Angiosarcoma Of Breast Research Articles

Solitary Subcutaneous Tissue Metastasis as Recurrence in a Case of Primary Angiosarcoma of Breast: Findings on 18F-Fluorodeoxyglucose Positron Emission Tomography-Computed Tomography.

Primary angiosarcomas of the breast are rare tumors, with a fatal outcome. We present a rare case of an operated primary angiosarcoma of the right breast in a 20-year-old female who showed disease recurrence in the right posterior arm subcutaneous tissue on 18F-fluorodeoxyglucose positron emission tomography-computed tomography after 1 year of surgery without any other visceral metastasis.

Breast Angiosarcoma Surveillance Study: UK national audit of management and outcomes of angiosarcoma of the breast and chest wall.

Breast angiosarcomas are rare tumours of vascular origin. Secondary angiosarcoma occurs following radiotherapy for breast cancer. Angiosarcomas have high recurrence and poor survival rates. This is concerning owing to the increasing use of adjuvant radiotherapy for the treatment of invasive breast cancer and ductal cancer in situ (DCIS), which could explain the rising incidence of angiosarcoma. Outcome data are limited and provide a poor evidence base for treatment. This paper presents a national, trainee-led, retrospective, multicentre study of a large angiosarcoma cohort. Data for patients with a diagnosis of breast/chest wall angiosarcoma between 2000 and 2015 were collected retrospectively from 15 centres. The cohort included 183 patients with 34 primary and 149 secondary angiosarcomas. Median latency from breast cancer to secondary angiosarcoma was 6 years. Only 78.9 per cent of patients were discussed at a sarcoma multidisciplinary team meeting. Rates of recurrence were high with 14 of 28 (50 per cent ) recurrences in patients with primary and 80 of 124 (64.5 per cent ) in those with secondary angiosarcoma at 5 years. Many patients had multiple recurrences: total of 94 recurrences in 162 patients (58.0 per cent). Median survival was 5 (range 0-16) years for patients with primary and 5 (0-15) years for those with secondary angiosarcoma. Development of secondary angiosarcoma had a negative impact on predicted breast cancer survival, with a median 10-year PREDICT prognostic rate of 69.6 per cent, compared with 54.0 per cent in the observed cohort. A detrimental impact of secondary angiosarcoma on breast cancer survival has been demonstrated. Although not statistically significant, almost all excess deaths were attributable to angiosarcoma. The increased use of adjuvant radiotherapy to treat low-risk breast cancer and DCIS is a cause for concern and warrants further study.

The significance of enhancer of zeste homolog 2 (EZH2) expression in spindle cell lesions of the breast.

Spindle cell lesions of the breast are rare entities and pose a diagnostic challenge for pathologists due to overlapping morphologic and immunohistochemical features. We evaluated EZH2 expression in various benign (fibromatosis (n = 8), myofibroblastoma (n = 7), neurofibroma (n = 1), nodular fasciitis (n = 5), benign phyllodes tumor (n = 18)) and malignant (malignant phyllodes tumor (n = 8), metaplastic breast carcinoma (n = 16) and angiosarcoma (n = 8)) spindle cell lesions as a potential diagnostic and therapeutic marker. The EZH2 expression was evaluated semi-quantitatively to categorize the cases as 'low' and 'high' expression. All benign lesions showed low EZH2 expression, whereas high EZH2 expression was observed in the majority (28/32; 88%) of malignant lesions. The study results suggest that EZH2 may be used both as an additional diagnostic tool to reach an accurate diagnosis of the spindle cell lesions of the breast and as a therapeutic target for the malignant lesions.

Primary breast angiosarcoma with disseminated intravascular coagulation is successfully treated with self‑subcutaneous unfractionated heparin calcium injection: A case report

Angiosarcoma is a rare sarcoma with a poor prognosis and is prone to disseminated intravascular coagulation (DIC), where DIC often interferes with chemotherapy. Primary angiosarcoma of the breast (PASB) is a type of angiosarcoma that is located in mammary parenchyma and is not associated with radiation exposure. The current study reported a 47-year-old female with DIC associated with PASB. The DIC of the patient relapsed during mono-chemotherapy with paclitaxel (PTX) after first-line anticoagulant therapy using thrombomodulin-α. The second-line danaparoid sodium therapy, followed by self-subcutaneous injection of unfractionated heparin calcium (UFH), resulted in long-term stabilization of DIC. Under this second-line anticoagulant therapy, the patient continued chemotherapy and chemoradiotherapy for >13 months in the outpatient setting without impairment of quality of life. The present case suggested that self-subcutaneous injections of UFH may be a useful therapeutic option for long-term control of DIC associated with PASB. However, further prospective clinical trails are needed to verify the efficacy of self-subcutaneous injection of UFH in similar settings.

