologic findings were analyzed. The relevant literature was also reviewed. Results: There were six males and three females, aged 30 to 73 years (mean 57 years). Grossly, the growth pattern of the tumor was classified as either mass formation or non-mass formation (sinusoidal). Microscopically, the mass-forming primary hepatic angiosarcoma were further subdivided into vasoformative or non-vasoformative growth patterns; and those non-vasoformative tumors had either epithelioid, spindled, or undifferentiated sarcomatoid features. Sinusoidal primary hepatic angiosarcoma on the other hand presented with markedly dilated and congested blood vessels of varying sizes, with mild to moderately atypical endothelial cells. Follow-up in all nine cases revealed 8 mortality ranging from 1 to 18 months (mean 5 months) from initial diagnosis. One patient was alive with disease within a period of 48 months. Conclusions: Primary hepatic angiosarcoma is a rare entity with a wide spectrum of histomorphology, and often misdiagnosed. It should be considered when there are dilated and congested sinusoids, with overt nuclear atypia. The overall biological behavior is aggressive, and the prognosis is worse.

PMID:37899319 | DOI:10.3760/cma.j.cn112151-20230328-00225
Muscle-invasive angiosarcoma of the urinary bladder: Case report

Fetched: October 28th, 2023, 5:00am GMT by Srigita Madiraju

Urol Case Rep. 2023 Oct 17;51:102593. doi: 10.1016/j.eucr.2023.102593. eCollection 2023 Nov.

ABSTRACT

A 71-year-old male with benign prostatic hyperplasia managed by self-catheterization presented with gross hematuria. A CT scan of abdomen and pelvis demonstrated abnormal bladder appearance with right sided mass and a diverticulum. Patient underwent transurethral resection of bladder tumor. Pathology was significant for high-grade muscle-invasive angiosarcoma. The malignant cells showed positive staining for vimentin and CD31. Given patient's underlying comorbidities and following multidisciplinary discussion, hospice care was pursued. The aim of this case report is to provide an overview on clinical presentation, diagnosis, and current management of this rare entity of genitourinary sarcoma.

PMID:37886347 | PMC:PMC10598485 | DOI:10.1016/j.eucr.2023.102593
Sclerosing Angiomatoid Nodular Transformation of the Spleen: A Report of Rare Case and Literature Review

Fetched: October 20th, 2023, 5:00am GMT by Fnu Raja

Cureus. 2023 Sep 17;15(9):e45422. doi: 10.7759/cureus.45422. eCollection 2023 Sep.

ABSTRACT

Sclerosing angiomatoid nodular transformation (SANT) is a benign vascular lesion of the spleen with uncertain etiology. It predominantly affects women between the ages of 30 and 60 years. Clinically, it is asymptomatic or can cause abdominal pain, but usually discovered incidentally on imaging, which can identify a mass but may not provide a definitive diagnosis. In uncertain vascular lesions, there is always a risk of spontaneous rupture of large vessels and the potential for spreading malignancy. Hence, the final diagnosis is rendered on microscopy after splenectomy. A middle-aged female came to the clinic complaining of abdominal pain. Radiology showed a solid splenic mass and the patient underwent splenectomy. Gross examination showed a 3 cm white firm mass with focal hemorrhage. Microscopy revealed multiple nodules of variable sizes surrounded by fibrosclerotic stroma. The nodules showed round to slit-like vascular spaces with numerous red blood cells. The internodular stroma consisted of dense fibrous tissue with scattered plump myofibroblasts and lymphoplasmacytic inflammatory cells. These distinctive features lead to the diagnosis of SANT. SANT possesses characteristic histologic features with distinctive immunohistochemistry (IHC). IHC reveals three different types of vessels within the nodules as follows: (1) small veins (CD34^-, CD31⁺, CD8^-), (2) sinusoids (CD34^-, CD31⁺, CD8⁺), and (3) capillaries (CD34⁺, CD31⁺, CD8^-). All three types of vessels are negative for CD21/CD35 and CD68. Hemangioma and littoral cell angioma are two frequent vascular tumors in the spleen that should be considered differential diagnoses. Both lesions lack the microscopic features of SANT and have only a single type of vessel. The vessels in hemangioma are (CD31⁺, CD34⁺, CD8^-), while in littoral cell angioma they are (CD31⁺, CD34^-, CD8^-, CD21⁺, CD68⁺). There are no specific clinical or radiologic findings for SANT. It is important to recognize these characteristic features and to differentiate them from other benign and malignant lesions, such as angiosarcoma. A thorough histopathologic examination and IHC are helpful in making the correct diagnosis.

PMID:37854759 | PMC:PMC10581663 | DOI:10.7759/cureus.45422
Clinical features and prognosis of retroperitoneal hemangiosarcoma in dogs with surgical resection with or without adjuvant doxorubicin

Fetched: October 20th, 2023, 5:00am GMT by Masanao Ichimata

J Vet Med Sci. 2023 Nov 18;85(11):1231-1236. doi: 10.1292/jvms.22-0533. Epub 2023 Oct 17.

ABSTRACT

Retroperitoneal hemangiosarcoma (RPHSA) is a rare tumor in dogs with a poorly understood prognosis after surgery. The objectives of this study were to investigate the clinical features and prognosis of canine RPHSA that had undergone surgical resection. In this single-center, retrospective cohort study, we reviewed the medical records of dogs that had undergone surgical resection for retroperitoneal tumors and received a histopathologic diagnosis of HSA between 2005 and 2021. The median progression-free survival (PFS) and overall survival (OS) were 77.5 days and 168 days, respectively. In the present study, canine RPHSA had an aggressive biological behavior similar to visceral HSA. Further studies in larger canine populations are needed to evaluate the efficacy of adjuvant chemotherapy.

PMID:37853623 | PMC:PMC10686775 | DOI:10.1292/jvms.22-0533
Treating rare tumors with liver transplantation

Fetched: October 19th, 2023, 5:01am GMT by Andrew Melehy

Curr Opin Organ Transplant. 2023 Oct 19. doi: 10.1097/MOT.0000000000001118. Online ahead of print.

ABSTRACT

PURPOSE OF REVIEW: The success of liver transplantation (LT) in treating unresectable hepatocellular carcinoma (HCC) has resulted in interest in LT for other oncologic conditions. Here, we discuss the role of LT for rare oncologic indications including metastatic gastroenteropancreatic neuroendocrine tumors (GEP-NETs), hepatic epitheliod hemangioendothelioma (HEHE), fibrolamellar hepatocellular carcinoma (FLC), and hepatic angiosarcoma (HAS).

RECENT FINDINGS: Conditions reviewed have been documented indications for LT in the available literature. We summarize the experience of LT for these indications and proposed management guidelines.

SUMMARY: GEP-NETs with isolated metastases to the liver can be treated with LT with excellent long-term outcomes (10-year survival 88%) if strict selection criteria are used (low-intermediate grade, Ki-67% < 20%, complete resection of primary tumor, stable disease for 6 months, <50% hepatic involvement). HEHE is a rare hepatic tumor for which LT can be performed with reported 10-year survival around 70%. FLC is a distinct clinical entity to HCC and is optimally treated with surgical resection though experience with LT is described in observational series (5-year survival 50%, recurrence in 10%). HAS is a rapidly progressive tumor with a dismal prognosis with or without treatment, including LT.

PMID:37851086 | DOI:10.1097/MOT.0000000000001118
Spectrum of malignant scalp tumours and its impact on management-a tertiary care cancer centre experience

Fetched: October 17th, 2023, 5:00am GMT by Bipin T Varghese

World J Surg Oncol. 2023 Oct 17;21(1):330. doi: 10.1186/s12957-023-03200-9.

ABSTRACT

BACKGROUND: Tumours on the scalp are diverse and often exhibit site- and histology-specific characteristics. Reconstructing the scalp after oncological resection has always been challenging because of its unique anatomy.

METHODOLOGY: A retrospective review of patients with malignant scalp tumour operated on at a single institution over 10 years was performed. Data were collected and analysed regarding the scalp tumour profile, treatment, and the outcome of these procedures.

RESULTS: Of the 66 patients in our study, 33 (50%) had SCC. In addition to this, 21% were sarcomas, 17% were appendageal carcinomas, 11% were BCCs, and 1% was neuroendocrine carcinoma. Cortical erosion was observed in 6 patients in the CT imaging, all with SCC histology. Among the eight patients with pathological nodal involvement, three had angiosarcoma, three had SCC, one had appendageal carcinoma, and one had neuroendocrine carcinoma. The mean surgical defect size was 67.4 cm². The surgical defect was reconstructed with local flaps in 58% of patients and primary closure in 27%. Local and systemic recurrence was noted in 25% of patients. Tumour size more than 6 cm, tumour histology (SCC & sarcoma), unplanned margin-positive excision, and residual disease in re-excision had higher recurrence, even though the p-value was not significant.

CONCLUSION: Scalp tumours are heterogeneous in their clinical profiles. Often, its tumour biology and microscopic extent are underestimated. High suspicion, histological diagnosis, and clear surgical margins are all requirements in successfully treating scalp tumours. In order to minimize morbidity and restore an aesthetic and functional outcome, it is critical to use the simplest scalp reconstruction whenever possible.

PMID:37845728 | PMC:PMC10580575 | DOI:10.1186/s12957-023-03200-9
Cutaneous angiosarcoma of the scalp in a pediatric patient with xeroderma pigmentosum

Fetched: October 17th, 2023, 5:00am GMT by Valeria S Oliver-García

JAAD Case Rep. 2023 Sep 7;41:37-39. doi: 10.1016/j.jdcr.2023.08.039. eCollection 2023 Nov.

NO ABSTRACT

PMID:37842153 | PMC:PMC10568230 | DOI:10.1016/j.jdcr.2023.08.039
Primary Cardiac Angiosarcoma in a Patient with Syncope: A Case Report

Fetched: October 17th, 2023, 5:00am GMT by Bo Eun Park

Chonnam Med J. 2023 Sep;59(3):211-212. doi: 10.4068/cmj.2023.59.3.211. Epub 2023 Sep 25.

NO ABSTRACT

PMID:37840675 | PMC:PMC10570853 | DOI:10.4068/cmj.2023.59.3.211
A case of a motor vehicle collision suspected as associated with development of angiosarcoma

Fetched: October 15th, 2023, 5:00am GMT by Haruka Ikeda

Leg Med (Tokyo). 2023 Oct 9:102332. doi: 10.1016/j.legalmed.2023.102332. Online ahead of print.

ABSTRACT

Trauma has been suspected as a factor leading to development of angiosarcoma, a malignant soft-tissue tumor. We conducted a forensic autopsy to investigate a putative relation between a motor vehicle collision and the driver's later death from angiosarcoma. A vehicle operated by a man in his 60 s collided with an oncoming vehicle at a curve. The victim noticed no injury at the scene. However, 45 days later, he was transferred to an emergency room with dyspnea and bloody sputum. After diagnosis of angiosarcoma, he died of respiratory failure 132 days later. The bereaved family speculated about a relation between the collision and angiosarcoma onset. At autopsy, tumor cells of the scalp had metastasized to the lung, pleura, liver, and spleen. Histopathological examinations revealed characteristic features of angiosarcoma with positive immune-staining for CD31, CD34, and factor VIII. When a person dies some time after a collision, it is designated as a delayed death. In such cases, the relevance of trauma to the person's death is often an issue of concern. Because the interval between trauma and angiosarcoma development was short, only 45 days, the angiosarcoma might be coincidental. Therefore, we rejected the relation. Forensic experts sometimes need to investigate such inquiries.

PMID:37833210 | DOI:10.1016/j.legalmed.2023.102332
Lymphedema-associated angiosarcoma uniquely localized on the left thigh and anterior abdominal wall secondary to central obesity and multiple surgeries

Fetched: October 14th, 2023, 5:00am GMT by Yui Hirano-Lotman

J Dermatol. 2023 Oct 13. doi: 10.1111/1346-8138.17005. Online ahead of print.

NO ABSTRACT

PMID:37830441 | DOI:10.1111/1346-8138.17005
Clinical and Imaging Features of MRI Screen-Detected Breast Cancer

Fetched: October 12th, 2023, 5:00am GMT by Naveen Ghuman

Clin Breast Cancer. 2023 Sep 22:S1526-8209(23)00253-7. doi: 10.1016/j.clbc.2023.09.012. Online ahead of print.

ABSTRACT

BACKGROUND: Supplemental screening with breast MRI is recommended annually for patients who have greater than 20% lifetime risk for breast cancer. While there is robust data regarding features of mammographic screen-detected breast cancers, there is limited data regarding MRI-screen-detected cancers.

PATIENTS AND METHODS: Screening breast MRIs performed between August 1, 2016 and July 30, 2022 identified 50 screen-detected breast cancers in 47 patients. Clinical and imaging features of all eligible cancers were recorded.

