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PubMed - Angiosarcoma (29 unread)

Face to a bilateral pneumothorax, just scratch your head!

Fetched: January 27th, 2023, 5:00am GMT by S Valery

Rev Mal Respir. 2023 Jan 24:S0761-8425(23)00026-8. doi: 10.1016/j.rmr.2023.01.003. Online ahead of print.

ABSTRACT

Head and neck skin angiosarcoma is a rare and aggressive tumor (1 % of head and neck tumors). Prognosis remains poor, with a 5-year survival rate ranging from 10 to 54%, depending on the initial stage. Metastatic disease markedly worsens the prognosis. Metastatic lung involvement is classic and can take on several forms. The cystic form is responsible for numerous complications, particularly pneumothorax. In this case, an 83-year-old patient was diagnosed with bilateral pneumothorax complicating cystic interstitial lung disease, which was revealed by hemoptoic sputum. Skin examination revealed two large necrotic lesions of the calvaria. Anatomo-pathological examination confirmed cutaneous angiosarcoma on both skin biopsy and lung resection. At a metastatic stage, only systemic treatment with paclitaxel can be proposed. The clinical course was unfavorable, leading to death before any specific treatment. This observation highlights the importance of a complete clinical skin examination in the assessment of pulmonary cystic lesions.

PMID:36702687 | DOI:10.1016/j.rmr.2023.01.003
Small intestinal angiosarcoma on clinical presentation, diagnosis, management and prognosis: A case report and review of the literature

Fetched: January 24th, 2023, 5:00am GMT by Xiao-Mei Ma

World J Gastroenterol. 2023 Jan 21;29(3):561-578. doi: 10.3748/wjg.v29.i3.561.

ABSTRACT

BACKGROUND: Angiosarcoma is a highly malignant soft-tissue sarcoma derived from vascular endothelial cells that mainly occurs in the skin and subcutaneous tissues. Small-intestinal angiosarcomas are rare, and the prognosis is poor.

CASE SUMMARY: We reported a case of primary multifocal ileal angiosarcoma and analyze previously reported cases to improve our understanding of small intestinal angiosarcoma. Small intestinal angiosarcoma is more common in elderly and male patients. Gastrointestinal bleeding, anemia, abdominal pain, weakness, and weight loss were the common symptoms. CD31, CD34, factor VIII-related antigen, ETS-related gene, friend leukemia integration 1, and von Willebrand factor are valuable immunohistochemical markers for the diagnosis of small-intestinal angiosarcoma. Small-intestinal angiosarcoma most commonly occurs in the jejunum, followed by the ileum and duodenum. Radiation and toxicant exposure are risk factors for angiosarcoma. After a definite diagnosis, the mean and median survival time was 8 mo and 3 mo, respectively. Kaplan-Meier survival analysis showed that age, infiltration depth, chemotherapy, and the number of small intestinal segments invaded by tumor lesions were prognostic factors for small intestinal angiosarcoma. Multivariate Cox regression analysis showed that chemotherapy and surgery significantly improved patient prognosis.

CONCLUSION: Angiosarcoma should be considered for unexplained melena and abdominal pain, especially in older men and patients with a history of radiation exposure. Prompt treatment, including surgery and adjuvant chemotherapy, is essential to prolonging patient survival.

PMID:36688020 | PMC:PMC9850938 | DOI:10.3748/wjg.v29.i3.561
Angiosarcoma in Chronic Filarial Lymphedema - A Report of Two Cases

Fetched: January 24th, 2023, 5:00am GMT by Amar Kamat

J Orthop Case Rep. 2022 Aug;12(8):110-114. doi: 10.13107/jocr.2022.v12.i08.2988.

ABSTRACT

INTRODUCTION: Cutaneous angiosarcoma is an uncommon aggressive tumor known to arise in chronic lymphedema of various etiologies with post mastectomy lymphedema being the most common cause also known as Stewart-Treves syndrome. Chronic filarial lymphedema is the most uncommon etiology for development of angiosarcoma.

CASE REPORT: We describe two cases of angiosarcoma developing in a setting of long-standing filarial lymphedema. The patient presented with the same clinical features of rapidly progressing nodules.

CONCLUSION: With India harboring most of the filarial load, this scenario should be kept in mind by clinicians for early diagnosis and treatment. Angiosarcoma is a rapidly progressing tumor and is fatal; hence, an idea about its diagnosis is of utmost importance.

PMID:36687479 | PMC:PMC9831239 | DOI:10.13107/jocr.2022.v12.i08.2988
Can depressed cancer patients with a borderline thiamine concentration develop deficiency within a short time period?

