Primary Epithelioid Hemangioendothelioma in the Cerebellum: Case Report with Reference to Drastic Change in the WHO Classification

Epithelioid hemangioendothelioma is a rare vascular brain tumor. It develops from endothelial cells, usually in the liver, lung, bone and soft tissue. Primary localization of this tumor in the intracranial space is very uncommon; only 47 cases have been described in the literature. Th is tumor was initially classifi ed as grade I (benign) in the World Health Organization (WHO) 2007 classifi cation. In 2016, this tumor was re-classifi ed as grade III (malignant). Herein, the fi rst case report of epithelioid hemangioendothelioma in the cerebellum of a male patient is presented. Complete surgical excision was done. No adjuvant therapy was administered. Magnetic resonance imaging performed 2 years after the surgery continued to show no recurrence of the tumor. To our knowledge, this is the fi rst report of cerebellar location of this rare tumor. In addition, the authors report drastic re-classifi cation of the epithelioid hemangioendothelioma from the benign tumor (WHO 2007) to a malignant one (2016), which signifi cantly changes postoperative management and follow up of this brain neoplasm.