RESULTS: During the study period, 50 MRI-screen detected cancers were identified in 47 patients. The majority of MRI-screen detected cancers (32/50, 64%) were invasive. Pathology revealed ductal carcinoma in situ (DCIS) in 36% (18/50), invasive ductal carcinoma (IDC) in 52% (26/50), invasive lobular carcinoma in 10% (5/50), and angiosarcoma in 2% (1/50). The majority of patients (43/47, 91%) were stage 0 or 1 at diagnosis and there were no breast cancer-related deaths during the follow-up periods. Cancers presented as masses in 50% (25/50), nonmass enhancement in 48% (25/50), and a focus in 2% (1/50). DCIS was more likely to present as nonmass enhancement (94.4%, 17/18), whereas invasive cancers were more likely to present as masses (75%, 24/32) (P < .001). All cancers that were stage 2 at diagnosis were detected either on a baseline exam or more than 4 years since the prior MRI exam.

CONCLUSION: MRI screen-detected breast cancers were most often invasive cancers. Cancers detected by MRI screening had an excellent prognosis in our study population. Invasive cancers most commonly presented as a mass.

PMID:37821332 | DOI:10.1016/j.clbc.2023.09.012
Bright Is Not Always Better: A Pictorial Review of Hyperechoic Malignant Breast Masses

Fetched: October 10th, 2023, 5:01am GMT by Shivya Parashar

Indian J Radiol Imaging. 2023 May 6;33(4):532-540. doi: 10.1055/s-0043-1768641. eCollection 2023 Oct.

ABSTRACT

Hyperechogenic breast lesions are a relatively rare finding at breast ultrasonography and are traditionally thought to be benign. However, hyperechogenicity on the ultrasound alone does not provide enough evidence to rule out malignancy completely. We herein reported a short series of nine cases of echogenic malignant breast lesions, which include invasive ductal carcinoma, ductal carcinoma in situ, invasive lobular carcinoma, angiosarcoma, lymphoma, and metastasis to the breast. Echogenic breast lesions should be carefully evaluated and properly categorized based on any other suspicious sonographic characteristics and must be correlated with mammographic findings and clinical history to lower the threshold for biopsy and avoid delay in diagnosis. Hyperechogenicity should not be considered as a characteristically benign feature and should not supersede the less specifically benign features of the same lesion on the other examination.

PMID:37811173 | PMC:PMC10556334 | DOI:10.1055/s-0043-1768641
Do Not Forget the Oncovascular: Acute Limb Ischaemia Due to Aortic Epithelioid Angiosarcoma

Fetched: October 10th, 2023, 5:01am GMT by Pierre Kara

EJVES Vasc Forum. 2023 Sep 15;60:53-56. doi: 10.1016/j.ejvsvf.2023.09.003. eCollection 2023.

ABSTRACT

INTRODUCTION: Aortic epithelioid angiosarcoma (AEA) is a rare malignant tumour and can cause acute limb ischaemia.

REPORT: A 66 year old man was admitted with acute pulmonary oedema due to bilateral renal artery stenosis. An incidental osteolytic left sacral lesion was found on computed tomography angiography, and extensive work up revealed an AEA. Follow up was marked by acute left lower limb ischaemia 13 months later and right chronic limb threatening ischaemia 15 months later.

DISCUSSION: Physicians need to consider AEA as an aetiology for acute or chronic limb ischaemia in patients with altered general status but mostly with intra-aortic irregular vegetations without any calcification and parietal involvement on computed tomography angiography.

PMID:37811160 | PMC:PMC10551559 | DOI:10.1016/j.ejvsvf.2023.09.003
Primary cerebral angiosarcoma: a case report

Fetched: October 10th, 2023, 5:01am GMT by Hasan R Arafat

Ann Med Surg (Lond). 2023 Aug 14;85(10):5127-5132. doi: 10.1097/MS9.0000000000001158. eCollection 2023 Oct.

ABSTRACT

INTRODUCTION AND IMPORTANCE: Central nervous system sarcomas are rare tumors of mesenchymal origin. Angiosarcomas are an even rarer subtype with poor prognosis and no consensus regarding therapeutic approach.

CASE PRESENTATION: This article presents the case of a 50-year-old Palestinian female patient with a history of treated breast cancer who presented to the emergency room with a tonic-clonic seizure. Brain computed tomography showed a cerebral space-occupying lesion managed with craniotomy and gross tumor resection. Histopathology revealed an epithelioid cerebral angiosarcoma. The patient was treated with concurrent chemoradiotherapy with temozolomide resulting in the resolution of the tumor as well as symptoms and complaints.

CLINICAL DISCUSSION: Primary cerebral epithelioid angiosarcoma is a highly malignant tumor of an unknown etiology. The patient history of breast created an additional challenge, as recurrence with metastasis had to be excluded. Signs of blood product degradation are an important radiological feature. According to existing literature, gross tumor resection followed by concurrent chemoradiotherapy offers the best approach and outcome.

CONCLUSION: Cerebral angiosarcoma is a rare disease with a challenging therapeutic approach due to the scarcity of available literature. It should be included in the differential diagnosis of space-occupying lesions, especially in those with a history of exposure to radiotherapy. Immunohistochemistry is key for diagnosis. Surgical resection followed by concurrent chemoradiotherapy is associated with a longer disease-free survival when compared to either option alone.

PMID:37811120 | PMC:PMC10553031 | DOI:10.1097/MS9.0000000000001158
Radiation-induced angiosarcoma of the breast: a case report

Fetched: October 10th, 2023, 5:01am GMT by Chafik Rhoul

Ann Med Surg (Lond). 2023 Sep 4;85(10):5047-5050. doi: 10.1097/MS9.0000000000001141. eCollection 2023 Oct.

ABSTRACT

INTRODUCTION: Breast angiosarcoma is a rare, aggressive tumour affecting adult women. It can occur in two forms, primary form and secondary forms or radiation-induced breast angiosarcoma affecting patients with history of breast or chest radiotherapy.

CASE PRESENTATION: The authors report a new case of breast angiosarcoma in 52-year-old women, with history of invasive ductal carcinoma, and reporting a discoloration of her breast skin. The patient did undergo a mastectomy of right breast and adjuvant chemotherapy.

CONCLUSION/DISCUSSION: Surgery with total excision associated or not to adjuvant chemotherapy remains the treatment of choice in breast angiosarcoma.

PMID:37811031 | PMC:PMC10553051 | DOI:10.1097/MS9.0000000000001141
Radiation-Associated Angiosarcoma as a Presentation of Disease Progression in a Patient on Immunotherapy for Metastatic Non-small Cell Lung Cancer

Fetched: October 10th, 2023, 5:01am GMT by Hannah Tan

Cureus. 2023 Sep 5;15(9):e44724. doi: 10.7759/cureus.44724. eCollection 2023 Sep.

ABSTRACT

Non-small cell lung cancer (NSCLC) is the dominant form of lung cancer, comprising around 85% of cases. Stage 4 NSCLC has a grim prognosis; however, immunotherapy and radiation therapy have become vital treatments for advanced-stage NSCLC, despite the risk of inducing a second primary malignancy. This case report focuses on a 45-year-old female diagnosed with NSCLC and metastasis to the 11th thoracic vertebral body. After various treatments, including radiation, a potential radiation-associated secondary malignancy, epithelial angiosarcoma, was discovered. Following treatment modification, the patient achieved complete metabolic remission, highlighting the importance of clinicians being cautious about secondary primary cancers in NSCLC patients with a history of radiation therapy. Accurate diagnosis through biopsy and continuous surveillance are essential in managing NSCLC patients effectively.

PMID:37809227 | PMC:PMC10552933 | DOI:10.7759/cureus.44724
Target gene regulatory network of miR-497 in angiosarcoma

Fetched: October 10th, 2023, 5:01am GMT by Annaleigh Benton

bioRxiv. 2023 Sep 25:2023.09.24.559218. doi: 10.1101/2023.09.24.559218. Preprint.

ABSTRACT

Angiosarcoma (AS) is a vascular sarcoma that is highly aggressive and metastatic. Due to its rarity, treatment options for patients are limited, therefore more research is needed to identify possible therapeutic vulnerabilities. We previously found that conditional deletion of Dicer1 drives AS development in mice. Given the role of DICER1 in canonical microRNA (miRNA) biogenesis, this suggests that miRNA loss is important in AS development. After testing miRNAs previously suggested to have a tumor-suppressive role in AS, microRNA-497-5p (miR-497) suppressed cell viability most significantly. We also found that miR-497 overexpression led to significantly reduced cell migration and tumor formation. To understand the mechanism of miR-497 in tumor suppression, we identified clinically relevant target genes using a combination of RNA-sequencing data in an AS cell line, expression data from AS patients, and target prediction algorithms. We validated miR-497 direct regulation of CCND2, CDK6, and VAT1. One of these genes, VAT1, is an understudied protein that has been suggested to promote cell migration and metastasis in other cancers. Indeed, we find that pharmacologic inhibition of VAT1 with the natural product Neocarzilin A reduces AS migration. This work provides insight into the mechanisms of miR-497 and its target genes in AS pathogenesis.

PMID:37808715 | PMC:PMC10557590 | DOI:10.1101/2023.09.24.559218
Cytopathology of mesenchymal tumors of the thyroid gland: analysis of 12 cases from multinational institutions with review of the literature

Fetched: October 9th, 2023, 5:01am GMT by Carla Saoud

J Am Soc Cytopathol. 2023 Nov-Dec;12(6):436-450. doi: 10.1016/j.jasc.2023.09.004. Epub 2023 Sep 13.

ABSTRACT

INTRODUCTION: Mesenchymal tumors of the thyroid gland are extremely rare. We report the cytomorphologic characteristics of 12 mesenchymal tumors occurring in the thyroid gland and highlight the diagnostic difficulties encountered in their cytologic evaluation.

MATERIALS AND METHODS: The cytopathology and surgical pathology archives from 5 large institutions were searched for thyroid mesenchymal tumors that had an FNA available for review. Clinicopathologic and cytomorphologic characteristics for each case were evaluated.

RESULTS: Twelve cases of mesenchymal tumors occurring in the thyroid were identified in our search. Patient age ranged from 28 to 84 years (median, 60 years). The cases occurred in 7 women and 5 men. The tumor size ranged from 1.4 to 14 cm (median, 3.3 cm). The tumors were as follows: hemangioma (n = 4; 33.3%), angiosarcoma (n = 2; 16.7%), schwannoma (n = 2; 16.7%), solitary fibrous tumor (n = 2, 16.7%), metastatic synovial sarcoma (n = 1, 8.3%) and metastatic pleomorphic rhabdomyosarcoma (n = 1, 8.3%). The cytomorphologic features of the tumors were similar to those of their counterparts occurring in different sites. An accurate diagnosis was achieved in six primary thyroid mesenchymal cases (60%). Five patients (41.7%) underwent total thyroidectomy, and 3 patients received partial thyroidectomy (25%). Three patients (25%) did not receive a thyroidectomy and subsequent surgical information was not available in 1 case (8.3%).

CONCLUSIONS: Mesenchymal tumors of the thyroid are extremely uncommon. Cytologic diagnosis of these tumors is often challenging due to the morphologic overlap with diverse epithelial and non-epithelial thyroid lesions. Ancillary studies such as immunohistochemistry and molecular studies are essential for accurate diagnosis.

PMID:37806808 | DOI:10.1016/j.jasc.2023.09.004
Intensity-modulated radiotherapy and chemotherapy for canine right atrial tumors: A retrospective study of seven dogs

Fetched: October 7th, 2023, 5:01am GMT by Steven Moirano

Vet Radiol Ultrasound. 2023 Oct 6. doi: 10.1111/vru.13303. Online ahead of print.

ABSTRACT

Most primary cardiac tumors in dogs are located in the right atrium/atrial appendage, with hemangiosarcoma being the most common. The aims of this retrospective, case series were to describe outcomes for seven dogs with right atrial tumors treated with hypofractionated intensity-modulated radiotherapy and concurrent vinblastine and propranolol. One dog had a complete response, four dogs had partial responses and two dogs had stable disease after treatment. Effusions resolved in all dogs. Median progression-free survival was 290 days. Five dogs died from metastatic disease, one dog from unrelated neoplasia, and one dog is alive. Median overall survival was 326 days. Three dogs with confirmed hemangiosarcoma survived 244, 326, and 445 days. Two dogs developed clinically significant, but nonfatal, cardiac arrhythmias. One dog that received three courses of radiation had subclinical myocardial and arterial fibrosis at necropsy. Hypofractionated chemoradiotherapy was well tolerated and may provide clinical benefit in dogs with right atrial tumors.

