Key result
A 65-year-old man with primary pulmonary epithelioid angiosarcoma achieved a reduction in pulmonary nodule size with steroids and cyclophosphamide, surviving nearly a year on maintenance therapy.
Case Report (n=1)
This case report demonstrates that primary pulmonary epithelioid angiosarcoma, though typically poorly responsive to chemotherapy, may show a partial response to a regimen including steroids, cyclophosphamide, gemcitabine, and docetaxel.
May support steroid-cyclophosphamide trial in this rare tumor; hypothesis-generating and leaves open need for prospective data.
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.
No takes yet. Share an insight, caveat, or question.
Shirey et al. (2018) conducted a case report in Primary pulmonary epithelioid angiosarcoma (n=1). Steroids followed by cyclophosphamide, maintained with gemcitabine and docetaxel was evaluated on Reduction in size of pulmonary nodules and survival. A 65-year-old man with primary pulmonary epithelioid angiosarcoma achieved a reduction in pulmonary nodule size with steroids and cyclophosphamide, surviving nearly a year on maintenance therapy.
Synapse has enriched 3 closely related papers on similar clinical questions. Consider them for comparative context: