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cerebellar_hemangioblastoma_in_von_hippel-lindau_disease

Cerebellar hemangioblastoma in von Hippel-Lindau disease

Symptoms and signs caused by cerebellar hemangioblastomas in von Hippel-Lindau disease are associated with edema and peritumoral cyst formation/propagation and are treated safely and effectively with resection. Cerebrospinal fluid diversion is rarely necessary after complete tumor removal in patients with preoperative hydrocephalus. Cerebellar hemangioblastomas are preferentially distributed in the posterior half of the cerebellum, as they are in the brainstem and spinal cord. Tumor recurrence is avoided by meticulous extracapsular resection 1).

Outcome

Solitary hemangioblastomas are for the most part considered benign, curable by total resection, except in those cases associated with von Hippel Lindau disease.

Case reports

a 37-year-old male patient who was diagnosed vHLD with multiple spinal and one infratentorial hemangioblastomas and holocord syrinx formation of the whole spinal cord. Combined microsurgical approaches to two spinal lesions and the cerebellar lesion followed by external beam radiotherapy of the posterior fossa and the whole spinal axis stabilized tumor growth of the asymptomatic lesions, while no recurrent tumors were detected at the site of surgery. A clinical deterioration connected to early postoperative deficits stabilized to a moderate gait ataxia. The follow-up after radiotherapy covered 60 months. A combination of microsurgery and radiosurgery for the surgically not amenable lesions is an adequate treatment regimen to stabilize tumor growth and clinical symptoms of multifocal spinal hemangioblastomas in vHLD, though the therapy should be limited to symptomatic or growing lesions 2).

1)
Jagannathan J, Lonser RR, Smith R, DeVroom HL, Oldfield EH. Surgical management of cerebellar hemangioblastomas in patients with von Hippel-Lindau disease. J Neurosurg. 2008 Feb;108(2):210-22. doi: 10.3171/JNS/2008/108/2/0210. PubMed PMID: 18240914.
2)
Knoop N, Seidel C, Frydrychowicz C, Meixensberger J. Combined Microsurgery and Radiotherapy for Multiple Spinal Cord Hemangioblastomas with Holocord Syrinx in von Hippel-Lindau Disease: A Case Report. J Neurol Surg Rep. 2019 Oct;80(4):e46-e50. doi: 10.1055/s-0039-3401808. Epub 2019 Dec 31. PubMed PMID: 31908906; PubMed Central PMCID: PMC6938460.
cerebellar_hemangioblastoma_in_von_hippel-lindau_disease.txt · Last modified: 2020/01/21 15:48 by administrador