PMID:37800663 | DOI:10.1111/vru.13303
Intimal angiosarcoma of the descending aorta presenting as atheroembolism: case report and description of intravascular biopsy

Fetched: October 7th, 2023, 5:01am GMT by Alejandra Bartolomé Sánchez

J Vasc Surg Cases Innov Tech. 2023 May 24;9(3):101230. doi: 10.1016/j.jvscit.2023.101230. eCollection 2023 Sep.

ABSTRACT

Aortic sarcoma is a rare entity. In most cases, the diagnosis is established late, owing to the course of the disease, with a median survival time of only a few months. We report the case of a 58-year-old patient with ischemic lesions in the lower limb. The lesions after several investigations were diagnosed by imaging studies (eg, magnetic resonance angiography, contrast-enhanced computed tomography [CT], CT angiography, or positron emission tomography-CT with fluorine-18 fluorodeoxyglucose) as possible primary angiosarcoma of the aorta. It was decided to perform endovascular aortic repair and endovascular biopsy of the lesion. This was chosen as a "palliative" treatment to avoid embolic events, given the presence of metastases, instead of open surgery, which would otherwise have been the treatment of choice. Subsequently, histologic examination confirmed aortic intimal angiosarcoma, and adjuvant chemotherapy was initiated. In the present report, we discuss the clinical presentation, diagnosis, and classification of primary aortic sarcomas. We also critically review the diagnostic and therapeutic management of these patients in previous series of studies to improve their treatment in subsequent cases.

PMID:37799831 | PMC:PMC10547730 | DOI:10.1016/j.jvscit.2023.101230
Primary sarcoma of the liver: A nationwide analysis of a rare mesenchymal tumor

Fetched: October 6th, 2023, 5:01am GMT by Nicole Finney

J Surg Oncol. 2023 Oct 5. doi: 10.1002/jso.27477. Online ahead of print.

ABSTRACT

BACKGROUND AND OBJECTIVES: Primary liver sarcomas are rare malignancies. Prognostic factors associated with long-term survival remain poorly understood. The objective of this study is to determine factors associated with long-term survival.

METHODS: The Surveillance, Epidemiology, and End Results (SEER) database was queried to identify patients with visceral sarcoma arising from the liver. Demographic factors, tumor characteristics, resection status, and survival were evaluated. Multivariate Cox regression analysis was performed to determine predictors of survival.

RESULTS: A total of 743 patients with primary hepatic sarcoma were identified. The median tumor size was 10 cm. Only 30% (n = 221) of patients in the cohort underwent surgery. The 5-year overall survival rates were 47.9% for localized disease, 29.5% for regional disease, and 16.5% for distant disease, p < 0.001. Among patients who underwent surgical resection, patients with embryonal sarcoma had better 5-year survival compared with angiosarcoma and other histologic subtypes. On multivariate analysis, surgery was associated with improved survival, while older age, higher stage, and angiosarcoma histology were the strongest independent predictors of poor survival.

CONCLUSIONS: Surgery remains the mainstay of treatment for this rare malignancy but is performed in less than one-third of patients. Angiosarcoma histology is associated with worse overall survival, while surgical resection remains the strongest predictor of improved overall survival.

PMID:37796036 | DOI:10.1002/jso.27477
Safety and clinical efficacy of an anti-PD-L1 antibody (c4G12) in dogs with advanced malignant tumours

Fetched: October 5th, 2023, 5:00am GMT by Naoya Maekawa

PLoS One. 2023 Oct 4;18(10):e0291727. doi: 10.1371/journal.pone.0291727. eCollection 2023.

ABSTRACT

Immune checkpoint inhibitors (ICIs) have been developed for canine tumour treatment, and pilot clinical studies have demonstrated their antitumour efficacy in dogs with oral malignant melanoma (OMM). Although ICIs have been approved for various human malignancies, their clinical benefits in other tumour types remain to be elucidated in dogs. Here, we conducted a clinical study of c4G12, a canine chimeric anti-PD-L1 antibody, to assess its safety and efficacy in dogs with various advanced malignant tumours (n = 12) at the Veterinary Teaching Hospital of Hokkaido University from 2018 to 2023. Dogs with digit or foot pad malignant melanoma (n = 4), osteosarcoma (n = 2), hemangiosarcoma (n = 1), transitional cell carcinoma (n = 1), nasal adenocarcinoma (n = 1), B-cell lymphoma (n = 1), or undifferentiated sarcoma (n = 2) were treated with 2 or 5 mg/kg c4G12 every 2 weeks. Treatment-related adverse events of any grade were observed in eight dogs (66.7%), including elevated aspartate aminotransferase (grade 3) in one dog (8.3%) and thrombocytopenia (grade 4) in another dog (8.3%). Among dogs with target disease at baseline (n = 8), as defined by the response evaluation criteria for solid tumours in dogs (cRECIST), one dog with nasal adenocarcinoma and another with osteosarcoma experienced a partial response (PR), with an objective response rate of 25.0% (2 PR out of 8 dogs; 95% confidence interval: 3.2-65.1%). These results suggest that c4G12 is safe and tolerable and shows antitumor effects in dogs with malignant tumours other than OMM. Further clinical studies are warranted to identify the tumour types that are most likely to benefit from c4G12 treatment.

PMID:37792729 | PMC:PMC10550157 | DOI:10.1371/journal.pone.0291727
Surgical Treatment for Benign Lymphangioendothelioma After Two Incomplete Excisions: A Case Report and Literature Review

Fetched: October 5th, 2023, 5:00am GMT by Wei Lu

Clin Cosmet Investig Dermatol. 2023 Sep 28;16:2697-2719. doi: 10.2147/CCID.S420019. eCollection 2023.

ABSTRACT

Benign lymphangioendothelioma (BL) is a rare, poorly identified, slow-growing benign vascular lesion characterized by asymptomatic, solitary, well-demarcated macules, or by mildly infiltrated plaque. We report a case of an atypical BL that arose as a tender, protuberant, flesh-colored mass with cyanotic vesicles, and then progressed to a persistent exudative wound after two incomplete excisions. The patient was also diagnosed with thoracic duct narrowing. Although the stenosis was removed by surgery, the right lower extremity ulceration and exudation did not improve. Thus, we performed a thorough excision and split-thickness skin graft transplant following vacuum sealing drainage, and eventually the patient had a favorable functional and cosmetic outcome. A biopsy revealed irregular, dilated vascular spaces lined with a single layer of flat endothelial cells extending from the superficial dermis to the subcutis that did not reach the striated muscles. Additionally, by reviewing the literature on BL, in this paper we summarize the diverse pathogenic, morphological, and immunohistochemical presentations for this rare disease, as well as the histopathological differential diagnosis of lymphangiomatosis, Kaposi's sarcoma, and angiosarcoma.

PMID:37790904 | PMC:PMC10544170 | DOI:10.2147/CCID.S420019
Scalp Volumetric Modulated Arc Therapy Using 3D Milled Bolus: Dosimetry, Toxicity and Outcome

Fetched: October 4th, 2023, 5:01am GMT by K Dibs

Int J Radiat Oncol Biol Phys. 2023 Oct 1;117(2S):e292-e293. doi: 10.1016/j.ijrobp.2023.06.1289.

ABSTRACT

PURPOSE/OBJECTIVE(S): Radiotherapy for scalp lesions is frequently challenging due to the competing needs for target volume coverage, sparing of numerous critical nearby OARs, and reproducible bolus setup despite the irregular and highly convex scalp geometry. Here, we evaluate the use of 3D milled bolus in treating such tumors.

MATERIALS/METHODS: A retrospective analysis of scalp patients (pts) treated between 2016-2022 using 3D milled bolus and VMAT. All pts were treated with 6 MV photon beams, IGRT via daily CBCT, and 6-degree of Freedom (6-DOF) couch tops. Pts demographics, tumor characteristics, DVHs, toxicities and outcomes were evaluated. Regional control (RC) defined as control of the disease in the rest of the scalp/neck and local control (LC) defined as disease control within the PTV.

RESULTS: A total of 23 pts were identified. Median age 74 (46-85), ECOG performance status was 0-1 in 20 pts (87%) and 92% were males. The histopathologies were squamous cell carcinoma (SCC) (61%), angiosarcoma (AS) (35%) and melanoma (4%). 35% of pts were treated with definitive intent to gross disease; the remaining 65% were treated post-operatively, 22% with microscopically positive margins and 43% with negative margins. 21% had perineural invasion (PNI), and none had lymphovascular invasion (LVI). 78% had T3/T4 and 13% had N+ disease. Median radiation dose was 66 Gy (60-69.96Gy). 44% received concurrent systemic therapy (Paclitaxel 22%, Cemiplimab 12%, Temozolomide 5%, Nivolumab 5%). In 40% of pts, the whole scalp was treated; in the remaining 60%, the median ratio of PTV volume to scalp volume was 35% (25-90%). 22% of pts (n = 5) had neck irradiation for prophylactic (3pts)/ neck lymph node involvement (2 pts). The median brain Dmax was 65 Gy (52.5-71.9), median brain mean dose was 15.4Gy (1.4-38.4), median eye Dmax was 10.9Gy (0.23-49), median cochlea mean was 8.4Gy (0.1-20.4), median lacrimal gland mean dose was 6.8Gy (0.11-38), median hippocampus Dmin was 5.9Gy (0.1-14.9) and Dmax was 10Gy (0.3-23). Most common acute side effects were grade 1-2 fatigue (100%), grade 1-2 pain (78%), grade 1 dysgeusia (22%), grade 1-2 dermatitis (74%). The only grade > = 3 adverse event was grade 3 dermatitis in 26%. Regarding long-term side effects, one pt had grade 2 osteoradionecrosis and other one developed grade 2 skin ulcer. 17% developed grade 1 memory impairment. 17% had grade 1 eye dryness. None developed brain radionecrosis. At a median follow-up was 14.4 months (1-73.5), the 1-yr LC was 96%. The 1-yr RC was 75% overall, 100% in angiosarcoma vs 62% in SCC (p = 0.18). Of the 4 regional recurrences, 1 pt had marginal recurrence along V1, 2 had neck nodal recurrence, and 1 had recurrence elsewhere in the scalp. 1-yr DMFS was 62% overall. 1-yr OS was 78% overall, 100% for AS vs 64% for SCC (P = 0.097).

CONCLUSION: VMAT planning with 3D milled bolus permits technically sound radiotherapy for scalp targets with an acceptable toxicity profile and relatively favorable clinical outcomes. This approach warrants further evaluation in a larger prospective study.

PMID:37785077 | DOI:10.1016/j.ijrobp.2023.06.1289
$Permalink for 'Hyperfractionated and Accelerated Reirradiation for Angiosarcoma Following Breast-Conserving Therapy: Long-Term Clinical Outcomes'$
Hyperfractionated and Accelerated Reirradiation for Angiosarcoma Following Breast-Conserving Therapy: Long-Term Clinical Outcomes

Fetched: October 4th, 2023, 5:01am GMT by A C Casper

Int J Radiat Oncol Biol Phys. 2023 Oct 1;117(2S):e167. doi: 10.1016/j.ijrobp.2023.06.1004.

ABSTRACT

PURPOSE/OBJECTIVE(S): Radiation-associated angiosarcoma (RAAS) of the breast is a highly aggressive tumor that can occur in women who have undergone breast conserving therapy (BCT). Due to the rare nature of the disease, no consensus exists regarding the optimum management. Historically, local control and disease-specific survival rates have been poor. In our study, we evaluate the long-term outcomes of a novel approach to treatment of this disease using hyperfractionated and accelerated reirradiation (HART). The fractionation scheme was motivated by the idea that rapidly-growing tumors would benefit from hyperfractionation and previously irradiated tissues would handle smaller doses per fraction better.

MATERIALS/METHODS: With IRB approval, charts were retrospectively reviewed and follow-up was obtained for 15 patients treated with HART for angiosarcoma of the breast which developed after BCT between November 1997 and April 2017. Mean time to diagnosis of RAAS after BCT was 7.6 years (range, 4.3 - 11.0). Seven patients were treated for recurrent angiosarcoma after initial surgical resection and 8 were treated for de novo angiosarcoma. Patients were treated to a median of 60 Gy (range, 43-75) using two (n = 2) or three (n = 13) daily treatments. Mean time to beginning of HART after the diagnosis of angiosarcoma was 1.91 months (0.13-8.84).

RESULTS: Median follow-up time was 137 months (range, 4.8 - 235.3). Seven patients were alive at the time of follow-up. Fifteen-year cause-specific survival and overall survival rates were 67% (95% CI: 43 - 89%) and 53% (95% CI: 29 - 76%), respectively. Six patients had progression of their disease after HART, none of which were in the radiation field. Two of these patients had evidence of metastatic disease before treatment began. Effects from radiation included skin, musculoskeletal, nerve, and lung toxicities, most of which resolved or were not clinically significant.

