Rosenbaum E, Jadeja B, Xu B, et al. - Researchers examined the clinicopathologic and molecular findings of a large cohort of 93 translocation-positive epithelioid hemangioendothelioma (a low-grade malignant vascular tumor with an intermediate clinical behavior among benign hemangiomas and high-grade angiosarcomas). To recognize secondary genetic alteration exhibiting that more than half of tumors had a genetic alteration beyond the disease-defining gene fusion, Memorial Sloan Kettering-IMPACT targeted DNA sequencing was done in 18 patients (15 with WWTR1-CAMTA1 and 3 with YAP1-TFE3). A less favorable outcome in comparison with the YAP1-TFE3 subset was noted in patients with conventional epithelioid hemangioendothelioma with WWTR1-CAMTA1 fusion. Soft tissue epithelioid hemangioendothelioma was often solitary, followed an uneventful clinical course being frequently managed with curative surgery. A significantly worse outcome was noted in multifocality, pleural involvement, lymph node or distant metastases. Moreover, with 22% and 30%, respectively, alive at 5 years, in contrast with > 70% survival rate in patients lacking these two adverse factors, patients with a pleural disease or lymph node metastases had an aggressive clinical course similar to high-grade sarcomas.