Acta Neurochirurgica

, Volume 161, Issue 2, pp 343–349 | Cite as

Disseminated central nervous system hemangioblastoma in a patient with no clinical or genetic evidence of von Hippel-Lindau disease—a case report and literature review

  • Simer J. BainsEmail author
  • Pitt F. Niehusmann
  • Torstein R. Meling
  • Cathrine Saxhaug
  • Mark Züchner
  • Petter Brandal
Case Report - Brain Tumors
Part of the following topical collections:
  1. Brain Tumors



Hemangioblastomas (HB) are benign tumors of the central nervous system (CNS) that can appear sporadic or as part of von Hippel-Lindau (VHL) disease. It is often curable with surgical resection, but upon relapse, the disease exhibits a treatment-refractory course.

Case report

A patient treated for sporadic cerebellar HB relapsed 12 years post-surgery. She developed disseminated disease throughout the CNS, including leptomeningeal manifestations. Repeat surgery and craniospinal radiation therapy were unsuccessful.


This case is in line with previous publications on disseminated non-VHL HB. Available treatment options are inefficient, emphasizing the need for improved understanding of HB biology to identify therapeutic targets.


Central nervous system Disseminated disease Hemangioblastoma Leptomeningeal carcinomatosis Metastatic disease von Hippel-Lindau disease 


Compliance with ethical standards

The patient has consented to the writing and submission of the case report. The interpretation and reporting of this case is the sole responsibility of the authors. The case report has been written in accordance with COPE guidelines and comply with the CARE statement. There are no conflicts of interests for any of the contributing authors. No portion of this case report has been presented or published previously.


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Copyright information

© Springer-Verlag GmbH Austria, part of Springer Nature 2019

Authors and Affiliations

  1. 1.Department of OncologyAkershus University HospitalLørenskogNorway
  2. 2.Department of NeuropathologyOslo University HospitalOsloNorway
  3. 3.Department of NeurosurgeryOslo University HospitalOsloNorway
  4. 4.Department of RadiologyOslo University HospitalOsloNorway
  5. 5.Department of OncologyOslo University HospitalOsloNorway
  6. 6.Section for Cancer Cytogenetics, Institute for Cancer Genetics and InformaticsOslo University HospitalOsloNorway
  7. 7.Centre for Cancer Biomedicine, Faculty of MedicineUniversity of OsloOsloNorway

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