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CC BY-NC-ND 4.0 · J Neurol Surg Rep 2019; 80(04): e46-e50
DOI: 10.1055/s-0039-3401808
Case Report
Georg Thieme Verlag KG Stuttgart · New York

Combined Microsurgery and Radiotherapy for Multiple Spinal Cord Hemangioblastomas with Holocord Syrinx in von Hippel-Lindau Disease: A Case Report

Nicolas Knoop
1   Department of Neurosurgery, University of Leipzig, Leipzig, Germany
,
Clemens Seidel
2   Department of Radiotherapy, University of Leipzig, Leipzig, Germany
,
Clara Frydrychowicz
3   Department of Neuropathology, University of Leipzig, Leipzig, Germany
,
Jürgen Meixensberger
1   Department of Neurosurgery, University of Leipzig, Leipzig, Germany
› Institutsangaben
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Publikationsverlauf

18. April 2019

28. September 2019

Publikationsdatum:
31. Dezember 2019 (online)

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Abstract

Spinal and cerebellar hemangioblastomas are common in von Hippel-Lindau disease (vHLD) and usually treated surgically. Multifocal presence and surgically not amenable locations are issues that require a combined microsurgical and radiosurgical approach to control complex cases.

We would like to present the case of 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.

Keywords

hemangioblastoma - von Hippel-Lindau - microsurgery - radiotherapy - syringomyelia - holocord syrinx
 

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