Fetched: January 24th, 2023, 5:00am GMT by Nozomu Uchida

Palliat Support Care. 2023 Jan 23:1-4. doi: 10.1017/S1478951522001808. Online ahead of print.

ABSTRACT

BACKGROUND: Despite increasing reports of thiamine deficiency (TD) among cancer patients, there remain some patients with borderline thiamine concentrations (BTC). However, it is unclear whether such patients subsequently develop TD.

METHODS: Here, we report cases of cancer patients progressing to TD within a short time period after presentation with BTC (24-28 ng/ml).

CASE 1: A 49-year-old female with lung cancer. During treatment for depression, the patient showed a decreased appetite, and a blood sample revealed BTC (25 ng/ml). Fourteen days later, she reported a continued loss of appetite, and despite the absence of the 3 classical signs of Wernicke encephalopathy (WE), additional testing showed a thiamine level of 23 ng/ml, leading to a diagnosis of TD.

CASE 2: A 65-year-old female developed depression during chemotherapy for angiosarcoma. Her blood sample revealed BTC (25 ng/ml). Seven days later, despite the absence of the classical signs of WE, a further testing revealed a thiamine level of 20 ng/ml.

CASE 3: A 41-year-old female developed depression during chemotherapy for ovarian cancer. No loss of appetite was observed, but a blood sample revealed BTC (25 ng/ml). Seven days later, despite the absence of the classical signs of WE or decreased appetite, further testing revealed a thiamine level of 19 ng/ml.

SIGNIFICANCE OF RESULTS: Depressed cancer patients with BTC may develop TD within a short time frame. To prevent TD, health-care professionals should maintain an awareness of its potential and the need for regular testing of thiamine level or prophylactic replacement therapy.

PMID:36683386 | DOI:10.1017/S1478951522001808
Genomic Characterization of Rare Primary Cardiac Sarcoma Entities

Fetched: January 22nd, 2023, 5:01am GMT by Livia Gozzellino

Diagnostics (Basel). 2023 Jan 6;13(2):214. doi: 10.3390/diagnostics13020214.

ABSTRACT

Primary cardiac sarcomas are considered rare malignant entities associated with poor prognosis. In fact, knowledge regarding their gene signature and possible treatments is still limited. In our study, whole-transcriptome sequencing on formalin-fixed paraffin-embedded (FFPE) samples from one cardiac osteosarcoma and one cardiac leiomyosarcoma was performed, to investigate their mutational profiles and to highlight differences and/or similarities to other cardiac histotypes. Both cases have been deeply detailed from a pathological point of view. The osteosarcoma sample presented mutations involving ATRX, ERCC5, and COL1A1, while the leiomyosarcoma case showed EXT2, DNM2, and PSIP1 alterations. Altered genes, along with the most differentially expressed genes in the leiomyosarcoma or osteosarcoma sample versus the cardiac angiosarcomas and intimal sarcomas (e.g., YAF2, PAK5, and CRABP1), appeared to be associated with cell growth, proliferation, apoptosis, and the repair of DNA damage, which are key mechanisms involved in tumorigenesis. Moreover, a distinct gene expression profile was detected in the osteosarcoma sample when compared to other cardiac sarcomas. For instance, WIF1, a marker of osteoblastic differentiation, was upregulated in our bone tumor. These findings pave the way for further studies on these entities, in order to identify targeted therapies and, therefore, improve patients' prognoses.

PMID:36673024 | PMC:PMC9858520 | DOI:10.3390/diagnostics13020214
Hemorrhage and Sudden Death in a Cat with Pancreatic Hemangiosarcoma

Fetched: January 21st, 2023, 5:00am GMT by Corina Toma

Vet Sci. 2022 Dec 24;10(1):8. doi: 10.3390/vetsci10010008.

ABSTRACT

A 15-year-old female, indoor, spayed, domestic shorthair cat was presented for post-mortem evaluation with a history of sudden death. A red-brown 3 cm x 4 cm neoplastic mass was identified within the body of the pancreas, along with multiple similar nodular structures within the omentum and liver. Associated lesions included hemoperitoneum, yellow discoloration of the peritoneal wall, and severe anemia. Spindle-shaped neoplastic cells exhibiting malignancy features, which occasionally contained within the cytoplasm haematoidin crystals and/or red blood cells, were observed during cytological examination. Histologically, the neoplastic cells were organized in short streams forming vascular spaces filled with erythrocytes. Immunohistochemically, the neoplastic cells were immunolabeled for vimentin and CD31, supporting the diagnosis of hemangiosarcoma. This report offers a complete post-mortem and histological evaluation of a rare tumor in cats with an unusual location and a comparative assessment of 3 anti-CD31 antibodies.

