Successful management of recurrent multiple intracerebral TFE3-rearranged epithelioid hemangioendothelioma with repeated intervention and active surveillance: illustrative case.

Abstract

Epithelioid hemangioendothelioma (EHE) is a rare vascular tumor with intermediate malignant potential. TFE3-rearranged intracerebral EHEs (iEHEs) have not been reported to date. Herein, the authors describe a rare case of recurrent multiple TFE3-rearranged iEHEs involving the lung and liver, which exhibited a favorable clinical course after repeated surgeries, radiation therapy, and active surveillance. A 20-year-old man presented with multiple pulmonary, hepatic, and intracerebral lesions. The