ASO Author Reflections: Breast Angiosarcoma—A Rare Disease with a Lot of Uncertainty

ASO Author Reflections: Breast Angiosarcoma—A Rare Disease with a Lot of Uncertainty

Characteristics and Long-Term Risk of Breast Angiosarcoma.

Angiosarcoma of the breast is rare and aggressive. It can occur as a de novo tumor or secondary to breast cancer treatment. The purpose of this study is to analyze differences between patients with primary and secondary angiosarcoma of the breast and investigate potential risk factors for its development. The Surveillance, Epidemiology, and End Results program of the National Cancer Institute database was queried to identify patients with angiosarcoma of the breast, trunk, shoulder, and upper arm. The population-based incidence was analyzed. Primary and secondary angiosarcoma cases were identified and compared. Breast cancer characteristics of secondary angiosarcoma patients were compared with all breast cancer patients in the database who did not develop angiosarcoma. Overall, 904 patients were included, and 65.4% were secondary angiosarcomas. These patients had worse survival, were older, more likely to be White, more likely to have regionally advanced disease, and had angiosarcoma tumors of higher pathologic grade. Independent factors associated with development of secondary angiosarcoma among breast cancer patients included White race, older age, invasive tumor, lymph node removal, lumpectomy, radiation treatment, and left-sided tumor. Although the mean time to develop angiosarcoma after breast cancer diagnosis was 8.2 years, the risk continues to increase up to 30 years after breast cancer treatment. Angiosarcoma is rare but increasing in incidence. Secondary angiosarcomas are more common and exhibit more aggressive behavior. Several factors for angiosarcoma after breast cancer treatment could be identified, which may help us counsel and identify patients at risk.

Abstract PS13-05: A phase 2 study of oral paclitaxel and encequidar (oPac+E) in the treatment of cutnaeous angiosarcoma: The breast angiosarcoma subgroup

Abstract Background Cutaneous angiosarcomas are highly aggressive malignant tumors with poor prognosis. Currently there is no FDA-approved treatment. oPac+E is a combination of oral paclitaxel and a novel oral P-glycoprotein inhibitor, Encequidar. Materials and Methods: This is a phase-2 study evaluating the activity, safety and tolerability of oPac 205mg/m2 + E 12.9 mg once daily for 3 consecutive days weekly for patients with unresectable cutaneous angiosarcoma. Tumour response was evaluated every 6 weeks using RECIST and photography. Results: From Aug-2018 to May-2020, 7 of 26 enrolled patients (pts) had breast cutaneous angiosarcoma. All had previous breast cancer, mastectomy and radiotherapy and/or adjuvant chemotherapy, no prior taxane for angiosacoma and no metastatic disease. Median age was 66 yrs (range 49-76). Best objective response rates were: complete response (CR) 43% (3/7 pts), partial response (PR) 14% (1/7 pts), stable disease (SD) 43% (3/7 pts), progressive disease (PD) 0%. Furthermore, 3 pts (43%) who had inoperable lesions were deemed operable after Oraxol treatment and received curative intent surgical resection. Median progression free survival (PFS) was 17 weeks. oPac+E was generally well tolerated. Grade-3 treatment related adverse events (AEs) occurred in 5 pts: diarrhea=1, fatigue=2, neutropenia =1, dyspnea=1, dehydration=1, pneumonitis=1. Grade-4 AE were neutropenia in 2 pts. Grade-2 paraesthesia=1. All pts fully recovered. Dose reductions were needed in 3 subjects. No patients discontinued treatment due to AEs. To date no patients died. Conclusions: oPac+E may provide an effective oral treatment for radiation-associated breast angiosarcoma, with a high clinical benefit rate (CR + PR+ SD=100%), durable response and enabled resection of unresectable tumors. It is generally well tolerated even in elderly patients; with low incidence of neuropathy. It may allow patients to avoid hospital IV chemotherapy visits and the option of home treatment. Citation Format: Vinod Ravi, Michael Wagner, Tom Wei-Wu Chen, Herbert H Loong, Robert Mennel, Cheuh-Chuan Yen, Glen Clack, David Cutler, Rudolf Kwan, Wing=Kai CHan. A phase 2 study of oral paclitaxel and encequidar (oPac+E) in the treatment of cutnaeous angiosarcoma: The breast angiosarcoma subgroup [abstract]. In: Proceedings of the 2020 San Antonio Breast Cancer Virtual Symposium; 2020 Dec 8-11; San Antonio, TX. Philadelphia (PA): AACR; Cancer Res 2021;81(4 Suppl):Abstract nr PS13-05.

MYC and NOTCH1-positive postradiation cutaneous angiosarcoma of the breast.

