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Ref Type Journal Article
PMID (41836264)
Authors Gong J, Zheng L, Tian F, Xiao P, Yu D, Jiang L, Bao P
Title A rare pulmonary epithelioid angiosarcoma with ALK rearrangement: a case report and literature review.
Journal Vol Issue Date Pages Journal Short Title
Frontiers in oncology 16 2026 1780353 Front Oncol
URL
Abstract Text 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.

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Molecular Profile Treatment Approach
Gene Name Source Synonyms Protein Domains Gene Description Gene Role
Therapy Name Drugs Efficacy Evidence Clinical Trials
Drug Name Trade Name Synonyms Drug Classes Drug Description
Gene Variant Impact Protein Effect Variant Description Associated with drug Resistance
Molecular Profile Indication/Tumor Type Response Type Therapy Name Approval Status Evidence Type Efficacy Evidence References
EML4 - ALK angiosarcoma predicted - sensitive Ensartinib Case Reports/Case Series Actionable In a clinical case study, Ensacove (ensartinib) treatment resulted in a partial response in a patient with epithelioid angiosarcoma harboring EML4-ALK (PMID: 41836264). 41836264