CONCLUSION: This suggests that HART can provide relatively high rates of local control and overall survival for RAAS after BCT. Additionally, it highlights the need for multi-institution trials regarding treatment with HART in this disease. To our knowledge, this study contains the longest follow-up available for patients with RAAS of the breast.

PMID:37784769 | DOI:10.1016/j.ijrobp.2023.06.1004
Dawn of Staging for Head and Neck Soft Tissue Sarcoma: Validation of the Novel 8th Edition AJCC T Classification and Proposed Stage Groupings

Fetched: October 4th, 2023, 5:01am GMT by R R Salunkhe

Int J Radiat Oncol Biol Phys. 2023 Oct 1;117(2S):S149. doi: 10.1016/j.ijrobp.2023.06.567.

ABSTRACT

PURPOSE/OBJECTIVE(S): After decades of stagnation, the 8^th edition TNM (TNM8) introduced a new T classification for head and neck (HN) soft tissue sarcomas (STS). New size cutoffs of 2 and 4 cm define T1-3, and a novel T4 category is defined by local invasion of adjoining structures. These size cutoffs had been chosen arbitrarily to advance data collection in this unique disease site since literature showed approximately 70% of HN STS did not reach the previous size threshold (5 cm) for the existing T1 category. The definition of the TNM8 T categories also align with mucosal HN cancers. No stage grouping for HN STS was defined since this new classification required more data collection to derive stage groups. This study aims to validate the TNM8 T classification and to propose stage groupings.

MATERIALS/METHODS: Clinical data of all adult (>16 years) HN STS patients treated from 1988 - 2019 with curative intent in our tertiary cancer center were retrieved from a prospective database, and supplemented with chart review. As per TNM8, cutaneous angiosarcoma, embryonal and alveolar rhabdomyosarcoma, Kaposi sarcoma, and dermatofibrosarcoma protuberans were excluded due to their different behavior. Multivariate analysis (MVA) identified prognostic factors for overall survival (OS). Adjusted hazard ratios (AHR) and recursive partitioning analysis (RPA) were used to derive stage groupings. Stage grouping performance for OS was assessed and also compared against the existing TNM8 groups for non-HN STS.

RESULTS: A total of 221 patients (N1: 2; M1: 2) were included. Of the 219 M0 patients, 63% were males; median tumor size was 3.0 cm (range: 0.3-14.0); the proportion of TNM8 T1-T4 were 35%, 34%, 26%, and 5%, respectively. Median follow up was 5.9 years. Five-year OS was 79%. MVA confirmed the prognostic value of T category (T4 HR 7.73, 95% CI 3.62-16.5) and grade (G2/3 vs G1 HR 3.7, 95% CI 1.82-7.53), in addition to age (HR 1.03, 95% CI 1.01-1.04) (all p<0.001) for OS. AHR model derived T1-3_Grade 1 as stage 1; T1-3_Grade 2/3 as stage II; and T4_any Grade or any T_N1 as stage III (Table 1); the corresponding 5-year OS was 93%, 73%, and 38%, respectively. Both patients with M1 died within 1.5 years after diagnosis and M1 disease was designated stage IV. The AHR-grouping outperformed the RPA and non-HN TNM8 stage grouping for hazard consistency, hazard discrimination, percent variance explained, hazard difference, and sample size balance.

CONCLUSION: The novel T4 category introduced in TNM8 is associated with a >7 fold increased risk of death. Grade continues to be a critical prognostic factor in HN STS. The TNM8 HN STS T classifications have been validated, and the proposed new stage groupings with TNM8 incorporating grade have excellent performance for OS.

PMID:37784378 | DOI:10.1016/j.ijrobp.2023.06.567
Outcomes for Patients with Head and Neck Sarcoma Treated Curatively with Radiation Therapy and Surgery

Fetched: October 4th, 2023, 5:01am GMT by A K Yoder

Int J Radiat Oncol Biol Phys. 2023 Oct 1;117(2S):S149-S150. doi: 10.1016/j.ijrobp.2023.06.568.

ABSTRACT

PURPOSE/OBJECTIVE(S): Soft tissue sarcomas (STS) of the head and neck are rare and diverse entities that are challenging to manage. Definitive treatment requires surgery, often with radiation therapy (RT). We sought to describe the outcomes of patients treated curatively with surgery and RT for head and neck STS.

MATERIALS/METHODS: We performed a retrospective review of patients treated at a tertiary cancer center for non-metastatic STS of the head and neck between 1968 and 2020; over half were treated in the modern era defined as 2005 or after. Patients with rhabdomyosarcomas or cutaneous angiosarcoma were excluded. The Kaplan-Meier method was used to estimate actuarial overall survival (OS), disease-specific survival (DSS) and local control (LC). Multivariable analyses (MVA) were conducted using Cox proportional hazards model.

RESULTS: Median follow-up was 82 months. Of the 192 patients, the majority were male (n = 111, 58%), White (n = 158, 82%), and median age was 49.5 [IQR 32.5-64]. The highest proportion of tumors were in the neck (n = 50, 26%), paranasal sinuses (n = 36, 19%), or face (n = 23, 12%). The most common histology was sarcoma, not otherwise specified (n = 44, 23%), followed by undifferentiated pleomorphic sarcoma (n = 32, 17%), and neurogenic sarcoma (n = 15, 8%). Most patients were treated with postoperative RT (n = 134, 70%). Patients treated with preoperative RT were older (median 56.5 yrs vs post-op 44 yrs, p = 0.009). Post-op RT doses were higher (median 60 Gy, pre-op 50 Gy, p<0.001), and margins were more likely to be negative in those treated with pre-op RT (n = 39, 67%, post-op 69, 51%, p = 0.02). 5-year LC, DSS, and OS for the entire cohort was 76% (95% CI 69-82), 74% (67-80), and 71% (64-77), respectively. LC was not affected by treatment sequence (pre-op RT 78% (63-88), post-op RT 75% (66-82), p = 0.48). Patients with negative margins had improved 5-yr LC (86% (77-92), positive/uncertain 65% (53-74), p = 0.003). On MVA, positive/uncertain margin was the only variable associated with LC (HR 2.54 (1.34-4.82), p = 0.004). Poorer 5-yr DSS was associated with higher grade (grade 3 75% (63-84), grade 1-2 89% (75-94), p = 0.02), and treatment era (pre-2005 68% (57-76), on/after 2005 80% (70-87), p = 0.04). These both remained significant on MVA (grade 3 HR 2.39 (1.07-5.36), p = 0.034; pre-2005 HR 2.31 (1.03-5.21), p = 0.043). Sixteen patients (8%) developed a late RT toxicity, including fibrosis (n = 4, 2%), necrosis (osteoradionecrosis n = 2, brain necrosis n = 1, soft tissue necrosis n = 1), and eye dryness (n = 2, 1%). Neither the timing of RT nor dose was found to be associated with developing a late RT toxicity.

CONCLUSION: Head and neck STS continues to have relatively poorer local control than STS of the trunk or extremities. Timing of RT did not impact oncologic or long-term RT-related toxicity outcomes. However, more data are needed to ascertain whether preoperative RT may impact acute surgical toxicities. These data contribute to multidisciplinary care planning for patients with STS in these challenging anatomic locations.

PMID:37784377 | DOI:10.1016/j.ijrobp.2023.06.568
Epithelioid angiosarcoma of the calf: a case report of an atypical imaging appearance

Fetched: October 4th, 2023, 5:01am GMT by Sean Rodich

Skeletal Radiol. 2023 Oct 3. doi: 10.1007/s00256-023-04456-8. Online ahead of print.

ABSTRACT

Angiosarcoma is an uncommon cause of soft tissue malignancy, accounting for approximately 2% of all soft tissue sarcomas. Of these, epithelioid angiosarcoma represents a morphologic subtype, where the malignant endothelial cells demonstrate a predominantly or exclusively epithelioid appearance. Overall, epithelioid angiosarcoma shares similar imaging characteristics to conventional angiosarcoma including a T1 hypointense to isointense and T2 hyperintense mass, which demonstrates avid enhancement, serpentine feeding vessels, and overlying skin thickening on MRI. The case herein describes a case of epithelioid angiosarcoma in a 65-year-old female presenting with an enlarging calf mass and lower extremity pain. Initial imaging features, particularly on MRI, were highly unusual for angiosarcoma which was thus not strongly considered in the initial differential diagnosis. However, once diagnosis of epithelioid angiosarcoma was confirmed following resection, pathologic correlates were utilized to account for the unusual imaging findings retrospectively. The goal of this study is to not only describe an atypical presentation of an uncommon diagnosis but also attempt to rationalize the unexpected imaging findings with gross and microscopic correlates. Further, the utility of radiology-pathology correlation demonstrated in this case may be useful to others when evaluating similar lesions with unexpected MRI characteristics.

PMID:37787920 | DOI:10.1007/s00256-023-04456-8
A case report of angiosarcoma of the tongue: A diagnostic dilemma at presentation

Fetched: October 4th, 2023, 5:01am GMT by Shilajit Bhattacharya

Indian J Dent Res. 2023 Apr-Jun;34(2):226-228. doi: 10.4103/ijdr.ijdr_741_22.

ABSTRACT

RATIONALE: Angiosarcoma is a malignant mesenchymal tumor arising from vascular endothelial cells. This is an aggressive malignancy associated with poor prognosis. Angiosarcoma in the head and neck is exceedingly rare, accounting for less than 4 % of tumors.^[1].

PATIENT CONCERN: We report a unique case of angiosarcoma involving the tongue underlying the diagnostic conundrum and pointers towards an accurate diagnosis.

TAKE-AWAY LESSON: Due to rarity of the disease, consensus on optimal treatment approach is lacking and multicenter prospective studies would be helpful to set clinical guidelines.

PMID:37787218 | DOI:10.4103/ijdr.ijdr_741_22
Dosimetric Comparison of Automated Non-Coplanar Volumetric-Modulated Arc Therapy and Intensity-Modulated Proton Therapy in Angiosarcoma of the Scalp

Fetched: October 4th, 2023, 5:01am GMT by S Inui

Int J Radiat Oncol Biol Phys. 2023 Oct 1;117(2S):e675. doi: 10.1016/j.ijrobp.2023.06.2128.

ABSTRACT

PURPOSE/OBJECTIVE(S): Angiosarcoma of the scalp (AS) is a rare tumor and has often been treated by total scalp irradiation (TSI). TSI is a challenging technique because of the target close to the organs at risks (OARs), located in the skin surface, and helmet-shape of the target. We performed the dosimetric comparison of automated non-coplanar volumetric-modulated arc therapy (HA) and intensity-modulated proton therapy (IMPT) in TSI for AS.

MATERIALS/METHODS: A planning study was performed on 20 patients with AS. The clinical target volume (CTV) 1 and CTV2 were defined as gross tumor volume with margin and entire scalp, respectively. For HA, planning target volume (PTV) 1 and PTV2 were defined as CTV1 and CTV2 with 5-mm margins, respectively. For IMPT, robust optimization was utilized which accounted for a 5-mm setup and a 3.5% range uncertainty and dose was prescribed to CTV1 robust and CTV2 robust. The prescription doses were 70 Gy and 56 Gy in 35 fractions to PTV1 (CTV1 robust) and PTV2 (CTV2 robust), respectively, using the simultaneous integrated boost technique. The HA plan was performed using a 6-MV photon beam machine and a 1-cm thick virtual bolus. The HA plan included three half non-coplanar and one full coplanar arc fields. The HA plan used collimator angles of 15°, 60°, 15°, and 120° in the beam with couch rotations of 0°, 45°, 90°, and 315°, respectively. The IMPT plan was performed using a compact proton beam machine dedicated to pencil beam scanning. The IMPT plan used gantry angles of 70°, 150°, 70°, 150°, and 30° in the beam with couch rotations of 0°, 0°, 180°, 180°, and 270°, respectively. The IMPT plan was optimized assuming a relative biological effectiveness of 1.1. The dose distribution and dosimetric parameters for each plan were evaluated.

RESULTS: All plans met the goals within the acceptable range in target volume coverage, conformity, and homogeneity. Table shows the dosimetric parameters of OARs in HA and IMPT plans. The doses receiving 0.1 cc of the volume for hippocampus, optic passway, eye, and lens in the IMPT plan were significantly lower than those in the HA plan. The IMPT plan showed a significant reduction in the percentage of brain volume receiving at least 5 Gy (V5 Gy) compared to the HA plan, while the HA plan showed a significant reduction of V10 Gy - V60 Gy and mean brain dose compared to the IMPT plan. The HA plan provided a shorter beam-on time (184 ± 9 s) than did the IMPT plan (446 ± 49 s).

CONCLUSION: The HA and IMPT plans demonstrated different strengths with respect to OARs sparing.