PMID:36669009 | PMC:PMC9863512 | DOI:10.3390/vetsci10010008
Primary Angiosarcoma of the Spleen: An Aggressive Neoplasm

Fetched: January 21st, 2023, 5:00am GMT by Mira Damouny

World J Oncol. 2022 Dec;13(6):337-342. doi: 10.14740/wjon1542. Epub 2022 Dec 24.

ABSTRACT

Primary tumors of the spleen are rare, with an incidence rate of about 0.1%. These tumors could be benign, usually asymptomatic, or malignant which are usually symptomatic with abdominal pain being the most common symptom. Lymphoid neoplasms are the most common primary splenic tumors. Primary angiosarcoma is one of the extremely rare malignant vascular neoplasms of the spleen, which carries a dismal prognosis. It constitutes almost 7.4% of all primary malignant splenic neoplasms and is well known as an aggressive tumor with high local recurrence and distant metastasis rates. Overall survival is up to 12 months following diagnosis, regardless of management strategy. Due to the broad differential diagnosis of splenic tumors, this tumor is often forgotten, and is very challenging to diagnose early. Less than 300 cases of primary splenic angiosarcoma have been reported in the English literature. The main issue of this article is to review the current English literature to figure out the characteristic demographic features, clinical presentation, imaging findings and management of such tumors, in order to increase awareness of the treating physicians to improve diagnosis, management, as well as overall survival.

PMID:36660212 | PMC:PMC9822683 | DOI:10.14740/wjon1542
Malignant primary and metastatic cardiac tumors - a single-center 27-year case review

Fetched: January 20th, 2023, 5:00am GMT by Pedro Brás

Oncology. 2023 Jan 19. doi: 10.1159/000528915. Online ahead of print.

ABSTRACT

BACKGROUND: Malignant primary cardiac tumors are exceedingly rare, and despite surgical exeresis or chemotherapy, their prognosis remains poor. Cardiac invasion by metastatic tumors, while more common, also entails an unsatisfactory outcome. This study aimed to review patients diagnosed with malignant primary and secondary cardiac tumors in a tertiary center between 1995 and 2022.

METHODS: Clinical data, echocardiographic, computed tomography, and magnetic resonance assessments of tumor location and morphology, histology, treatment, and survival were retrospectively analyzed.

RESULTS: Sixty malignant cardiac tumors were diagnosed: 17 primary (A) and 43 metastatic (B) tumors. A: The most common types were angiosarcoma (41%), undifferentiated sarcoma (23%), and fibrosarcoma (18%). Patients with primary tumors were younger than patients with metastatic tumors (41 ± 13 years vs. 57 ± 18 years, p = 0.001), with no significant gender difference. The most frequent presentations were heart failure (59%) and arrhythmia (23%). The most prevalent tumor location was the right heart chambers (71%), mostly in the right atrium (35%). 47% were submitted to tumor resection, and 29% received chemotherapy. The mortality rate was 82% with a median survival of 6.0 (IQR 1.0-11.8) months after diagnosis (minimum of 12 days and maximum of 19 years). One patient with fibrosarcoma underwent heart transplantation and was still alive and well after 19 years. B: Regarding metastatic cardiac invasion, the most common primary tumor sites were lung carcinomas (38%), thymomas (17%), and lymphomas (14%). Presentation with pericardial effusion was common (33%). The mortality rate was 72%, with a median survival of 3.6 (1.0-13.4) months (minimum of 7 days, maximum of 5 years).

CONCLUSION: Diagnosis of metastatic cardiac tumors was more common than malignant primary tumors, both with a dismal prognosis. When radical exeresis is not possible, heart transplantation can be an option with a favourable outcome in carefully selected patients with sarcomas.

PMID:36657399 | DOI:10.1159/000528915
A rare coexistence: Poland's syndrome and cardiac angiosarcoma

Fetched: January 20th, 2023, 5:00am GMT by Fatih Kizilyel

J Cardiothorac Surg. 2023 Jan 18;18(1):35. doi: 10.1186/s13019-023-02138-z.

ABSTRACT

Poland's syndrome, a rare genetic disorder that accompanies malignancies, musculoskeletal disorders, cardiac and genitourinary syndromes. There is no study that represents the association between cardiac angiosarcoma and Poland's syndrome. A 24-year-old female patient previously diagnosed with Poland's syndrome was admitted to our hospital complaining of dyspnea. Diagnostic imaging showed an irregular mass in the right atrial cavity. After successful surgery, she was discharged uneventfully and the 3rd month oncologic follow-up reveals none of residual mass. The coexistence has not been diagnosed and treated in a cardiac surgery department before. With this presentation, we aimed to contribute to the literature with this presentation, for the right and early diagnosis and management of possible new cases in the future can be diagnosed and treated correctly and early.

