Abstract
Introduction: The breast's angiosarcoma is a rare entity, representing 1% of the breast's malignant neoplasms and is classified as primary and secondary; the latter is associated with radiotherapy and chronic lymphedema. Clinically both present as a voluminous and rapidly growing lesion. Surgery is the cornerstone of a treatment since it provides the most outstanding local control, whereas radiotherapy and chemotherapy have little impact on the disease. Similarly, the oncological prognosis is poor because this type of tumor has a high recurrence rate and overall, 5-year survival of only 20%. Objective: Two cases of angiosarcoma of the breast (primary and secondary) are reported, both treated in the Breast Tumor Service and a bibliographic review of the disease is presented. Materials and Methods: All breast cancer cases were reviewed in the Breast Tumor Service for two years. Results: In the first clinical case, a 61-year-old patient was presented with a 5-month-old primary angiosarcoma in the right breast, treated with modified radical mastectomy. While in the second case, the clinical history of a 76-year-old woman with secondary breast angiosarcoma is related; the patient had a history of right breast cancer, treated ten years previously with conservative surgery, sentinel node and adjuvant radiotherapy. However, the woman presented local recurrence six years later, and she was operated on with a total mastectomy. Three years later, she developed an angiosarcoma in the surgical bed that warranted a wide resection of the chest wall. Conclusion: The breast's angiosarcoma is an aggressive and rare tumor of unknown aetiology, since it has a high recurrence rate and poor survival. Its main prognostic factors are tumor volume and resection margins. Therefore, early diagnosis is essential because it allows complete resection of the lesion.
Highlights
The breast's angiosarcoma is a rare entity, representing 1% of the breast's malignant neoplasms and is classified as primary and secondary; the latter is associated with radiotherapy and chronic lymphedema
In the first clinical case, a 61-year-old patient was presented with a 5-month-old primary angiosarcoma in the right breast, treated with modified radical mastectomy
While in the second case, the clinical history of a 76-year-old woman with secondary breast angiosarcoma is related; the patient had a history of right breast cancer, treated ten years previously with conservative surgery, sentinel node and adjuvant radiotherapy
Summary
Sarcomas of the breast include a rare heterogeneous group of neoplasms, accounting for less than 5% of soft tissue sarcomas and only 1% of malignant neoplasms in this region In this sense, angiosarcoma is an even less frequent malignant neoplasm originating from the blood vessels' endothelial cells and is characterized by a rapid proliferation of neoplastic cells that extensively infiltrate local tissues [1, 2]. Secondary angiosarcoma presents at a variable age; its peak incidence appears ten years after the end of radiotherapy treatments to the chest. It is usually associated with chronic lymphedema (Stewart-Treves syndrome). About the development of secondary angiosarcoma, the main associated factor is a chronic inflammation of the tissues: radiotherapy, radical mastectomy and axilla dissection [3, 8]. For primary angiosarcoma, it is even higher, from 0.002 to 0.05% [9]
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