PMID:37785989 | DOI:10.1016/j.ijrobp.2023.06.2128
Outcomes of Head and Neck Cutaneous Angiosarcoma Treated in the IMRT Era

Fetched: October 4th, 2023, 5:01am GMT by S Saha

Int J Radiat Oncol Biol Phys. 2023 Oct 1;117(2S):e620-e621. doi: 10.1016/j.ijrobp.2023.06.2004.

ABSTRACT

PURPOSE/OBJECTIVE(S): Clinical behavior, natural history, and varied presentations of cutaneous angiosarcomas of the head and neck region (HN), in conjunction with its rarity, have rendered standardization of treatment elusive. We aimed to assess outcomes and patterns of failure for patients treated with surgery and radiation (Sx+RT), and radiation alone (RT).

MATERIALS/METHODS: A retrospective review of all HN angiosarcoma patients amenable for upfront Sx or RT in our institution between 2004-2018 was completed. Generally, treatment included Sx when feasible, and RT for large or extensive/ill-defined tumors. Demographic, tumor characteristics, local (LC), regional (RC), distant control (DC), and overall survival (OS), as well as patterns (in-field, marginal, out-of-field) of local failure at 5-year were estimated. Univariate analysis (UVA) was conducted to assess association with outcomes.

RESULTS: A total of 33 patients were eligible (14 Sx+RT and 19 RT). Tumor locations were: scalp (16, 48%). face (n = 12, 36%), and overlapping (5, 15%). Lesion types were: nodular (n = 23, 70%), flat (n = 4, 12%) and mixed (n = 6, 18%). Tumor size was larger in the RT group (median: 10.00 vs 2.85 cm, p<0.01). RT and Sx+RT patients had otherwise similar baseline characteristics: median age 74.3; male 70%; and ECOG performance status ≤1 85%. RT dose fractionations ranged from 50-70 Gy in 25-35 fractions in the RT group and 50-66 Gy in 25-33 fractions in the Sx+RT group. Four (12%) patients received neoadjuvant chemotherapy. Median follow up was 5.5 years. Five-year LC, RC, DC, and OS for RT vs Sx+RT groups were 68% vs 85% (p = 0.28); 95% vs 86% (p = 0.89); 79% vs 86% (p = 0.39); and 45% vs 55% (p = 0.71), respectively. The in-field/marginal/out-of-field local failure rate at 5 years were 16% vs 7% (p = 0.46), 26% vs 15% (p = 0.41), and 13% vs 0% (p = 0.24) for the RT vs Sx+RT groups, respectively. UVA showed that scalp location and ulceration/bleeding were strong adverse features for OS. Bone invasion was significantly associated with lower DC (Table). Lesion type (nodular/flat/mixed), tumor size, and treatment type (Sx+RT vs RT), were not significantly associated with LC or pattern of local failure.

CONCLUSION: Scalp tumors, as compared to face, portended poorer prognosis, and ulceration/bleeding and bone invasion were associated with increased distant metastases. Sx+RT was the preferred treatment modality when possible and typically used for smaller and better defined tumors. RT was reserved for larger and extensive/ill-defined disease; despite this, in the IMRT era, RT achieves reasonable rates of control, markedly superior to historical series.

PMID:37785859 | DOI:10.1016/j.ijrobp.2023.06.2004
Clinical Characteristics, Patterns of Care, and Treatment Outcomes of Radiation-Associated Sarcomas

Fetched: October 4th, 2023, 5:01am GMT by R Raj

Int J Radiat Oncol Biol Phys. 2023 Oct 1;117(2S):e334-e335. doi: 10.1016/j.ijrobp.2023.06.2389.

ABSTRACT

PURPOSE/OBJECTIVE(S): In this study, we describe the clinical presentation, patterns of care, and treatment outcomes for radiation-associated sarcomas (RAS) patients seen at our institution.

MATERIALS/METHODS: Patients with RAS treated at The Ohio State University Comprehensive Cancer Center between January 1, 2015 and January 20, 2021 were included. Demographic and clinical variables, as well as outcomes, were abstracted from chart review. Differences in outcomes were assessed based on log-rank test.

RESULTS: A total of 38 patients with a diagnosis of RAS were identified (17 males, 21 females). The median age at radiotherapy for index malignancy was 55.3 years (range 0.2-77.5). The median latency following index radiotherapy until the RAS diagnosis was 9.1 years (range 3.7-46.3). The median age at RAS diagnosis was 68.4 years (range 27.9-85.4). The most common index malignancy associated with a subsequent RAS was breast carcinoma (n = 9 [23.7%]). The most common RAS histology was angiosarcoma (n = 10 [26.3%]). Median RAS tumor size was 5.25 cm (range 0.4-30.5). RAS grade was most commonly grade 3 (n = 25 [65.8%]). RAS locations included abdominopelvic (n = 15 [39.5%]); head & neck (n = 10 [26.3%]); and thorax (n = 10 [26.3%]). Genomic profiling identified mutations in TP53 (n = 12 [31.6%]); MYC (n = 6 [15.8%]); and CDK2A/4 (n = 5 [13.2%]). Median tumor mutation burden (TMB) was 2.6 mutations/megabase (range 0.5-6.3). Among the 33 patients with non-metastatic RAS at presentation, 78.8% (n = 26) underwent oncologic resection. Margins were R0 in 69.2% (n = 18), R1 in 19.2% (n = 5), and R2 in 11.5% (n = 3). 54.5% (n = 18) received systemic therapy and 24.2% (n = 8) received radiotherapy as part of curative-intent treatment (median dose 50.4 Gy [range 45-57.5]). For the entire cohort, median PFS was 9.0 months and median OS was 11.1 months. Among patients who presented with non-metastatic RAS, the median LRFS was 9.5 months (range 2.0-34.7); median PFS was 9.0 months (range 2.0-34.7); and median OS was 10.6 months (range 0.6-31.6). Amongst patients with metastatic RAS at presentation, median PFS was 7.4 months (range 1.4-15.8) and median OS was 13.1 months (range 6.0-14.9). Patients who presented with non-metastatic vs metastatic RAS had a significantly longer PFS (p = 0.025) and OS (p = 0.033). Amongst non-metastatic RAS, an R0 resection was associated with significantly improved mean OS (75.3 months [95% CI 54.9-95.8]) in comparison to R1 (15.2 months [95% CI 5.3-25.1]) and R2 margins (19.7 months [95% CI 10.7-28.7]), p = 0.016. Among patients managed with curative intent, there was no significant association between receipt of either systemic therapy or radiotherapy and duration of LC, PFS, or OS.

CONCLUSION: Outcomes for RAS are strikingly poor. Patients with non-metastatic RAS had a significantly longer PFS and OS compared to patients who presented with metastatic RAS. As part of a curative-intent treatment, R0 surgical margins, but not receipt of systemic therapy or radiotherapy, were associated with improved OS in our cohort.

PMID:37785176 | DOI:10.1016/j.ijrobp.2023.06.2389
Clinico-demographic characteristics and outcomes of radiation-induced sarcomas (RIS): a CanSaRCC study

Fetched: October 3rd, 2023, 5:00am GMT by Mauricio Fernando Ribeiro

Ther Adv Med Oncol. 2023 Sep 28;15:17588359231198943. doi: 10.1177/17588359231198943. eCollection 2023.

ABSTRACT

BACKGROUND: Radiation-induced sarcomas (RIS) tend to have aggressive behaviour and because of their rarity, the most appropriate management for these malignancies is uncertain.

OBJECTIVES: Using the Canadian Sarcoma Research and Clinical Collaboration (CanSaRCC) database, a national sarcoma registry, we aimed to investigate prognostic factors and outcomes for RIS.

DESIGN: Retrospective study of RIS patients treated from 1996 to 2021 at three Canadian centres.

METHODS: RIS was defined as a sarcoma arising in a previously irradiated field following a 3+ year latency period, whose histology was distinct from the initially irradiated tumour. Clinicopathologic and treatment-related information was extracted from the CanSaRCC database. Overall survival (OS) was defined as the time from RIS diagnosis to death from any cause. Response rate (RR) to neoadjuvant chemotherapy (NACT) was based on physician assessment. Time-to-event analyses were estimated using the Kaplan-Meier method, with Cox regression for multivariate analysis. We considered a two-tailed p-value of <0.05 as statistically significant.

RESULTS: One hundred seven tumours met the criteria for RIS and were divided into three subgroups: breast angiosarcoma (BAS, n = 54), osteosarcoma (OST, n = 16), and other soft-tissue sarcomas (STS, n = 37). Patients were mostly female (n = 85, 79%), treated initially for breast carcinomas (n = 54, 50.5%), and diagnosed with high-grade tumours (n = 61/71, 86%). None had evidence of synchronous metastasis. Patients with OST were younger (median age: 48 years, p < 0.001), and BAS had the shortest latency interval (8 versus 18 years for OST/STS, p < 0.001). Most patients underwent surgery, 76% (n = 76/100) R0; 24% (n = 26) received radiation therapy, mostly (n = 15, 57.7%) neoadjuvant. Among those receiving chemotherapy, 30 (75%) underwent NACT; among patients with documented response assessment, the RR was 68% (n = 17/25), being even higher in the BAS population (89.5%, n = 13/17). Median OS was 53 months (95% CI 34-101), with a 5-year OS of 47.6%; larger tumour size, high histologic grade and older age were independent prognostic factors for worse OS.

CONCLUSION: Surgery is standard, and NACT might be useful to downsize large lesions, especially in BAS patients. Raising RIS awareness is fundamental to promoting appropriate management and fostering research through multi-institutional collaborations.

PMID:37781501 | PMC:PMC10540571 | DOI:10.1177/17588359231198943
Conservatively treated wound for four months turned out to be highly malignant cutaneous angiosarcoma

Fetched: September 29th, 2023, 5:01am GMT by Theresia Skytte Eriksen

Ugeskr Laeger. 2023 Sep 4;185(36):V03230138.

ABSTRACT

Angiosarcomas are rare, highly malignant tumours of vascular origin. They present as fast growing, haematoma-like and diffuse lesions. In this case report, a 71-year-old woman presented with what was assessed as an abscess arising from a traumatic haematoma of the forehead. The lesion was primarily treated conservatively. The condition progressed despite of treatment to a point where the tumour covered a third of her face. After four months, skin biopsies were taken and the diagnosis angiosarcoma was made. This case report should lead to an increased focus on angiosarcomas and the importance of sufficient biopsies.

PMID:37767866
LINE-1 Methylation Status in Canine Splenic Hemangiosarcoma Tissue and Cell-Free DNA

Fetched: September 29th, 2023, 5:01am GMT by Hiroki Sato

Animals (Basel). 2023 Sep 21;13(18):2987. doi: 10.3390/ani13182987.

ABSTRACT

Splenic hemangiosarcoma is one of the most common malignant tumors in dogs, and early diagnosis is of great importance for achieving a good prognosis. DNA methylation plays an important role in cancer development. Long interspersed nuclear element 1 (LINE-1) is the most abundant repetitive element in the genome. LINE-1 hypomethylation has been shown to be related to carcinogenesis in humans, and it has been used as a novel cancer biomarker. This study aimed to evaluate the methylation status of LINE-1 in tumor tissue and circulating cell-free DNA and assess its clinical significance in canine splenic hemangiosarcoma. Genomic DNA was isolated from splenic masses of 13 dogs with hemangiosarcoma, 11 with other malignant tumors, and 15 with benign lesions. LINE-1 methylation was quantified using methylation-sensitive and -insensitive restriction enzyme digestion followed by real-time polymerase chain reaction. Additionally, blood samples were collected from eight patients to isolate cell-free DNA to determine LINE-1 methylation status changes during the treatment course. LINE-1 methylation in tumor samples was significantly lower in patients with hemangiosarcoma than in those with other malignant tumors and benign lesions. Non-significant but similar results were observed for the cell-free DNA samples. Our results demonstrate that LINE-1 methylation status is a potential biomarker for splenic hemangiosarcoma.

PMID:37760387 | PMC:PMC10525518 | DOI:10.3390/ani13182987
Clinical characteristics of sarcoma patients: a population-based data analysis from a German clinical cancer registry

Fetched: September 27th, 2023, 5:01am GMT by Jörg Andreas Müller

J Cancer Res Clin Oncol. 2023 Dec;149(19):17051-17069. doi: 10.1007/s00432-023-05350-5. Epub 2023 Sep 26.

ABSTRACT

PURPOSE: Sarcomas are a heterogeneous group of malignant neoplasms with a wide range of histological types and occur in almost any anatomic site and side. This study evaluated the prognostic factors in sarcoma patients based on German clinical cancer registry data.