PMID:36653856 | PMC:PMC9847129 | DOI:10.1186/s13019-023-02138-z
Cutaneous Angiosarcoma

Fetched: January 19th, 2023, 5:00am GMT by Jason C Klein

JAMA Dermatol. 2023 Jan 18. doi: 10.1001/jamadermatol.2022.5446. Online ahead of print.

NO ABSTRACT

PMID:36652219 | DOI:10.1001/jamadermatol.2022.5446
Cutaneous angiosarcoma of the head: a case from Italy

Fetched: January 19th, 2023, 5:00am GMT by Dario A Marletta

Ital J Dermatol Venerol. 2022 Dec;157(6):534-535. doi: 10.23736/S2784-8671.22.07346-7.

NO ABSTRACT

PMID:36651213 | DOI:10.23736/S2784-8671.22.07346-7
Multiply Recurrent Composite Hemangioendothelioma of Penis with Histologic Progression to High-Grade Features

Fetched: January 18th, 2023, 5:00am GMT by Chau M Bui

Dermatopathology (Basel). 2023 Jan 11;10(1):41-45. doi: 10.3390/dermatopathology10010005.

ABSTRACT

Composite hemangioendothelioma (CHE) is a very rare low-grade malignant vascular neoplasm. Here, we present the first case of it occurring on a penis with two local recurrences over a 9 year span and its progression to a high-grade morphology.

PMID:36648783 | PMC:PMC9844429 | DOI:10.3390/dermatopathology10010005
Timely adjuvant chemotherapy improves outcome in dogs with non-metastatic splenic hemangiosarcoma undergoing splenectomy

Fetched: January 13th, 2023, 5:00am GMT by Eugenio Faroni

Vet Comp Oncol. 2023 Jan 12. doi: 10.1111/vco.12875. Online ahead of print.

ABSTRACT

Timely delivery of adjuvant chemotherapy has been shown to be advantageous in many human cancers and canine osteosarcoma. Adjuvant chemotherapy has been shown to improve outcome for canine splenic hemangiosarcoma. The aim of this retrospective study was to investigate whether timely adjuvant chemotherapy administration resulted in better outcome in dogs with non-metastatic splenic hemangiosarcoma undergoing splenectomy. Medical records were searched for dogs with non-metastatic, splenic hemangiosarcoma that received splenectomy and adjuvant chemotherapy. The number of days from surgery to the first chemotherapy dose (StoC) was evaluated to identify the cut-off value associated with the best survival advantage. StoC and other possible prognostic factors were tested for influence on time to metastasis (TTM) and overall survival (OS). Seventy dogs were included. Median StoC was 20 days (range: 4-70). The time interval associated with the greatest survival benefit was 21 days. Median TTM and OS of dogs with StoC ≤ 21 days were significantly longer than those with StoC >21 days (TTM: 163 vs. 118 days, p = .001; OS: 238 vs. 146 days, p < .001). On multivariable analysis, StoC >21 days was the only variable significantly associated with increased risk of tumour progression (HR 2.1, p = .010) and death (HR 2.3; p = .008). Starting adjuvant chemotherapy within 21 days of surgery may be associated with a survival benefit in dogs with non-metastatic splenic hemangiosarcoma, possibly due to the early targeting of newly recruited metastatic cells after surgery.

PMID:36633399 | DOI:10.1111/vco.12875
"Time Shall Unfold…": The Clinical Value of Grading Mammary Angiosarcoma

Fetched: January 10th, 2023, 5:00am GMT by Syed A Hoda

Am J Surg Pathol. 2023 Jan 10. doi: 10.1097/PAS.0000000000002015. Online ahead of print.

NO ABSTRACT

PMID:36624551 | DOI:10.1097/PAS.0000000000002015
Advances in molecular pathology of primary cardiac angiosarcoma

Fetched: January 10th, 2023, 5:00am GMT by R Liu

Zhonghua Bing Li Xue Za Zhi. 2023 Jan 8;52(1):87-90. doi: 10.3760/cma.j.cn112151-20220531-00477.

ABSTRACT

原发性心脏血管肉瘤（primary cardiac angiosarcoma，PCAS）十分罕见，恶性程度高，患者常表现为非特异性症状，早期诊断困难，预后差。目前对于PCAS治疗主要以手术切除为主并辅以放化疗，但远期生存情况尚不理想。了解心脏血管肉瘤的分子病理研究进展，寻找靶向治疗的可能有效靶点，是目前延长PCAS患者生存期的希望。.

