Supratentorial hemangioblastoma with unusual features.

Abstract

Hemangioblastomas of the central nervous system (CNS) are benign tumors that most commonly occur in the cerebellum, followed by the brainstem and spinal cord [1]. Supratentorial location is extremely rare [2]. CNS hemangioblastomas can occur sporadically or in association with von Hippel-Lindau disease [2]. Histologically, the tumors are composed of two main components: large and vacuolated stromal cells and numerous thinwalled vessels. Due to the characteristic lipid-containing cytoplasm and foci of pleomorphic nuclei, the differential diagnosis of hemangioblastoma from some other malignant tumors, such as clear cell renal cell carcinoma (RCC), adrenal cortical carcinoma and paraganglioma, remains challenging, especially in cellular variants. Hyaline globules can be found in many different tumors and benign tissues [3], including CNS hemangioblastomas, and they contribute to the diagnostic difficulty. Herein, we report a case of sporadic supratentorial hemangioblastoma with unusual features and discuss the differential diagnosis of CNS hemangioblastoma.