Pulmonary epithelioid angiosarcoma responsive to chemotherapy: A case report

Primary pulmonary epithelioid angiosarcoma (AS) is an extremely rare cancer with a poor prognosis. The presenting symptoms and imaging results are nonspecific and hold similarities with more common lung pathology, contributing to missed or delayed diagnosis. Complementing radiological imaging with patient information, such as presenting symptoms and exposures, is important for early consideration of pulmonary epithelioid AS. Even with supportive imaging findings and clinical suspicion for pulmonary epithelioid AS, the most reliable and definitive method for diagnosis is through immunohistochemistry. We describe the case of a 65-year-old patient who presented with dyspnea, cough, and hemoptysis in whom pauci-immune vasculitis was initially suspected before immunohistochemical diagnosis of primary pulmonary epithelioid AS. Due to the rarity of this disease, treatment options have not been well-studied and consist of any combination of surgical resection, chemotherapy, and radiation therapy. Although typically poorly responsive to chemotherapy, our patient achieved a reduction in size of his pulmonary nodules after a course of steroids followed by cyclophosphamide and was later maintained with gemcitabine and docetaxel until his death nearly a year after presentation.