METHODS: The German clinical cancer register of Saxony-Anhalt was used for all data analyses. Sarcoma cases of all clinical or pathological T-stages (T1a-T4c), all N-stages (N0-3) and M-stages (0-1b) corresponding to the Union for International Cancer Control (UICC) stages I to IVB were considered. In our analyses, 787 cases diagnosed between 2005 and 2022 were included. Further, we assessed the association of cancer-related parameters with mortality and hazard ratios (HR) from the Cox proportional hazard models. We included sex, age at diagnosis, histological grade, T-, N- and M-stages, tumor size, tumor localization and tumor side as parameters in our regression models.

RESULTS: The majority of sarcoma patients were diagnosed with leiomyosarcoma (12%), liposarcoma (11%), angiosarcoma (5.3%) and myxofibrosarcoma (2.7%). In our univariate regression models, tumors localized in more than one location, head, face and neck region as well as the pelvis and lower extremity were associated with increased mortality risk (more than one location: HR 7.10, 95% CI 2.20-22.9; head, face and neck: HR 1.35, 95% CI 0.89-2.06; pelvis: HR 1.27, 95% CI 0.86-1.89; lower extremity: HR 1.44, 95% CI 1.05-1.96). Higher histological grades, UICC-grades and TNM-stages were related to a higher mortality risk. Differing histological subtypes had significant influence on overall survival and progression-free survival. Patients diagnosed with fibromyxoid sarcoma, rhabdomyosarcoma and angiosarcoma were related to higher mortality risk compared to other histological subtypes (fibromyxoid sarcoma: HR 5.2, 95% CI 0.71-38.1; rhabdomyosarcoma: HR 2.93, 95% CI 1.44-6.00; angiosarcoma: HR 1.07, 95% CI 0.53-2.18).

CONCLUSIONS: Histological grade, tumor size, nodal and distant metastasis, tumor localization and histological subtype were determined as prognostic factors in terms of survival.

PMID:37750956 | DOI:10.1007/s00432-023-05350-5
Cardiac Angiosarcoma With Pulmonary Metastasis: A Rare and Challenging Case

Fetched: September 26th, 2023, 5:01am GMT by Adesola A Agboola

Cureus. 2023 Aug 23;15(8):e43962. doi: 10.7759/cureus.43962. eCollection 2023 Aug.

ABSTRACT

Cardiac angiosarcoma is a rare and aggressive malignant tumor arising from the endothelial cells of the heart. It accounts for only a small fraction of all cardiac neoplasms and has a poor prognosis. We present a challenging case of a 20-year-old student who presented exertional dyspnea, palpitation, and occasional chest discomfort. Her clinical picture, radiological and pathological investigations confirm the diagnosis of cardiac angiosarcoma with pulmonary metastasis. This case highlights the importance of early diagnosis and multidisciplinary management for improved patient outcomes.

PMID:37746442 | PMC:PMC10515094 | DOI:10.7759/cureus.43962
Worker studies suggest unique liver carcinogenicity potential of polyvinyl chloride microplastics

Fetched: September 25th, 2023, 5:01am GMT by Gregory M Zarus

Am J Ind Med. 2023 Dec;66(12):1033-1047. doi: 10.1002/ajim.23540. Epub 2023 Sep 24.

ABSTRACT

BACKGROUND: Plastic debris pervades our environment. Some breaks down into microplastics (MPs) that can enter and distribute in living organisms causing effects in multiple target organs. MPs have been demonstrated to harm animals through environmental exposure. Laboratory animal studies are still insufficient to evaluate human impact. And while MPs have been found in human tissues, the health effects at environmental exposure levels are unclear.

AIM: We reviewed and summarized existing evidence on health effects from occupational exposure to MPs. Additionally, the diverse effects documented for workers were organized by MP type and associated co-contaminants. Evidence of the unique effects of polyvinyl chloride (PVC) on liver was then highlighted.

METHODS: We conducted two stepwise online literature reviews of publications focused on the health risks associated with occupational MP exposures. This information was supplemented with findings from animal studies.

RESULTS: Our analysis focused on 34 published studies on occupational health effects from MP exposure with half involving exposure to PVC and the other half a variety of other MPs to compare. Liver effects following PVC exposure were reported for workers. While PVC exposure causes liver toxicity and increases the risk of liver cancers, including angiosarcomas and hepatocellular carcinomas, the carcinogenic effects of work-related exposure to other MPs, such as polystyrene and polyethylene, are not well understood.

CONCLUSION: The data supporting liver toxicity are strongest for PVC exposure. Overall, the evidence of liver toxicity from occupational exposure to MPs other than PVC is lacking. The PVC worker data summarized here can be useful in assisting clinicians evaluating exposure histories from PVC exposure and designing future cell, animal, and population exposure-effect research studies.

PMID:37742097 | DOI:10.1002/ajim.23540
Primary cardiac angiosarcoma presenting with haemoptysis

Fetched: September 22nd, 2023, 5:01am GMT by Abraham Tolentino-de la Mora

BMJ Case Rep. 2023 Sep 21;16(9):e256596. doi: 10.1136/bcr-2023-256596.

ABSTRACT

A male patient in his 30s presented to the emergency room with a 1-week history of dyspnoea that progressed to haemoptysis, having coughed up approximately 200 mL of blood on two occasions. On diagnostic investigation, a mediastinal tumour infiltrating the free wall of the right atrium and multiple pulmonary nodules were discovered. The first suspicion was a neoplasm of pulmonary origin, and a bronchoscopy was performed, histology reported a probable cardiac origin for the neoplasm. A subsequent biopsy confirmed the presence of a primary cardiac angiosarcoma. An extension CT scan revealed brain metastases. The patient received chemotherapy treatment, resulting in a partial response to date. This case is one of the few reported instances of cardiac neoplasm presenting with respiratory symptoms.

PMID:37734864 | PMC:PMC10514630 | DOI:10.1136/bcr-2023-256596
Identification of genomic alterations with clinical impact in canine splenic hemangiosarcoma

Fetched: September 22nd, 2023, 5:01am GMT by Timothy Estabrooks

Vet Comp Oncol. 2023 Sep 21. doi: 10.1111/vco.12925. Online ahead of print.

ABSTRACT

Canine hemangiosarcoma (HSA) is an aggressive cancer of endothelial cells with short survival times. Understanding the genomic landscape of HSA may aid in developing therapeutic strategies for dogs and may also inform therapies for the rare and aggressive human cancer angiosarcoma. The objectives of this study were to build a framework for leveraging real-world genomic and clinical data that could provide the foundation for precision medicine in veterinary oncology, and to determine the relationships between genomic and clinical features in canine splenic HSA. One hundred and nine dogs with primary splenic HSA treated by splenectomy that had tumour sequencing via the FidoCure® Precision Medicine Platform targeted sequencing panel were enrolled. Patient signalment, weight, metastasis at diagnosis and overall survival time were retrospectively evaluated. The incidence of genomic alterations in individual genes and their relationship to patient variables including outcome were assessed. Somatic mutations in TP53 (n = 44), NRAS (n = 20) and PIK3CA (n = 19) were most common. Survival was associated with presence of metastases at diagnosis and germline variants in SETD2 and NOTCH1. Age at diagnosis was associated with somatic NRAS mutations and breed. TP53 and PIK3CA somatic mutations were found in larger dogs, while germline SETD2 variants were found in smaller dogs. We identified both somatic mutations and germline variants associated with clinical variables including age, breed and overall survival. These genetic changes may be useful prognostic factors and provide insight into the genomic landscape of hemangiosarcoma.

PMID:37734854 | DOI:10.1111/vco.12925
Adrenal tumors treated by adrenalectomy following spontaneous rupture carry an overall favorable prognosis: retrospective evaluation of outcomes in 59 dogs and 3 cats (2000-2021)

Fetched: September 22nd, 2023, 5:01am GMT by Marine Traverson

J Am Vet Med Assoc. 2023 Sep 21:1-9. doi: 10.2460/javma.23.06.0324. Online ahead of print.

ABSTRACT

OBJECTIVE: To conduct a retrospective multi-institutional study reporting short- and long-term outcomes of adrenalectomy in patients presenting with acute hemorrhage secondary to spontaneous adrenal rupture.

ANIMALS: 59 dogs and 3 cats.

METHODS: Medical records of dogs and cats undergoing adrenalectomy between 2000 and 2021 for ruptured adrenal masses were reviewed. Data collected included clinical presentation, preoperative diagnostics, surgical report, anesthesia and hospitalization findings, histopathology, adjuvant treatments, and long-term outcome (recurrence, metastasis, and survival).

RESULTS: Median time from hospital admission to surgery was 3 days, with 34% of surgeries being performed emergently (within 1 day of presentation). Need for intraoperative blood transfusion was significantly associated with emergent surgery and presence of active intraoperative hemorrhage. The short-term (≤ 14 days) complication and mortality rates were 42% and 21%, respectively. Negative prognostic factors for short-term survival included emergent surgery, intraoperative hypotension, and performing additional surgical procedures. Diagnoses included adrenocortical neoplasia (malignant [41%], benign [12%], and undetermined [5%]), pheochromocytoma (38%), a single case of adrenal fibrosis and hemorrhage (2%), and a single case of hemangiosarcoma (2%). Local recurrence and metastasis of adrenocortical carcinoma were confirmed in 1 and 3 cases, respectively. Overall median survival time was 574 days and 900 days when short-term mortality was censored. No significant relationship was found between histopathological diagnosis and survival.

CLINICAL RELEVANCE: Adrenalectomy for ruptured adrenal gland masses was associated with similar short- and long-term outcomes as compared with previously reported nonruptured cases. If hemodynamic stability can be achieved, delaying surgery and limiting additional procedures appear indicated to optimize short-term survival.

PMID:37734721 | DOI:10.2460/javma.23.06.0324
Adult Kasabach-Merritt Syndrome secondary to hepatic angiosarcoma

Fetched: September 22nd, 2023, 5:01am GMT by Kaige Deng

Clin Res Hepatol Gastroenterol. 2023 Oct;47(8):102211. doi: 10.1016/j.clinre.2023.102211. Epub 2023 Sep 19.

NO ABSTRACT

PMID:37734443 | DOI:10.1016/j.clinre.2023.102211
Epithelioid Hemangioendothelioma of Tongue: A Rare Presentation

Fetched: September 21st, 2023, 5:00am GMT by Durga Bai Yendluri

J Dent (Shiraz). 2023 Sep;24(3):352-355. doi: 10.30476/dentjods.2023.96208.1927.

ABSTRACT

Hemangioendothelioma is a diverse set of proliferative and neoplastic vascular lesions with biological characteristics that fall halfway between benign hemangioma and malignant angiosarcoma. Hemangioendothelioma of the oral cavity is extremely rare and if present, it is seen on lips, gingiva, tongue, maxilla, and mandible. The following case report is about a lesion on the right ventrolateral border of the tongue of a six-year-old female patient. A Laser excision was done. Histopathology revealed the features of hemangioendothelioma. An immunohistochemical (IHC) study was done to correlate the findings with a histopathological picture and arrived at the final diagnosis of epithelioid hemangioendothelioma (EHE). The patient was followed up for two years and no recurrence was noticed.

PMID:37727360 | PMC:PMC10506149 | DOI:10.30476/dentjods.2023.96208.1927
A case of primer angiosarcoma ın a young woman: lessons from multi-modality ımaging

Fetched: September 20th, 2023, 5:00am GMT by Ersin Doganozu

Int J Cardiovasc Imaging. 2022 Sep;38(9):2093-2098. doi: 10.1007/s10554-022-02635-7. Epub 2022 May 18.

NO ABSTRACT

PMID:37726613 | DOI:10.1007/s10554-022-02635-7
Secondary Breast Angiosarcoma After a Primary Diagnosis of Breast Cancer: A Retrospective Analysis of the Surveillance, Epidemiology, and End Results (SEER) Database

Fetched: September 20th, 2023, 5:00am GMT by Bonny Chau

Am J Clin Oncol. 2023 Sep 19. doi: 10.1097/COC.0000000000001045. Online ahead of print.

ABSTRACT

OBJECTIVES: Angiosarcoma is a rare complication of breast-conserving therapy. This study evaluated the change in incidence between 1992 and 2016 of secondary breast angiosarcoma (SBA) in patients with a history of breast cancer and the impact of management strategies for the original breast carcinoma on angiosarcoma treatment.

METHODS: Breast cancer and angiosarcoma cases were abstracted from the Surveillance, Epidemiology, and End Result (SEER) database. SBAs were defined as angiosarcomas located in the breast occurring after a prior breast cancer diagnosis. Primary breast angiosarcomas (PBAs) were defined as an angiosarcoma diagnosis listed as "one primary only." Incidence rates were estimated using a proportion of the US total population. Survival was analyzed by the Kaplan-Meier method, and Cox proportional hazard models were used to assess the association of clinicopathologic characteristics on overall survival.

