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Congresso Internacional de Neurocirurgia-Congreso Internacional de Neurocirugía. Neuroiberia 2016
Portugal, 11-14 mayo 2016
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24. VASCULAR
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P254 - A supratentorial dural-based hemangioblastoma in a patient without von Hippel Lindau Syndrome

E. Oliveira1, J.P. Lavrador1, J. Pimentel2 and N. Simas1

1Serviço de Neurocirurgia; 2Laboratório de Neuropatologia, Centro Hospitalar Lisboa Norte.

Introduction: Hemangioblastomas are benign tumours of vascular origin. They are commonly associated with von Hippel-Lindau (VHL) Syndrome, an autosomal dominant genetic transmission on chromosome 3. Most common locations of hemangioblastomas in patients with VHL are in the cerebellum and spinal cord. They are rarely found in a supratentorial location. A dural-based characteristic is exceedingly rare with only 8 known published cases in patients without VHL Syndrome.

Case report: The authors present a 75 year-old male with a history of gait instability and an episodic fall after a loss of strength in the right lower limb. A CT scan showed a left frontal hypodense mass with significant vasogenic edema. Afterwards the MRI confirmed a 25 mm left frontal dural-based lesion with intensive contrast enhancement with vascular voids and a cystic component. After surgical removal, the histology revealed a dural-based WHO Grade 1 supratentorial hemangioblastoma. Family history was negative for VHL Syndrome. Neuro-Axis MRI was negative for other lesions.

Discussion: This entity is a very rare with only 8 cases published in the literature. We report the ninth case of an isolated supratentorial dural-based hemangioblastoma not associated with von Hippel-Lindau syndrome.

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