We present a rare case of irradiation induced angiosarcoma of breast metastasing to pleura .
A 67 year old white female was diagnosed with stage I infiltrating ductal carcinoma of the right breast in 1997 and treated with lumpectomy, lymph node dissection and radiation. She completed 5 years of Tamoxifen. In Sept. 2004 she presented with right breast pain and violaceous nodules. She underwent a biopsy that showed angiosarcoma. Epithelia tumor markers were negative. CD34 was negative, however CD31, factor VIII and vimentin were positive suggesting an endothelial origin. She was treated with simple mastectomy and chemotherapy.Two months later she presented with shortness of breath and clinical exam showed recurrence of angiosarcoma as violaceous nodules on the muscle flap and chest wall. She had developed a large right pleural effusion. CT scan of chest showed right sided effusion, bilateral lung nodules, mediastinal lymphadenopathy and axillary lyphadenopathy along with nodules on the flap. After fluid reaccumulation despite two thoracentesis, she underwent medical thoracoscopy for pleurodesis. Biopsies were taken from the nodular parietal pleura revealing angiosarcoma. She was treated with palliative chemotherapy but died one month later.
Pleural angiosarcomas are malignant vascular tumours. Because of epitheliod appearance these are also called epitheloid angiosarcomas. Confirnation of endothelial origin is by immunohistochemical staining with Factor VIII, CD34, CD31 related antigens. Epithelial markers like keratin are used to exclude epithelial origin. Primary and metastatic pleural involvement by lung angiosarcoma as been well documented, but post-irradiation angiosarcoma of the breast with pleural metastases has not been reported.Angiosarcoma of breast can occur spontaneously (primary) or after local radiation and in association with lymphedema (secondary). The first reported case of post irradiation breast angiosarcoma was described in 1987. Total of 100 cases of post-irradiation breast AS have been reported in English literature, but none reported with metastases to pleura. One case has reported recurrence with pleural lesions. Pleural metastases are rare at initial presentation (1%).Criteria for radiation induced AS were described in 1948 by Cahan et al 1) tumour arising in an irradiated area; 2) tumor is histologically different from first tumor; 3)A long period of latency between irradiation ad occurance of AS.Treatment is with primarily surgical resection, radiation and chemotherapy with little success and has poor prognosis high rate of recurrence. Most patients die within 3-5 years .Average interval between irradiation and dignosis of angiosarcoma is 7 years with range 3 - 20 years.These are highly aggressive tumours and has high rate of recurrence with in months of radical resection .
Breast AS has a poor prognosis. As more women are treated with breast conservation therapy for early breast cancer the incidence is expected to rise. Early clinical detection will require high index of suspicion by health care providers.
Nikhat Salamat, None.