RESULTS: Between 1992 and 2016, 193 cases of SBA were reported in the SEER dataset in patients with a prior history of breast cancer. The incidence of breast angiosarcoma in patients with a prior diagnosis of breast cancer increased 3-fold from about 10 cases per 100,000 person-years to about 30 cases per 100,000 person-years over this same period (P=0.0037). For treatment of SBA (n=193), almost all (95%) had surgery. Nine percent received radiation (compared with 35% of patients with PBA, P<0.001) and 23% received chemotherapy (vs. 45% for PBA, P=0.11).

CONCLUSIONS: We demonstrate an increasing incidence of SBA over the study period. These data can help inform shared decision-making for optimal management of locoregional breast cancer and raise awareness of secondary angiosarcoma.

PMID:37725702 | DOI:10.1097/COC.0000000000001045
Primary Hepatic Angiosarcoma Presenting As Cryptogenic Cirrhosis

Fetched: September 19th, 2023, 5:01am GMT by Ricardo Anguiano-Albarran

Cureus. 2023 Aug 15;15(8):e43529. doi: 10.7759/cureus.43529. eCollection 2023 Aug.

ABSTRACT

Primary hepatic angiosarcoma (PHA) is an exceedingly rare and aggressive neoplasm of mesenchymal origin. PHA makes a very small portion of primary liver tumors and conveys a poor prognosis. Symptomatology can be vague and often mimics primary hepatocellular carcinoma upon presentation. Diagnosis requires careful immunohistopathologic confirmation. We present a case of PHA in a patient with abdominal pain and suspected underlying cryptogenic cirrhosis.

PMID:37719603 | PMC:PMC10501742 | DOI:10.7759/cureus.43529
A rare benign tumor of the liver mimicking angiosarcoma: Anastomosing hemangioma

Fetched: September 19th, 2023, 5:01am GMT by Rashad Ismayilov

North Clin Istanb. 2023 Jul 31;10(4):524-526. doi: 10.14744/nci.2022.21957. eCollection 2023.

ABSTRACT

Anastomosing hemangioma of the liver (AHL) is a very rare condition and limited to a few cases. It is often confused with well-differentiated angiosarcomas and causes overtreatment. In this report, we present a 53-year-old female patient diagnosed with AHL. Since the tumor is rarely seen, it is important to define well the imaging and pathological features for preventing unnecessary surgeries and related morbidities.

PMID:37719253 | PMC:PMC10500233 | DOI:10.14744/nci.2022.21957
Case report: a case of primary intracranial parasagittal meningeal angiosarcoma

Fetched: September 17th, 2023, 5:00am GMT by Jun Cao

Diagn Pathol. 2023 Sep 16;18(1):104. doi: 10.1186/s13000-023-01389-7.

ABSTRACT

BACKGROUND: Angiosarcoma, also known as malignant hemangioendothelioma, is a rare vasogenic malignant tumor, commonly found on the skin of the head and neck, rarely occurring in the intracranial region. As for intracranial meningeal angiosarcoma, only 8 cases have been reported before and there is no clinical study with large sample size. We report here a case of parasagittal meningeal angiosarcoma.

CASE DESCRIPTION: A 48-year-old Chinese male patient was admitted to our hospital due to headache accompanied by bilateral lower limb weakness. On admission, CT showed a high-density mass on both sides of the sagittal sinus at the top of the frontal lobe. We performed exploratory surgical resection of the tumor. During the operation, it was found that the tumor originated from the dura mater and extensively invaded the surrounding brain tissue and skull, and the surrounding hemosiderin deposition was observed. Postoperative pathology suggested angiosarcoma.

CONCLUSIONS: Intracranial meningeal angiosarcoma is difficult to accurately diagnose before surgery, so radiologists and neurosurgeons need to strengthen their understanding of this disease. The presence of extensive superficial hemosiderin deposition during operation may contribute to the diagnosis, and immunohistochemistry is very important for the diagnosis of intracranial angiosarcoma.

PMID:37717004 | PMC:PMC10504741 | DOI:10.1186/s13000-023-01389-7
Survival Outcomes of Rare Cutaneous Malignancies within an Insured Cohort of Patients, 1988-2018

Fetched: September 17th, 2023, 5:00am GMT by Amanda Rosenthal

J Am Acad Dermatol. 2023 Sep 13:S0190-9622(23)02691-9. doi: 10.1016/j.jaad.2023.08.087. Online ahead of print.

ABSTRACT

BACKGROUND: There are limited survival data on cutaneous angiosarcoma (CAS), dermatofibrosarcoma protuberans (DFSP), Merkel cell carcinoma (MCC), and sebaceous carcinoma (SC).

OBJECTIVE: To analyze survival trends in CAS, DFSP, MCC, and SC among a racially diverse, insured cohort of patients.

METHODS: Using data from the Kaiser Permanente Southern California Cancer Registry, we identified adults diagnosed with CAS, DFSP, MCC, or SC between January 1, 1988 and December 31 2018, followed through December 31, 2021.

RESULTS: Our cohort consisted of 83 diagnoses of CAS, 490 diagnoses of DFSP, 411 diagnoses of MCC, and 249 diagnoses of SC. Our analysis revealed no significant differences in overall or disease-specific 1000 person-years mortality rates among our populations of non-Hispanic Whites, Hispanics, African American/Blacks, and Asian American/Pacific Islanders diagnosed with CAS, DFSP, MCC, or SC. On multivariate analysis, controlling for patient and tumor characteristics, there was similarly no increased risk of overall mortality for minorities diagnosed with CAS, DFSP, MCC, or SC.

LIMITATIONS: Retrospective nature of the analysis and small sample size.

CONCLUSION: Contrary to existing literature, our results show a notable lack of racially driven survival disparities among insured individuals with CAS, DFSP, MCC, and SC, emphasizing the importance of health care coverage.

PMID:37714218 | DOI:10.1016/j.jaad.2023.08.087
Angiosarcoma in the breast: a population-based cohort from Sweden

Fetched: September 15th, 2023, 5:01am GMT by Susanne Olander

Br J Surg. 2023 Sep 14:znad290. doi: 10.1093/bjs/znad290. Online ahead of print.

ABSTRACT

BACKGROUND: Breast angiosarcoma is a rare disease mostly observed in breast cancer (BC) patients who have previously received radiotherapy (RT). Little is known about angiosarcoma aetiology, management, and outcome. The study aim was to estimate risk and to characterize breast angiosarcoma in a Swedish population-based cohort.

METHODS: The Swedish Cancer Registry was searched for breast angiosarcoma between 1992 and 2018 in three Swedish healthcare regions (population 5.5 million). Information on previous BC, RT, management, and outcome were retrieved from medical records.

RESULTS: Overall, 49 angiosarcomas located in the breast, chest wall, or axilla were identified, 8 primary and 41 secondary to BC treatment. Median age was 51 and 73 years, respectively. The minimum latency period of secondary angiosarcoma after a BC diagnosis was 4 years (range 4-21 years). The cumulative incidence of angiosarcoma after breast RT increased continuously, reaching 1.4‰ after 20 years. Among 44 women with angiosarcoma treated by surgery, 29 developed subsequent local recurrence. Median recurrence-free survival was 3.4 and 1.8 years for primary and secondary angiosarcoma, respectively. The 5-year overall survival probability for the whole cohort was 50 per cent (95 per cent c.i., 21 per cent-100 per cent) for primary breast angiosarcoma and 35 per cent (95 per cent c.i., 23 per cent-54 per cent) for secondary angiosarcoma.

CONCLUSION: Breast angiosarcoma is a rare disease strongly associated with a history of previous BC RT. Overall survival is poor with high rates of local recurrences and distant metastasis.

PMID:37708086 | DOI:10.1093/bjs/znad290
Efficacy and toxicity of carboplatin in the treatment of macroscopic mesenchymal neoplasia in dogs

Fetched: September 15th, 2023, 5:01am GMT by Céleste Pritchard

Vet Comp Oncol. 2023 Sep 13. doi: 10.1111/vco.12936. Online ahead of print.

ABSTRACT

Palliative chemotherapy options for dogs with macroscopic non-osseous mesenchymal tumours are limited. The purpose of this study was to assess the response rate of these tumours to carboplatin chemotherapy. Medical records of 28 dogs treated with carboplatin for macroscopic mesenchymal neoplasia between 1990 and 2022 were retrospectively reviewed. Sixteen dogs with soft tissue sarcoma and 12 dogs with haemangiosarcoma were included. Responses observed included one complete response and three partial responses, for an overall response rate of 14.2% (4/28) and median time to progression of 42 days (range 21-259 days). Responses were only seen in patients with haemangiosarcoma, for a response rate of 33.3% (4/12) and median time to progression for responders of 103 days (range 39-252 days). Median time to progression for dogs with metastatic disease was similar to those with only local disease (distant median: 44 days; local median: 23 days, p = 0.56). Dogs with chemotherapy-naïve disease were compared to dogs having received previous chemotherapy treatment and had a median time to progression of 75 days and 40.5 days respectively (p = 0.13). Twenty-two dogs experienced 48 adverse events, with most being grade 1 or 2 (79%). Carboplatin was well tolerated, with variable macroscopic anti-tumour activity and short response duration. Carboplatin may be an acceptable rescue option for dogs with macroscopic haemangiosarcoma, especially those patients that cannot receive doxorubicin.

PMID:37705417 | DOI:10.1111/vco.12936
Mediastinal Epithelioid Angiosarcoma, New Insights into an Uncommon Diagnosis: A Case Report and Literature Review

Fetched: September 12th, 2023, 5:00am GMT by Janira M Navarro Sanchez

Hawaii J Health Soc Welf. 2023 Sep;82(9):208-212.

ABSTRACT

Angiosarcoma is an uncommon malignant mesenchymal neoplasm, accounting for 1-2% of all sarcomas. More than half are cutaneous, with the remainder arising in the deep soft tissue, breast, bone or viscera, particularly the liver, spleen and heart. Mediastinal angiosarcomas are exceedingly uncommon. While epithelioid morphology is sometimes a minor component in conventional angiosarcoma, tumors with a predominance of epithelioid morphologic features are designated as epithelioid angiosarcoma (EAS). This is a report of a 58-year-old woman presenting with severe chest pain, accompanied by worsening dyspnea and dysphagia. Chest computed tomography (CT) revealed a large pericardial effusion and a bulky mediastinal mass. Biopsy revealed a malignant neoplasm with vascular differentiation consistent with high-grade EAS. By immunohistochemistry, epithelioid angiosarcomas express endothelial cell markers, such as CD31, CD34, ERG and FLI-1. A variable proportion express low molecular weight cytokeratin (CK), epithelial membrane antigen (EMA) and CD30. The use of molecular techniques has proven useful in the diagnosis of this rare neoplasm. Targeted next generation sequencing showed aberrations in multiple genes including NRAS, KRAS, MYC and TP53.

PMID:37694219 | PMC:PMC10485769
A Phase 2, Single-Arm, Open-Label Clinical Trial on Adjuvant Peptide-Based Vaccination in Dogs with Aggressive Hemangiosarcoma Undergoing Surgery and Chemotherapy

Fetched: September 10th, 2023, 5:00am GMT by Laura Marconato

Cancers (Basel). 2023 Aug 22;15(17):4209. doi: 10.3390/cancers15174209.

ABSTRACT

To test the antitumor effect and safety of peptide-based anticancer vaccination in dogs with hemangiosarcoma undergoing the standard of care (SOC; surgery and doxorubicin), canine hemangiosarcoma cells were infected with Salmonella typhi Ty21a to release immunogenic endoplasmic reticulum stress-related peptides into the extracellular milieu via CX43 hemichannels opening. The infected tumor cell secretome constituted the vaccine. Following the SOC, dogs with biologically aggressive hemangiosarcoma were vaccinated a total of five times, once every 3 weeks, and were followed up with serial imaging. A retrospective population of dogs undergoing the SOC alone served as controls. The primary endpoints were the time to progression (TTP) and overall survival (OS), and the secondary endpoints were toxicity and immune responses. A total of 28 dogs were vaccinated along with the SOC, and 32 received only the SOC. A tumor-specific humoral response along with a vaccine-specific T-cell response was observed. Toxicity did not occur. The TTP and OS were significantly longer in vaccinated versus unvaccinated dogs (TTP: 195 vs. 160 days, respectively; p = 0.001; OS: 276 vs. 175 days, respectively; p = 0.002). One-year survival rates were 35.7% and 6.3% for vaccinated and unvaccinated dogs, respectively. In dogs with hemangiosarcoma undergoing the SOC, the addition of a peptide-based vaccine increased the TTP and OS, while maintaining a safe profile. Moreover, vaccinated dogs developed a tumor-specific response, supporting the feasibility of future phase three studies.

