A rare pulmonary epithelioid angiosarcoma with ALK rearrangement: a case report and literature review

Epithelioid angiosarcoma is a rare type of malignant tumor that progresses rapidly and currently lacks standard and effective treatment methods. We present herein the first reported case of rare pulmonary epithelioid angiosarcoma harboring an EML4-ALK fusion, in which targeted therapy demonstrated efficacy. This advanced, unresectable epithelioid angiosarcoma continued to progress despite prior treatments, including chemotherapy, anti-angiogenic therapy, immunotherapy, and radioactive particle implantation. Given the absence of standardized treatment protocols for this malignancy, we performed next-generation sequencing (NGS) to identify potential therapeutic targets, which revealed an ALK fusion. Subsequent ALK -targeted therapy proved effective, providing novel therapeutic insights for patients with advanced, unresectable disease. Further studies are warranted to elucidate the mutational landscape of pulmonary epithelioid angiosarcoma and its implications for disease pathogenesis, progression, treatment response, and prognosis.