PMID:36617920 | DOI:10.3760/cma.j.cn112151-20220531-00477
Clinical, Dermoscopic, Ultrasonographic, and Histopathologic Correlations in Kaposi's Sarcoma Lesions and Their Differential Diagnoses: A Single-Center Prospective Study

Fetched: January 9th, 2023, 5:00am GMT by Athanasia Tourlaki

J Clin Med. 2022 Dec 29;12(1):278. doi: 10.3390/jcm12010278.

ABSTRACT

(1) Background: Kaposi's sarcoma (KS) is an angioproliferative neoplasm typically appearing as angiomatous patches, plaques, and/or nodules on the skin. Dermoscopy and ultrasonography have been suggested as an aid in the diagnosis of KS, but there is little evidence in the literature, especially regarding its possible differential diagnoses. Our aim is to describe and compare the clinical, dermoscopic, and ultrasonographic features of KS and KS-like lesions. (2) Methods: we conducted a prospective study on 25 consecutive patients who were first referred to our tertiary care center from January to May 2021 for a possible KS. (3) Results: 41 cutaneous lesions were examined by means of dermoscopy, Doppler ultrasonography, and pathology, 32 of which were KS-related, while the remaining 9 were lesions with clinical resemblance to KS. On dermoscopy, a purplish-red pigmentation, scaly surface, and the collarette sign were the most common features among KS lesions (81.3%, 46.9%, and 28.1%, respectively). On US, all 9 KS plaques and 21 KS nodules presented a hypoechoic image. Dermoscopic and Doppler ultrasonographic findings of KS-like lesions, such as cherry angioma, venous lake, glomus tumor, pyogenic granuloma, and angiosarcoma were also analyzed. (4) Conclusions: dermoscopy and Doppler ultrasonography can be useful to better assess the features of KS lesions and in diagnosing equivocal KS-like lesions.

PMID:36615078 | PMC:PMC9821103 | DOI:10.3390/jcm12010278
MYC amplification in angiosarcoma depends on etiological/clinical subgroups - Diagnostic and prognostic value

Fetched: January 9th, 2023, 5:00am GMT by A G Hogeboom-Gimeno

Ann Diagn Pathol. 2022 Dec 28;63:152096. doi: 10.1016/j.anndiagpath.2022.152096. Online ahead of print.

NO ABSTRACT

PMID:36610315 | DOI:10.1016/j.anndiagpath.2022.152096
Primary Hepatic Angiosarcoma: A Rare and Very Aggressive Liver Tumour

Fetched: January 5th, 2023, 5:00am GMT by Selahattin Durmaz

J Coll Physicians Surg Pak. 2022 Dec;32(12):SS209-SS211. doi: 10.29271/jcpsp.2022.Supp.S209.

ABSTRACT

Primary hepatic angiosarcoma (PHA) is a sporadic and aggressive tumour of the liver that originates from mesenchymal cells and represents less than 2% of all primary liver tumours. It is known to be associated with several environmental and industrial carcinogens; however, in 75% of cases, aetiology remains unclear. Patients generally present with nonspecific symptoms and laboratory findings. Imaging has a limited role in the diagnosis. We herein present a case of a 52-year-old man with a history of hepatitis B-related cirrhosis who was referred to our hospital for liver transplantation assessment. Magnetic resonance imaging (MRI) revealed two small nodular lesions of 5 and 6 mm in segment IV of the liver, categorised as Liver Imaging Reporting and Data System (LI-RADS) category 3. The patient was discussed at a multidisciplinary tumour meeting, and an MRI follow-up in three months was planned. Three months later, MRI depicted a substantial increase in the lesion size measured 8.5 cm. An ultrasound-guided tru-cut biopsy was performed, and the diagnosis of PHA was confirmed by pathology. In this report, we aim to highlight PHA's MRI features and underline this rare entity's rapid and fatal progression.

PMID:36597341 | DOI:10.29271/jcpsp.2022.Supp.S209
The Role of Imaging in the Diagnosis of Primary and Secondary Breast Angiosarcoma: Twenty-Five-Year Experience of a Provincial Cancer Institution

Fetched: December 28th, 2022, 5:00am GMT by Helena Bentley

Clin Breast Cancer. 2022 Nov 17:S1526-8209(22)00248-8. doi: 10.1016/j.clbc.2022.11.003. Online ahead of print.

ABSTRACT

BACKGROUND: Breast angiosarcoma may arise spontaneously (primary breast angiosarcoma (PBA)) or may arise secondary to a biological insult, such as radiation therapy (secondary breast angiosarcoma (SBA)). We evaluated the imaging findings of patients diagnosed with PBA and SBA within the province of British Columbia, Canada.

