Supratentorial Hemangioblastoma with Unusual Features
신유주, Kim Seok-Hwi, 이현우, 방희진, 서연림,
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신유주 ( Shin Yoo-Ju )
Sungkyunkwan University School of Medicine Samsung Medical Center Department of Pathology and Translational Genomics
( Kim Seok-Hwi )
Sungkyunkwan University School of Medicine Samsung Medical Center Department of Pathology and Translational Genomics
이현우 ( Lee Hyun-Woo )
Sungkyunkwan University School of Medicine Samsung Medical Center Department of Pathology and Translational Genomics
방희진 ( Bang Hee-Jin )
Sungkyunkwan University School of Medicine Samsung Medical Center Department of Pathology and Translational Genomics
서연림 ( Suh Yeon-Lim )
Sungkyunkwan University School of Medicine Samsung Medical Center Department of Pathology and Translational Genomics
KMID : 0357920140480060462
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 emangioblastomas can occur sporadicallyor 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 hemangioblastomawith unusual features and discuss the differential diagnosis of CNS hemangioblastoma.
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