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Abstract

Primary angiosarcoma of the breast is a rare form of breast cancer, accounting for only 0.04% of all malignant breast tumours. We report a 35-year-old woman who presented with a recurrent lump in the left breast for four months. Fine needle aspiration cytology (FNAC) was suspicious of angiosarcoma and this diagnosis was confirmed on core needle biopsy. Modified radical mastectomy was performed as sole treatment. No chemo radiotherapy was given as tumor was grade I, margins were negative and there was no lymph node involvement. She is on regular follow up and no recurrence reported since the surgery. The 5-year disease-free survival for grade I tumours can be as high as 76%, and up to 70% for grade II tumours whereas for grade III tumours it is reportedly about 15%. Primary angiosarcoma of the breast is a rare malignancy with a poor prognosis, even after complete resection. Surgery is the mainstay of treatment with a limited role for chemotherapy and radiotherapy. INTRODUCTION Angiosarcomas are rare tumors of vascular origin. Sarcomas are extremely raretumours, accounting for <1% of all malignant breast tumours. Incidence of Primary angiosarcoma of the breast has been found to be 0.05%. The first case of Primary angiosarcoma of the breast reported by Schmidt in 1887 and since then about 219 cases have been reported in literature.1,2 They have a high mortality rate with only 10-20% of the patients surviving beyond 5 years. Secondary angiosarcomas of the breast are more common and occur after breast irradiation. Here we discuss a patient with primary angiosarcoma of the left breast.CASE REPORT35 year old lady, P1L1, with no known medical co-morbidities, came with are  current left breast lump of scan showed a tumor of the left breast with vascular enhancement. The fat planes between the tumor and chest walls were maintained. Core needle biopsy showed a low to intermediate grade vascular neoplasm. Fine needle aspiration cytology revealed features of a spindle cell neoplasm Aleft modified radical mastectomy was performed. Histopathological evaluation of the lesion showed a tumour comprising of anastomosing slit like vascular spaces with papillary projections at places infiltrating into the underlying breast parenchyma and fibroadipose tissue. These spaces were lined by cells exhibiting mild pleomorphism, oval nuclei and scant cytoplasm The histologic features of angiosarcoma of the breast are classified into grades I, II and III. Angiosarcomas have a wide range of histological appearances from well-differentiated grade I tumours consisting infiltrating bland vascular channels to poorly differentiated grade III tumours with a sarcomatous spindle cell pattern. Large amounts of blood are often present. Papillary clusters of tumours cells, which can be mistaken for ductal carcinoma in situ, may also occur. Factor VIII and CD31 immunostaining confirmthe diagnosis.9Ultrasonography generally shows a solid mass that may have well-defined or lobulated margins, with both hypoechoic and hyperechoic appearance. There is oftenno acoustic shadowing. A contrast-enhanced MRI of the breast generally shows an enhancing vascular mass.10Paclitaxel has been shown to produce excellent response in a number of studies in patients with primary angiosarcoma of breast. New agents against angiogenesis, such as bevacizumab or rapamycin, might also be useful against this tumour. Immunohistochemical staining for endoglin, known to be expressed mainly on the surface of endothelial cells, suggests the possibility of treating angiosarcoma with anti-endoglin monoclonal antibodies.11Radiation treatment has been used in the adjuvant setting of breast sarcoma with the intent of improving both locoregional controlsafter surgical excision and survival. Even after mastectomy, radiotherapy has been thought to be beneficial for patient with microscopically positive margins.12The prognosis, however, remains poor despite early diagnosis and treatment.The overall 5 year survival ranges from 8-50%. The 5 years disease free survival rate for low grade tumourscan be as high as 76% and up to 70% for intermediate grade tumours. Whereas 5 years survival rate for high grade tumours is about 15%,