Breast Angiossarcoma in a Male Patient: A Case Report

Abstract

Primary sarcomas of the breast originate from connective tissue and are responsible for less than 1% of all breast malignancies with an incidence of 5 cases per million in the United States. Primary breast angiosarcoma originates in the parenchyma and can secondarily compromise the skin and pectoral muscles in advanced cases. Sarcoma is present more in women between the ages of 14 and 82, mainly in the third and fourth decades of life. At diagnosis, as in other sarcomas, the size is bigger than 5 cm, with a direct correlation with prognosis; because of few data in literature due to its incidence and frequent error and the inespecific clinical and radiological signs, we report a case of breast angiosarcoma in a male patient from the Hospital Santo Antônio/Obras Sociais Irmã Dulce, Salvador, BA. It is the case of a 42-year-old man with a nodule in the upper medial quadrant of the right breast, measuring 2 cm. The mammogram and ultrasound showed a 1.4-cm regular nodule in the upper medial quadrant, BI-RADS 4. The patient underwent a core biopsy with a pathology reporting a chronic inflammatory process and a nonmalignant neoplasia; immunohistochemical positive for CD 68 and LCA and negative for cytokeratin 34beta12, P63, and cytokeratin AE1/AE3. Then, the nodule was excised and the pathology result showed a fusiform cell neoplasia with a positive posterior margin confirmed by immunohistochemical that neoplastic cells were positive for CD34 and CD31, negative for cytokeratin AE1/AE3, and inconclusive to smooth muscle actin with KI-67 <10%, leading to the diagnosis of angiosarcoma. After that, the margins re-excision the pathological staging (American Joint Committee on Cancer) ypT0. No evidence was found for metastases in other sites. The patient is now waiting for radiotherapy for local control benefits. There were 16 fractions in the right breast and a multidisciplinary follow-up. The discussion showed a rare case in the literature in agreement with the 170 cases reported, with a great impact when seen in men since the case reported prevalence in women. In relation to diagnosis, it becomes a challenge, especially in low-grade malignant tumors with multiple tissue pieces and needed the best pathology analysis, which could delay treatment. The inespecific alterations in imaging examinations as well as at tests, such as the presence of fatty tissue in a mammogram, would include hemangiomas and angiolipomas as differential diagnosis contributing to delay in the diagnosis. As treating large tumor resection due to aggressive behavior is recommended, it is a therapeutic option if associated with radiotherapy reducing risk by 20–50%. That was the treatment adopted for the patient described above. This study, besides contributing to the literature on angiosarcoma incidence, also affects the possible presentation in male patients, elevating the diagnostic hypothesis of nodule in the cases of early adequate treatment.