Primary Pulmonary Angiosarcoma: A Case Report and Literature Review

Primary sarcomas of the thorax are rare. They are classified according to their histologic features and constitute a large group of tumors that occur in the lung, mediastinum, pleura, and chest wall. Although primary thoracic sarcomas commonly manifest as large, heterogeneous masses, they have a wide spectrum of radiologic manifestations, including solitary pulmonary nodules, central endobronchial tumors, and intraluminal masses within the pulmonary arteries. Angiosarcomas are rare, malignant vascular tumors, representing about 2% of all soft tissue sarcomas. The most frequently primarily affected sites include the heart, liver, breast, skin, and scalp, and they have a high rate of metastases to the lungs and, less commonly, liver, regional lymph nodes, and bone. Definitive diagnosis is made based on histopathological and immunohistochemical findings. No standard treatment regimen has been established for pulmonary angiosarcoma. Radiation therapy, chemotherapy, surgical resection, and immunotherapy have been attempted. The use of radiation therapy in conjunction with surgery has resulted in local control and excellent functional and cosmetic outcome, for patients with angiosarcoma of the head and neck, but not the lung. For advanced-stage disease, other than the combination of doxorubicin and ifosfamide-based regimens, there have been few other chemotherapeutic options for the treatment of local or metastatic angiosarcoma.