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61st Annual Meeting of the German Society of Neurosurgery (DGNC) as part of the Neurowoche 2010
Joint Meeting with the Brazilian Society of Neurosurgery on the 20 September 2010

German Society of Neurosurgery (DGNC)

21 - 25 September 2010, Mannheim

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Hemangioblastomas cause neurogenic polyglobulia

Meeting Abstract

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  • Sven Gläsker - Department of Neurosurgery, Freiburg University Medical Center, Germany
  • Julia Klompen - Department of Neurosurgery, Freiburg University Medical Center, Germany
  • Beate Hippchen - Department of Neurosurgery, Freiburg University Medical Center, Germany
  • Hartmut P. H. Neumann - Department of Internal Medicine, Freiburg University Medical Center, Germany
  • Vera Van Velthoven - Department of Neurosurgery, Freiburg University Medical Center, Germany

Deutsche Gesellschaft für Neurochirurgie. 61. Jahrestagung der Deutschen Gesellschaft für Neurochirurgie (DGNC) im Rahmen der Neurowoche 2010. Mannheim, 21.-25.09.2010. Düsseldorf: German Medical Science GMS Publishing House; 2010. DocP1855

doi: 10.3205/10dgnc326, urn:nbn:de:0183-10dgnc3263

Published: September 16, 2010

© 2010 Gläsker et al.
This is an Open Access article distributed under the terms of the Creative Commons Attribution License (http://creativecommons.org/licenses/by-nc-nd/3.0/deed.en). You are free: to Share – to copy, distribute and transmit the work, provided the original author and source are credited.

Outline

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Objective: Neurogenic polyglobulia has been reported to occur with CNS hemangioblastomas. Suggested mechanisms of haemoglobin elevation include extramedullary hematopoiesis in the tumor tissue, endocrine paraneoplastic effects and germline mutations of the VHL tumor suppressor gene (causing either Chuvash polycythemia or von Hippel-Lindau disease).

Methods: To clarify the frequency and pathomechanism of polyglobulia in CNS hemangioblastomas, we investigated the preoperative and postoperative haemoglobin levels in a series of 164 patients with hemangioblastomas.

Results: Preoperative haemoglobin levels were available from 163 patients. On average, the preoperative haemoglobin levels of hemangioblastoma patients did not differ significantly from the normal population. However, in 24 patients we observed high preoperative haemoglobin levels (>17 g/dl in male or >15 g/dl in female). 8 of these patients had a pathological elevation of preoperative haemoglobin levels (>18,5 g/dl male or >16,5 g/dl female). In all of these patients, removal of the hemangioblastoma resulted in a permanent cure of polyglobulia. 6 of the 8 patients with pathological haemoglobin elevation carried a germline mutation of the VHL tumor suppressor gene.

Conclusions: Neurogenic polyglobulia occurs in a subset of patients with hemangioblastomas. This phenomenon is observed in patients with VHL germline mutations as well as in patients with sporadic hemangioblastomas. Removal of the tumor results in permanent cure of polyglobulia. Our observations suggest that polyglobulia is an effect by the tumor itself, either due to paraneoplasia or extramedullary hematopoiesis.