MATERIALS AND METHODS: This was a multi-center, retrospective study of patients diagnosed with PBA and SBA over a 25-year period. Patients were identified via a provincial database which registers all cases of sarcoma. Patients diagnosed with histologically proven PBA and SBA were eligible for inclusion. Multimodal breast imaging reviewed included mammography, ultrasound, magnetic resonance imaging, and computed tomography.

RESULTS: Thirteen patients were diagnosed with PBA and 22 patients were diagnosed with SBA. The median (interquartile range (IQR)) age of patients diagnosed with PBA (45.5 years (19.7 years)) was less than that of patients diagnosed with SBA (75.8 years (13.8 years), P < .001). Patients diagnosed with PBA (90.9%) were more likely to present with a parenchymal mass clinically and radiographically than those with SBA (28.6%, P < .002). Patients diagnosed with SBA (71.4%) were more likely to present with cutaneous findings than patients diagnosed with PBA (0.0%, P < .05). Without specific clinical context, the imaging findings of PBA and SBA were observed to be non-specific.

CONCLUSION: This is the only study which evaluated the imaging findings of patients diagnosed with PBA and SBA within a large, defined geographical area. Given non-specific imaging findings, awareness of the disease and clear and timely communication between radiologists and clinicians is required to ensure appropriate diagnosis and management.

PMID:36575102 | DOI:10.1016/j.clbc.2022.11.003
Multiple Gastrointestinal Angiosarcoma Mimicking Kaposi's Sarcoma

Fetched: December 28th, 2022, 5:00am GMT by Takuto Saiki

Intern Med. 2022 Dec 28. doi: 10.2169/internalmedicine.1177-22. Online ahead of print.

NO ABSTRACT

PMID:36575019 | DOI:10.2169/internalmedicine.1177-22
Primary Breast Angiosarcoma: Comparative Transcriptome Analysis

Fetched: December 24th, 2022, 5:01am GMT by Andrés Rincón-Riveros

Int J Mol Sci. 2022 Dec 16;23(24):16032. doi: 10.3390/ijms232416032.

ABSTRACT

Primary breast angiosarcoma, with de novo appearance and not associated with exposure to radiation or lymphedema, is a rare pathology representing less than 0.05% of the neoplasms related to this organ. The pathology is characterized by its aggressiveness, poor prognosis, and difficulties in its differential diagnosis. This article reports the case of a 55-year-old white woman with no family history of cancer, with a rapidly growing mass in the left mammary gland that ulcerates and bleeds. It is confirmed as primary breast angiosarcoma by immunostaining in the tumor tissue for CD31, CD34, and FLI-1. In addition, a sample of neoplastic and healthy tissues is collected from the patient for RNA sequencing; the results are contrasted with a tissue sample from a patient with Luminal A subtype of breast cancer, as well as data from other cases of angiosarcoma available in public databases. These findings revealed a genetic profile associated with the immune and inflammatory response in the patient's sample when compared to available angiosarcoma data; these molecular patterns are consistent with other recent studies. Due to the rarity of the disease, the studies carried out on each patient contribute to the expanding knowledge of the etiology and molecular pathways that are still partially known and continue to be the subject of research. Aside from a comparative transcriptome study, this article aims to provide an update on the state of knowledge about this disease.

PMID:36555675 | PMC:PMC9781631 | DOI:10.3390/ijms232416032
Pilot safety evaluation of doxorubicin chemotherapy combined with non-specific immunotherapy (Immunocidin®) for canine splenic hemangiosarcoma

Fetched: December 23rd, 2022, 5:00am GMT by Margaret L Musser

PLoS One. 2022 Dec 22;17(12):e0279594. doi: 10.1371/journal.pone.0279594. eCollection 2022.

ABSTRACT

Canine splenic hemangiosarcoma (HSA) is an aggressive tumor with a short overall survival time (OST) despite treatment with splenectomy and adjuvant doxorubicin. Modulation of the immune system has been shown to be effective for a variety of human tumors, and may be effective for canine tumors, including HSA. Immunocidin® is a non-specific immunotherapy based on a mycobacterial cell wall fraction. Preliminary work suggests Immunocidin® is safe to give intravenously (IV) in tumor-bearing dogs. This work aimed to evaluate the safety of doxorubicin and Immunocidin® combination in dogs with naturally occurring splenic HSA. A secondary aim of this study was to collect preliminary efficacy data to support a subsequent comprehensive, prospective clinical trial in canine patients with HSA, if the combination of doxorubicin and Immunocidin® was found to be safe. Eighteen dogs with stage II-III splenic HSA were recruited to receive 5 doses of sequential IV doxorubicin and Immunocidin® at two-week intervals following splenectomy. Adverse events (AEs) were graded according to the Veterinary Cooperative Oncology Group v1.1 (VCOG) scheme. Overall survival time was calculated from the date of splenectomy to date of death or loss to follow-up. AEs during administration were infrequent, the most common being hypertension. One patient developed limb and facial twitching and was removed from the study. After infusion, common AEs included lethargy, hyporexia, and diarrhea. One patient developed VCOG grade 5 diarrhea, thrombocytopenia, and anemia. Modifications in the treatment regimen were made to prevent these signs in subsequent patients. The median OST in dogs treated with the combination therapy was estimated at 147 days (range: 39-668 days). Although generally safe, the combination of doxorubicin and Immunocidin® appeared to cause more gastrointestinal effects than doxorubicin alone, and no apparent improvement in OST was noted in this population of dogs.

