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

backgroundEpithelioid 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. OBSERVATIONS: A 20-year-old man presented with multiple pulmonary, hepatic, and intracerebral lesions. The patient was diagnosed with EHE via biopsy of pulmonary lesions. During follow-up without treatment, progressive perilesional edema was observed in the left temporal lobe, leading to resection. Histological findings were consistent with EHE, and immunohistochemical analysis revealed overexpression of TFE3 in the tumor cells. Hence, a diagnosis of TFE3-rearranged iEHE was established. Immediately after detecting progressive lesions, the patient received multiple interventions including surgery and radiation therapy. Radiological findings revealed multiple cystic lesions with hemorrhagic components. The tumor has been successfully controlled for 13 years with repeated surgeries, radiation therapy, and active surveillance. LESSONS: TFE3-rearranged iEHE is an extremely rare type of tumor and can present with multiple cystic lesions containing hemorrhagic components on imaging. TFE3-rearranged iEHE can be successfully managed with repeated intervention and active surveillance. https://thejns.org/doi/10.3171/CASE25416.