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

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 pres...

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Main Authors: Nicolas Knoop, Clemens Seidel, Clara Frydrychowicz, Jürgen Meixensberger
Format: Article
Language:English
Published: Georg Thieme Verlag KG 2019-10-01
Series:Journal of Neurological Surgery Reports
Subjects:
Online Access:http://www.thieme-connect.de/DOI/DOI?10.1055/s-0039-3401808
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author Nicolas Knoop
Clemens Seidel
Clara Frydrychowicz
Jürgen Meixensberger
author_facet Nicolas Knoop
Clemens Seidel
Clara Frydrychowicz
Jürgen Meixensberger
author_sort Nicolas Knoop
collection DOAJ
description 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.
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spelling doaj.art-e48c8dc3c1784075913619b1886d1b5e2022-12-22T00:20:39ZengGeorg Thieme Verlag KGJournal of Neurological Surgery Reports2193-63582193-63662019-10-018004e46e5010.1055/s-0039-3401808Combined Microsurgery and Radiotherapy for Multiple Spinal Cord Hemangioblastomas with Holocord Syrinx in von Hippel-Lindau Disease: A Case ReportNicolas Knoop0Clemens Seidel1Clara Frydrychowicz2Jürgen Meixensberger3Department of Neurosurgery, University of Leipzig, Leipzig, GermanyDepartment of Radiotherapy, University of Leipzig, Leipzig, GermanyDepartment of Neuropathology, University of Leipzig, Leipzig, GermanyDepartment of Neurosurgery, University of Leipzig, Leipzig, GermanySpinal 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.http://www.thieme-connect.de/DOI/DOI?10.1055/s-0039-3401808hemangioblastomavon hippel-lindaumicrosurgeryradiotherapysyringomyeliaholocord syrinx
spellingShingle Nicolas Knoop
Clemens Seidel
Clara Frydrychowicz
Jürgen Meixensberger
Combined Microsurgery and Radiotherapy for Multiple Spinal Cord Hemangioblastomas with Holocord Syrinx in von Hippel-Lindau Disease: A Case Report
Journal of Neurological Surgery Reports
hemangioblastoma
von hippel-lindau
microsurgery
radiotherapy
syringomyelia
holocord syrinx
title Combined Microsurgery and Radiotherapy for Multiple Spinal Cord Hemangioblastomas with Holocord Syrinx in von Hippel-Lindau Disease: A Case Report
title_full Combined Microsurgery and Radiotherapy for Multiple Spinal Cord Hemangioblastomas with Holocord Syrinx in von Hippel-Lindau Disease: A Case Report
title_fullStr Combined Microsurgery and Radiotherapy for Multiple Spinal Cord Hemangioblastomas with Holocord Syrinx in von Hippel-Lindau Disease: A Case Report
title_full_unstemmed Combined Microsurgery and Radiotherapy for Multiple Spinal Cord Hemangioblastomas with Holocord Syrinx in von Hippel-Lindau Disease: A Case Report
title_short Combined Microsurgery and Radiotherapy for Multiple Spinal Cord Hemangioblastomas with Holocord Syrinx in von Hippel-Lindau Disease: A Case Report
title_sort combined microsurgery and radiotherapy for multiple spinal cord hemangioblastomas with holocord syrinx in von hippel lindau disease a case report
topic hemangioblastoma
von hippel-lindau
microsurgery
radiotherapy
syringomyelia
holocord syrinx
url http://www.thieme-connect.de/DOI/DOI?10.1055/s-0039-3401808
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