Postradiation cutaneous angiosarcoma of the breast is a rare, delayed complication of adjuvant radiation treatment for breast carcinoma and is associated with a worse prognosis than the original primary cancer. Recent studies have characterized the diagnostic utility of MYC and NOTCH1 receptor expression as markers for secondary radiation-associated angiosarcomas. Herein, we report an exophytic secondary breast angiosarcoma with MYC and NOTCH1 immunoreactivity. This case illustrates the utility of these markers for the identification of radiation-associated angiosarcoma with MYC and NOTCH1 expression, potential for targeted therapy and need to identify patients for further studies of the clinicopathologic and prognostic significance.

Primary breast angiosarcoma – a case report

Introduction. Angiosarcoma is a rare breast cancer that can be primary or secondary after surgery or after breast cancer radiotherapy. It is important that breast angiosarcoma belongs to tumors with a non-specific clinical and radiological picture. Aim. The study of the biopsies contained aggressive vasomotor hyperplasia. Description of the case. The presented case concerns the primary angiosarcoma of the right breast in a 56-year-old woman who had never had a surgical procedure before, nor radiotherapy in the area of the breast. Conclusion. Histopathological examination supported by immunohistochemistry is a reliable and indispensable diagnostic element in the diagnosis of vascular sarcoma.

Primary breast angiosarcoma in postmenopausal women with a picture like Kasabach-Merritt syndrome: A case report

Introduction. Primary breast angiosarcoma is a very rare tumor and accounts for 0.04% of all breast malignant tumors and most commonly occur in young women. Kasabach-Merritt syndrome (KMS) is described as consumption coagulopathy with thrombocytopenia, and without adequate therapy almost certainly leads to a very fast lethal outcome. Case report. We present a rare case of 60-year-old postmenopausal woman with metastatic primary angiosarcoma of the breast associated with a picture like Kasabach-Merritt's syndrome (thrombocytopenia and anemia without the coagulation factor disorder with massive bleeding in the tumor). Conclusion. Primary breast angiosarcoma in postmenopausal women is a very rare tumor, and may be associated with anemia and thrombocytopenia without other laboratory parameters for Kasabach-Merritt's syndrome. Anemia and thrombocytopenia are refractory to standard treatment protocols, and also significantly reduces the quality of life of these patients. The literature contains only a few cases of Passociated with thrombocytopenia or with KMS and there are no clear defined protocols for the treatment of these patients, which requires the presentation of as many cases as possible.

Radio-induced breast angiosarcoma: features of diagnostics and treatment (a clinical case and literature review)

Breast angiosarcoma (BAS) is an extremely rare and poorly studied neoplasm, the etiology of primary BAS remains controversial, and secondary BAS is most often radio-induced. Radio-induced breast tumors usually appear 10–20 years after the initial treatment; however, for BAS this period is much shorter and is about 4–7 years. This review presents literature data on the features of the clinic, diagnosis and treatment of BAS, as well as own clinical case observation of radio-induced sarcoma of the left breast, that developed 4 years after the primary breast cancer treatment.

Primary breast angiosarcoma: A clinicopathologic analysis of cases from the last 16 years

This is all about the mammary angiosarcoma as a clinicopathologic analysis of cases from the last 16 years (Primary breast angiosarcoma).

A Case Report of Primary Breast Angiosarcoma: Clinical Presentation and Outcome After Adjuvant Radiotherapy

Angiosarcomas of the breast are infrequent subtypes of sarcoma that are often diagnosed after radiation therapy for primary breast cancer. Primary angiosarcomas (PAS) are rare tumors that constitute 0.04% of all malignant breast tumors. We report a case of a 40-year-old woman with a lump in the right breast and diagnosed as angiosarcoma by pathological evaluation. She underwent simple mastectomy followed by adjuvant radiation. She is alive and disease-free for 66 months although tumor size was large and one surgical margin was tumor positive. Breast angiosarcoma is often in advanced stage at diagnosis and tends to recur locally. Although surgical methods constitute the primary treatment, we believe that a multidisciplinary treatment strategy should be used in high-risk patients with large primary tumors and tumor positive margins.

Radiation-Induced Breast Angiosarcoma After Breast Cancer: A Case Report

Radiation-Induced Breast Angiosarcoma After Breast Cancer: A Case Report

Male Breast Cancer in Togo: Imaging and Clinicopathological Findings.