PMID:36548371 | PMC:PMC9778498 | DOI:10.1371/journal.pone.0279594
Echocardiographic identification of right atrial mass: a case of cardiac angiosarcoma

Fetched: December 23rd, 2022, 5:00am GMT by Jing Li

Med Ultrason. 2022 Dec 21;24(4):505-506. doi: 10.11152/mu-3780.

NO ABSTRACT

PMID:36546440 | DOI:10.11152/mu-3780
Population-based analysis of POT1 variants in a cutaneous melanoma case-control cohort

Fetched: December 21st, 2022, 5:00am GMT by Irving Simonin-Wilmer

J Med Genet. 2022 Dec 20:jmg-2022-108776. doi: 10.1136/jmg-2022-108776. Online ahead of print.

ABSTRACT

Pathogenic germline variants in the protection of telomeres 1 gene (POT1) have been associated with predisposition to a range of tumour types, including melanoma, glioma, leukaemia and cardiac angiosarcoma. We sequenced all coding exons of the POT1 gene in 2928 European-descent melanoma cases and 3298 controls, identifying 43 protein-changing genetic variants. We performed POT1-telomere binding assays for all missense and stop-gained variants, finding nine variants that impair or disrupt protein-telomere complex formation, and we further define the role of variants in the regulation of telomere length and complex formation through molecular dynamics simulations. We determine that POT1 coding variants are a minor contributor to melanoma burden in the general population, with only about 0.5% of melanoma cases carrying germline pathogenic variants in this gene, but should be screened in individuals with a strong family history of melanoma and/or multiple malignancies.

PMID:36539277 | DOI:10.1136/jmg-2022-108776
The complex management of the breast angiosarcoma: a retrospective study

Fetched: December 21st, 2022, 5:00am GMT by Lucia Cannella

Oncology. 2022 Dec 20. doi: 10.1159/000525146. Online ahead of print.

ABSTRACT

BACKGROUND/AIM: Breast angiosarcoma is a rare and aggressive disease with a poor prognosis. Two subtypes have been identified: primary angiosarcoma (PBA) and secondary breast angiosarcoma (SBA). In this retrospective analysis, we describe and compare our institute experience with the data existing in the literature.

MATERIALS AND METHODS: We included in our analysis 29 patients who received a diagnosis of PBA or SBA between 2006 and 2019.

RESULTS: All patients received surgery as frontline treatment, but only 6 patients underwent to adjuvant treatment. Neoadjuvant chemotherapy was administeredon 2 patients. The preferred chemotherapeutic regimen was taxanes with or without gemcitabine, andassociated with anthracyclines. A lower median RFS and OS was reported in patients with PBA compared to those with SBA, but the difference observed was not statistically significant. Patients with PBA had alower median age at the diagnosis (38 vs 75).

CONCLUSION: In our analysis we have shown a lower median RFS and OS in patients with PBA compared with those with SBA, and a significantly younger age at diagnosis in patients affected by PBA.

PMID:36538913 | DOI:10.1159/000525146
Primary angiosarcoma of the ascending colon diagnosed after the discovery of intraoral tumor

Fetched: December 21st, 2022, 5:00am GMT by Masaya Tojo

Clin J Gastroenterol. 2022 Dec 20. doi: 10.1007/s12328-022-01745-y. Online ahead of print.

ABSTRACT

Here, we report a case of primary angiosarcoma of the ascending colon diagnosed after the discovery of an oral tumor. An 86-year-old woman presented to our hospital with severe anemia. Although she did not report any oral symptoms, an intraoral mass 15 mm in size with ulceration was observed. Since colonic tumors and osteolytic tumors in the maxilla and sacra were also identified by fluorodeoxyglucose (FDG)-positron emission tomography (PET), colonoscopy was performed. A semi-peripheral tumor with ulceration from the cecum to the ascending colon was detected. Biopsies of the oral cavity and colon revealed a poorly differentiated tumor, and multiple additional immunohistochemical stains were performed to confirm the diagnosis of angiosarcoma. Angiosarcoma progresses rapidly and has a very poor prognosis. Hence, although it is rare, angiosarcoma should always be considered in the differential diagnoses of malignancy of the gastrointestinal tract.