PMID:37686485 | PMC:PMC10486958 | DOI:10.3390/cancers15174209
Axitinib in patients with advanced/metastatic soft tissue sarcoma (Axi-STS): an open-label, multicentre, phase II trial in four histological strata

Fetched: September 10th, 2023, 5:00am GMT by Penella J Woll

Br J Cancer. 2023 Sep 8. doi: 10.1038/s41416-023-02416-6. Online ahead of print.

ABSTRACT

BACKGROUND: Axitinib is an oral vascular endothelial growth factor receptor inhibitor with anti-tumour activity in renal, thyroid, and pancreatic cancer.

METHODS: Axi-STS was a pathologically-stratified, non-randomised, open-label, multi-centre, phase II trial of continuous axitinib treatment in patients ≥16 years, performance status ≤2, with pathologically-confirmed advanced/metastatic soft tissue sarcoma (STS). Patients were recruited within four tumour strata, each analysed separately: angiosarcoma, leiomyosarcoma, synovial sarcoma, or other eligible STSs. The primary outcome was progression-free survival at 12 weeks (PFS12). A Simon's two-stage design with activity defined as PFS12 rate of 40% determined a sample size of 33 patients per strata.

RESULTS: Between 31-August-2010 and 29-January-2016, 145 patients were recruited: 38 angiosarcoma, 37 leiomyosarcoma, 36 synovial sarcoma, and 34 other subtypes. PFS12 rate for each stratum analysed was 42% (95% lower confidence interval (LCI); 29), 45% (95% LCI; 32), 57% (95% LCI; 42), and 33% (95% LCI; 21), respectively. There were 74 serious adverse events including two treatment-related deaths of pulmonary haemorrhage and gastrointestinal bleeding. Fatigue and hypertension were the most common grade 3 adverse events.

CONCLUSIONS: Axitinib showed clinical activity in all STS strata investigated. The adverse event profile was acceptable, supporting further investigation in phase III trials.

CLINICAL TRIAL REGISTRATION: ISRCTN 60791336.

PMID:37684354 | DOI:10.1038/s41416-023-02416-6
Primary Angiosarcoma of Lumbar Pedicle and Transverse Process Revealed by FDG PET/CT

Fetched: September 9th, 2023, 5:00am GMT by Pei Yang

Clin Nucl Med. 2023 Oct 1;48(10):905-906. doi: 10.1097/RLU.0000000000004812. Epub 2023 Aug 30.

ABSTRACT

The primary angiosarcoma of bone is rare. It typically occurs in tubular bones, pelvis, and trunk. However, its occurrence in the lumbar pedicle, and transverse process is infrequent. Thus, we present the imaging findings of FDG PET/CT in a rare case of primary angiosarcoma of lumbar pedicle and transverse process. It presented as solitary osteolytic bone destruction in the right pedicle and transverse process of L4 with intense FDG uptake. This case added knowledge of another rare occurrence site of primary angiosarcoma of bone, which should be considered as a differential diagnosis when we meet similar image appearance on FDG PET/CT.

PMID:37682608 | DOI:10.1097/RLU.0000000000004812
Angiosarcoma of the ovary treated with polyadenosine ribose polymerase Inhibition, a case report and review of the literature

Fetched: September 9th, 2023, 5:00am GMT by Andrea M Johnson

Gynecol Oncol Rep. 2023 Aug 23;49:101264. doi: 10.1016/j.gore.2023.101264. eCollection 2023 Oct.

ABSTRACT

•Primary angiosarcoma of the ovary historically has no standard treatment due to its rarity, and outcomes have been variable.•Olaparib may represent a viable treatment option for primary angiosarcoma of the ovary with a somatic BRCA mutation.•Next-generation sequencing may play an important component in treatment of very rare cancers to guide new or uncommon therapies.

PMID:37680504 | PMC:PMC10481173 | DOI:10.1016/j.gore.2023.101264
A Rare Case of Chronic Lymphedema Secondary to Cutaneous Angiosarcoma in a Patient on Hemodialysis

Fetched: September 4th, 2023, 5:00am GMT by A M Carballido Vázquez

Actas Dermosifiliogr. 2023 Sep 3:S0001-7310(23)00684-1. doi: 10.1016/j.ad.2022.09.028. Online ahead of print.

NO ABSTRACT

PMID:37661078 | DOI:10.1016/j.ad.2022.09.028
Untying the Gordian knot of composite hemangioendothelioma: Discovery of novel fusions

Fetched: September 3rd, 2023, 5:01am GMT by Konstantinos Linos

Genes Chromosomes Cancer. 2023 Sep 2. doi: 10.1002/gcc.23198. Online ahead of print.

ABSTRACT

Composite hemangioendothelioma is a rare, locally aggressive, and rarely metastasizing vascular neoplasm which affects both children and adults. Recently, a number of gene fusions including YAP1::MAML2, PTBP1::MAML2, and EPC1::PHC2 have been detected in a small subset of cases with or without neuroendocrine expression. Herein, we present four additional cases with novel in-frame fusions. The cohort comprises two females and two males with a wide age range at diagnosis (24-80 years). Two tumors were deep involving the right brachial plexus and mediastinum, while the remaining were superficial (right plantar foot and abdominal wall). The size ranged from 1.5 to 4.8 cm in greatest dimension. Morphologically, all tumors had an admixture of at least two architectural patterns including retiform hemangioendothelioma, hemangioma, epithelioid hemangioendothelioma, or angiosarcoma. The tumors were positive for endothelial markers CD31 (3/3), ERG (4/4), and D2-40 (1/4, focal), while SMA was expressed in 2/3 highlighting the surrounding pericytes. Synaptophysin showed immunoreactivity in 2/3 cases. One patient had a local recurrence after 40 months, while two patients had no evidence of disease 4 months post-resection. Targeted RNA sequencing detected novel in-frame fusions in each of the cases: HSPG2::FGFR1, YAP1::FOXR1, ACTB::MAML2, and ARID1B::MAML2. The two cases with neuroendocrine expression occurred as superficial lesions and harbored YAP1::FOXR1 and ARID1B::MAML2 fusions. Our study expands on the molecular spectrum of this enigmatic tumor, further enhancing our current understanding of the disease.

PMID:37658696 | DOI:10.1002/gcc.23198

Trial - Angiosarcoma

PARP Inhibition, Stereotactic Body Radiotherapy and Immunotherapy for Metastatic or Advanced Sarcoma (PRIMA): a Prospective Phase 2 Trial

Posted: October 10th, 2023, 2:00am GMT

Conditions: Sarcoma; Sarcoma,Soft Tissue; Sarcoma of Bone
Interventions: Combination Product: Camrelizumab and fluzoparib with concurrent stereotactic body radiotherapy (SBRT)
Sponsors: Ruijin Hospital
Recruiting
Hepatic Small Vessel Neoplasm

Posted: October 4th, 2023, 2:00am GMT

Conditions: Hepatic Small Vessel Neoplasm
Interventions: Other: Imaging
Sponsors: Assistance Publique - Hôpitaux de Paris
Not yet recruiting
Propranolol and Pembrolizumab in Advanced Soft Tissue Sarcoma Patients

Posted: July 27th, 2023, 2:00pm GMT

Conditions: Soft Tissue Sarcoma Adult; Angiosarcoma; Undifferentiated Pleomorphic Sarcoma
Interventions: Drug: Propranolol; Drug: Pembrolizumab
Sponsors: Niels Junker; Aarhus University Hospital; Oslo University Hospital; Karolinska University Hospital
Recruiting
A Study of MQ710 With and Without Pembrolizumab in People With Solid Tumor Cancer

Posted: May 15th, 2023, 2:00pm GMT

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
Recruiting
Phase I Study of TH1 Dendritic Cell Immunotherapy for the Treatment of Cutaneous Angiosarcoma

Posted: April 5th, 2023, 2:00pm GMT

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
Not yet recruiting
Evaluating the Accuracy of Microvessel Ultrasound Imaging for Detecting Extent of Disease in Patients With Angiosarcoma of the Skin

Posted: February 15th, 2023, 3:00pm GMT

Condition: Skin Angiosarcoma
Intervention: Procedure: Ultrasound Microvessel Imaging
Sponsor: Mayo Clinic
Recruiting
A Phase II Study by Using CICS-1 and SPM-011 Commissioned by CICS and STELLA PHARMA

Posted: November 1st, 2022, 2:00am GMT

Conditions: Unresectable Angiosarcoma
Interventions: Radiation: BNCT
Sponsors: Stella Pharma Corporation; Cancer Intelligence Care Systems, Inc.
Recruiting
Sintilimab for the Treatment of Locally Advanced, Metastatic, or Recurrent Angiosarcoma, the SiARa Cancer Study

Posted: August 30th, 2021, 2:00pm GMT

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 TTI-621) Given Alone and in Combination With Doxorubicin in People With Leiomyosarcoma

Posted: August 9th, 2021, 2:00pm GMT

Conditions: Leiomyosarcoma
Interventions: Drug: Ontorpacept (TTI-621); Drug: Doxorubicin
Sponsors: Pfizer
Active, not recruiting
Lenvatinib and Pembrolizumab in People With Advanced Soft Tissue Sarcoma

Posted: March 5th, 2021, 3:00pm GMT

Conditions: Advanced Sarcoma
Interventions: Drug: Lenvatinib; Drug: Pembrolizumab
Sponsors: Memorial Sloan Kettering Cancer Center; Merck Sharp & Dohme LLC; Parker Institute for Cancer Immunotherapy
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, 3:00pm GMT

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
Propranolol in Angiosarcoma

Posted: August 19th, 2020, 2:00am GMT

Conditions: Angiosarcoma
Interventions: Drug: Propranolol
Sponsors: The Netherlands Cancer Institute; Anticancer Fund, Belgium
Completed
Testing the Addition of Nivolumab to Chemotherapy in Treatment of Soft Tissue Sarcoma

Posted: April 9th, 2020, 2:00am GMT

Conditions: Skin Angiosarcoma; Skin Radiation-Related Angiosarcoma; Visceral Angiosarcoma
Interventions: Procedure: Biospecimen Collection; Drug: Cabozantinib S-malate; Procedure: Computed Tomography; Procedure: FDG-Positron Emission Tomography; Procedure: Magnetic Resonance Imaging; Biological: Nivolumab; Drug: Paclitaxel; Other: Quality-of-Life Assessment; Other: Questionnaire Administration
Sponsors: National Cancer Institute (NCI)
Active, not recruiting
Efficacy and Safety of Regorafenib as Maintenance Therapy After First-line Treatment in Patients With Bone Sarcomas

Posted: August 13th, 2019, 2:00pm GMT

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

Posted: April 19th, 2019, 2:00pm GMT

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

Posted: March 1st, 2019, 3:00pm GMT

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
A Study of NKTR-214 in Combination With Nivolumab in Patients With Metastatic and/or Locally Advanced Sarcoma

Posted: September 13th, 2017, 2:00am GMT

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, 2:00pm GMT

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
Sponsor: Grupo Espanol de Investigacion en Sarcomas
Recruiting
A Study of Talimogene Laherparepvec (T-VEC) in Combination With Pembrolizumab in Patients With Metastatic and/or Locally Advanced Sarcoma

Posted: March 3rd, 2017, 3:00am GMT

Conditions: Sarcoma; Epithelioid Sarcoma; Cutaneous Angiosarcoma
Interventions: Drug: Talimogene Laherparepvec (T-VEC); Drug: Pembrolizumab
Sponsors: Memorial Sloan Kettering Cancer Center; Amgen; Merck Sharp & Dohme LLC
Active, not recruiting
Multi-Arm Study to Test the Efficacy of Immunotherapeutic Agents in Multiple Sarcoma Subtypes

Posted: June 28th, 2016, 2:00am GMT

Conditions: Advanced and/or Metastatic Sarcoma
Interventions: Drug: Durvalumab; Drug: Tremelimumab
Sponsors: M.D. Anderson Cancer Center; MedImmune LLC
Active, not recruiting
Doxorubicin With Upfront Dexrazoxane for the Treatment of Advanced or Metastatic Soft Tissue Sarcoma

Posted: October 22nd, 2015, 2:00am GMT

Conditions: Sarcoma, Soft Tissue; Soft Tissue Sarcoma; Undifferentiated Pleomorphic Sarcoma; Leiomyosarcoma; Liposarcoma; Synovial Sarcoma; Myxofibrosarcoma; Angiosarcoma; Fibrosarcoma; Malignant Peripheral Nerve Sheath Tumor; Epithelioid Sarcoma
Interventions: Drug: Dexrazoxane; Drug: Doxorubicin
Sponsors: Washington University School of Medicine
Completed
I-SPY TRIAL: Neoadjuvant and Personalized Adaptive Novel Agents to Treat Breast Cancer

Posted: January 5th, 2010, 3:00pm GMT

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
Sponsor: QuantumLeap Healthcare Collaborative
Recruiting

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Rare Cancer News & Clinical Trials » Angiosarcoma

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Angiosarcoma (222 unread)

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