Primary Angiosarcoma of Breast: A Case Report

Abstract

Primary breast sarcomas are rare entities. These malignant tumors originate from mesenchymal glandular breast tissue and account for <1 % of all breast cancer cases. Angiosarcomas are rare malignant tumors that arise from endothelial cells lining vascular channels. Most angiosarcomas are secondary to radiotherapy treatments for breast cancer or to an arm lymphoedema subsequent to a modified radical mastectomy. Primary angiosarcomas are rare and account for 0.04 % of all malignant breast tumors. Keywords Breast . Primary angiosarcoma Case reportA 22 year old female presented with 4 months history of awareness of painless progressive lump in left breast. There was no history of previous breast surgery or irradiation. On examination there was a large lump occupying all quadrants of left breast with skin nodules and plaques and pinkish blue discoloration (Fig. 1a). Examination of axilla was unremarkable. Trucut biopsy from the lump was suggestive of vascular channels dissecting the stroma. Vascular channels were lined by plump endothelial cells with extensive haemorrhage and focal mitotic activity. Morphology suggested possibility of angiosarcoma. Immunohistochemistry (IHC) was positive for CD 31 and CD 34. Based on these findings provisional diagnosis of angiosarcoma of left breast was kept. Chest radiograph, ultrasonography of abdomen and F-18 bone scan were negative for metastatic disease. Patient underwent total mastectomy with level I axillary lymph node dissection with latissimus dorsi myocutaneous flap reconsruction. On gross examination tumour was well circumscribed measuring 17× 14×7 cm. Histopathologic examination (HPE) showed large gaping vascular channels lined by endothelial cells, focally arranged as lobular aggregates with atypical mitosis (5-6 per 10 high power fields) and focally congested vascular channels (Fig. 1b). All margins were free of tumour. All 7 nodes resected were negative for malignancy. IHC was negative for cytokeratin and positive for CD 31 (Fig.1c) and CD 34. IHC was negative for estrogen and progesterone receptors. More than 75 % of bulk of tumour was formed by well differentiated pattern but there were significant foci showing solid areas with marked pleomorphism and mitotic activity. So, diagnosis of primary intermediate grade angiosarcoma of breast was made. Patient received 50 Gy radiation therapy to chest wall (2 Gy per fraction over 5 weeks) followed by three cycles of adriamycin and ifosfamide based chemotherapy. Following third cycle of chemotherapy she complained of bone pains. F-18 bone scan was done and it was suggestive of multiple bone metastasis while contrast enhanced computed tomogram of chest, abdomen and pelvis was negative for metastasis. DiscussionPrimary breast angiosarcoma is more frequent in young women (20 to 50 years) with no previous cancer history or other known risk factors [1,2]. Between 6 and 12 % of primary breast angiosarcomas are diagnosed during pregnancy or shortly after, suggesting hormonal involvement. However, cases reported to display ...