Supratentorial Hemangioblastomas: Three Case Reports and Review of the Literature
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Correspondence
Supratentorial Hemangioblastomas: Three Case Reports and Review of the Literature D. J. She · Z. Xing · Y. Liu · D. R. Cao
Received: 17 August 2012 / Accepted: 25 October 2012 / Published online: 4 December 2012 © Springer-Verlag Berlin Heidelberg 2012
Abstract Hemangioblastoma (HBL) within the central nervous system is a benign vascular neoplasm that usually occurs in the cerebellum. Supratentorial occurrence of HBL is an extremely rare event. Till date, approximately 129 cases of supratentorial HBL have been reported in the literature. Here, we present three new cases of supratentorial hemangioblatomas, one of which was found to have the lesions in a unique location of the choroidal fissure. The clinical, histopathological, and neuroradiological characteristics, as well as management of this rare disease are discussed with a review of the pertinent literature. Keywords Supratentorial hemangioblastoma · MRI · Histopathological examination · Immunohistochemical staining · Diagnostic imaging differences Abbreviations HBL Hemangioblastoma VHL von Hippel–Lindau disease MRI Magnetic resonance imaging CT Computed tomography MRA Magnetic resonance angiography CTA Computed tomography angiography DSA Digital subtraction angiography
D. R. Cao, MD () · D. J. She, MD · Z. Xing, MD · Y. Liu, MD Department of Radiology, First Affiliated Hospital, Fujian Medical University, 20 Cha-Zhong Road Fujian, 350005 Fuzhou, P.R. China e-mail: [email protected]
Introduction Hemangioblastomas (HBLs) of the CNS are infrequent, benign, and vascularized neoplasms of unknown origin that comprise approximately 1.5–2.5 % of all primary intracranial tumors and 7–10 % of primary posterior fossa tumors [1]. They can occur either sporadically (67 % of cases) or as a component of von Hippel–Lindau disease (VHL; 33 % of cases) [2]. HBLs are found predominantly in the posterior fossa or spinal cord. Supratentorial location is exceedingly rare, with only 129 cases reported from 1902 to 2011 [3]. To our best knowledge, choroidal fissure HBL has not been previously reported in the literature. Here, we report three cases of supratentorial HBLs not associated with VHL and make a short review of the literature related to the clinical presentation, histopathological findings, imaging features, and therapeutic strategies of the rare disease. Case Reports Case 1 A 60-year-old woman was admitted to our hospital with a 1-year history of headache and 15 days of blurred vision. Her physical examination was normal. Neurological examination on admission was unremarkable. Her medical and family history was uninformative about cancer or inherited diseases. Magnetic resonance imaging (MRI) revealed a cystic mass adherent to cerebral falx, with a 0.6 cm × 0.4 cm slightly hyperintense mural nodule at its inner side (Fig. 1a). The solid nodule was significantly enhanced, while the cystic compartment was not on the axial gadolinium-enhanced T1-weighted image (Fig. 1b). During surgery, a small cyst with xanthochromic fluid was found and the cystic
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