PMID:36538257 | DOI:10.1007/s12328-022-01745-y
Primary adrenal anastomosing haemangioma

Fetched: December 21st, 2022, 5:00am GMT by Zhi-Bo Zuo

Endokrynol Pol. 2022 Dec 20. doi: 10.5603/EP.a2022.0091. Online ahead of print.

ABSTRACT

Not required for Clinical Vignette.

PMID:36537188 | DOI:10.5603/EP.a2022.0091
Angiosarcoma arising in a schwannoma of the peripheral nervous system: illustrative case

Fetched: December 21st, 2022, 5:00am GMT by Nikita Lakomkin

J Neurosurg Case Lessons. 2022 Dec 19;4(25):CASE22452. doi: 10.3171/CASE22452. Print 2022 Dec 19.

ABSTRACT

BACKGROUND: Schwannomas of the peripheral nerves are benign tumors that can very rarely undergo malignant transformation. These lesions are particularly challenging to diagnose via noninvasive techniques but can have significant implications for treatment.

OBSERVATIONS: This is a case of a 70-year-old female with a prior history of a right sciatic notch tumor that was diagnosed as a conventional schwannoma via histology from an initial biopsy and subsequent surgical debulking. Unfortunately, she experienced significant worsening of her motor deficit, whereby her postoperative foot weakness progressed to complete foot drop in less than 2 years. In addition, she demonstrated significant radiological progression, with more than 1 to 2 cm of growth in each dimension at her subsequent evaluation, along with intractable right leg pain. An additional operation was performed to completely remove the 7 × 8 cm tumor, and histology demonstrated angiosarcoma within a schwannoma. There was no evidence of recurrence at 15 months, and the patient had significant improvement in her pain.

LESSONS: Rapidly worsening function and radiological progression are not typically seen with conventional benign nerve sheath tumors and should prompt consideration of other lesions. Angiosarcoma within schwannoma is a rare pathology and optimal therapies for these tumors in terms of surgical timing and adjuvant therapy are still unknown.

PMID:36536527 | PMC:PMC9764372 | DOI:10.3171/CASE22452
Neuroinflammation and neovascularization in diabetic eye diseases (DEDs): identification of potential pharmacotherapeutic targets

Fetched: December 16th, 2022, 5:00am GMT by Siva Prasad Panda

Mol Biol Rep. 2022 Dec 13. doi: 10.1007/s11033-022-08113-6. Online ahead of print.

ABSTRACT

The goal of this review is to increase public knowledge of the etiopathogenesis of diabetic eye diseases (DEDs), such as diabetic retinopathy (DR) and ocular angiosarcoma (ASO), and the likelihood of blindness among elderly widows. A widow's life in North India, in general, is fraught with peril because of the economic and social isolation it brings, as well as the increased risk of death from heart disease, hypertension, diabetes, depression, and dementia. Neovascularization, neuroinflammation, and edema in the ocular tissue are hallmarks of the ASO, a rare form of malignant tumor. When diabetes, hypertension, and aging all contribute to increased oxidative stress, the DR can proceed to ASO. Microglia in the retina of the optic nerve head are responsible for causing inflammation, discomfort, and neurodegeneration. Those that come into contact with them will get blind as a result of this. Advanced glycation end products (AGE), vascular endothelial growth factor (VEGF), protein kinase C (PKC), poly-ADP-ribose polymerase (PARP), metalloproteinase9 (MMP9), nuclear factor kappaB (NFkB), program death ligand1 (PDL-1), factor VIII (FVIII), and von Willebrand factor (VWF) are potent agents for ocular neovascularisation (ONV), neuroinflammation and edema in the ocular tissue. AGE/VEGF, DAG/PKC, PARP/NFkB, RAS/VEGF, PDL-1/PD-1, VWF/FVIII/VEGF, and RAS/VEGF are all linked to the pathophysiology of DEDs. The interaction between ONV and ASO is mostly determined by the VWF/FVIII/VEGF and PDL-1/PD-1 axis. This study focused on retinoprotective medications that can pass the blood-retinal barrier and cure DEDs, as well as the factors that influence the etiology of neovascularization and neuroinflammation in the eye.

PMID:36513866 | DOI:10.1007/s11033-022-08113-6

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