Background Breast cancer in men is a rare condition, often diagnosed late. The purpose of this study was to describe its epidemiological, histopathological, and radiographic aspects in Togo. Materials and Methods This was a descriptive retrospective study on cases of breast cancer in humans diagnosed histologically at the Laboratory of Anatomy Pathological and Imagery of the University Hospital in Lomé, over a period of 25 years (1995 to 2019). The parameters studied were epidemiological, anatomopathological, and imaging. Results Eighty-two (82) cases were diagnosed, an annual frequency of 3.28 cases. The mean age was 45 ± 2.5 years; the range was 27-63 years. The family history of 47 patients (57.32%) was known. Carcinomas represented the predominant histological group with predominantly nonspecific invasive carcinoma (87.5%). These cancers were diagnosed at late stages (75.71% grade II). They were mainly of luminal B profile (38.75%) and associated with mutations of the BRCA2 and BRCA1 genes in 14.63% of the cases. The lesions were classified ACR 5 in 61.5% (11/18). Two cases of breast angiosarcoma were diagnosed by the identification of CD31 markers and factor VIII in immunohistochemistry. Hormone therapy such as tamoxifen was prescribed in all luminal patients (43 patients). Radiotherapy was administered to 15 patients (18.3%), with acute toxicity in 20% of the cases. After a median follow-up of 36 months, the evolution was complete remission in 27 patients (32.93%). Conclusion Breast cancer in men is rare, often diagnosed late with a poor prognosis.

Primary breast angiosarcoma in a postmenopausal female.

Primary breast angiosarcoma in a postmenopausal female.

Novel Body Composition Predictors of Outcome in Patients With Angiosarcoma of the Breast: A Preliminary Study.

The aim of the study was to determine abdominal and breast adipose tissue parameters on 18-fluorodeoxyglucose positron emission tomography/computed tomography (CT) that may serve as outcome predictors in breast angiosarcoma patients. Women with breast angiosarcoma (n = 13) who underwent 18-fluorodeoxyglucose positron emission tomography/CT were identified. A control group was selected (n = 25). Abdominal subcutaneous (SAT) and visceral adipose tissue (VAT) were assessed on unenhanced computed tomographies. Breast adipose tissue (BAT) volumes of the uninvolved breast were quantified. Metabolic activity of VAT, SAT, and BAT was calculated (standardized uptake value [SUV]). Breast angiosarcoma patients had higher metabolic activity of VAT compared with controls (SUV 0.93 ± 0.39 vs 0.64 ± 0.11, P = 0.044). Within the patient group, there were 6 deaths (46.2%). Patients who died had higher SAT activity (SUV 0.52 ± 0.24 vs 0.29 ± 0.06, P = 0.027) and higher BAT metabolic activity (SUV 0.48 ± 0.20 vs 0.27 ± 0.11, P = 0.045) compared with nondeceased patients. Patients with breast angiosarcoma have higher metabolic activity of VAT. Higher abdominal SAT and higher BAT metabolic activity of the uninvolved breast might predict mortality.

Bilateral primary angiosarcoma of breast in young patient: A case report with review of literature

Angiosarcomas are rare tumors arising from endothelial lining of vascular channels. The first case of breast angiosarcoma was presented in 1907 by Borrman and the first case of secondary angiosarcoma described in 1987 by Body. There are 2 main types. Haemangiosarcomas in blood vessels and lymphangiosarcomas in lymphatic vessel walls.All angiosarcomas tend to be aggressive and are mostly multicentric. These tumors have very high local recurrence rate and metastasis because of their intrinsic biologic properties. They are often misdiagnosed leading to a poor prognosis and a high mortality rate. We present a case of a 21-year-old female presented with an ulcerated right breast lesion which was diagnosed as primary angiosarcoma of breast with metastasis to left breast after 4 years.

Two cases of breast angiosarcoma after breast conserving surgery

BackgroundBreast angiosarcoma (AS) is a rare malignant breast tumor arising from endothelial cells lining the blood vessels. The prognosis of AS is reportedly poor. However, the effectiveness of chemotherapy and radiation is still controversial. Surgery is the only curable treatment, and removal of AS with adequate surgical margin is important.Case presentationWe report two cases of primary and radiation-induced breast angiosarcoma (AS) after performing breast conserving surgery (BCS) for breast cancer. In case 1, a 72-year-old woman underwent right BCS with adjuvant radiation therapy (RT) for breast cancer 5 years prior. She was diagnosed with AS of the right breast and underwent mastectomy with a wide skin resection of the breast. As the tumor cells were positive for c-myc, this tumor was diagnosed as a radiation-induced AS. In case 2, an 80-year-old woman underwent BCS without adjuvant RT. She was diagnosed with AS 3 years after BCS and underwent mastectomy with a wide skin resection of the breast. The tumor was diagnosed to be a primary AS because there were no episodes of RT or lymphedema. Both cases developed local recurrence within 1 year of surgery.ConclusionOur cases suggest that surgical margin is associated with the risk of local recurrence, and the difficulty of deciding a safe surgical margin should be set during preoperative diagnosis.

Reaching for the Brass Ring

Reaching for the Brass Ring