gms | German Medical Science

60th Annual Meeting of the German Society of Neurosurgery (DGNC)
Joint Meeting with the Benelux countries and Bulgaria

German Society of Neurosurgery (DGNC)

24 - 27 May 2009, Münster

The origin of CNS hemangioblastoma

Meeting Abstract

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  • S. Gläsker - Abteilung Allgemeine Neurochirurgie, Universitätsklinikum Freiburg
  • A. Vortmeyer - Yale University Medical Center, New Haven, USA

Deutsche Gesellschaft für Neurochirurgie. 60. Jahrestagung der Deutschen Gesellschaft für Neurochirurgie (DGNC), Joint Meeting mit den Benelux-Ländern und Bulgarien. Münster, 24.-27.05.2009. Düsseldorf: German Medical Science GMS Publishing House; 2009. DocP08-01

DOI: 10.3205/09dgnc329, URN: urn:nbn:de:0183-09dgnc3299

Published: May 20, 2009

© 2009 Gläsker et al.
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Outline

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Objective: Hemangioblastomas are benign, highly vascularized tumors that can occur sporadically or as a component of von Hippel-Lindau disease, an autosomal dominant tumor syndrome. The cytological and anatomic origin of hemangioblastomas remains unknown.

Methods: To clarify the origin of these tumors we investigated the expression of hemangioblastic marker proteins in ten tumor samples. In addition we performed pathologic-anatomic autopsy studies on grossly normal appearing spinal cord of deceased VHL patients to clarify the anatomic origin of the tumors containing multiple microscopic lesions.

Results: We found that hemangioblastoma tumor cells express proteins (Scl, brachyury, Csf-1R, Gata-1, Flk-1 and Tie-2) that characterize embryonic hemangioblast progenitor cells and suggest that these cells represent the cytological equivalent of the tumor cells. Pathologic-anatomic studies revealed that hemangioblastomas arise at the dorsal root entry zone and progress from microscopic lesions to macroscopic tumors in a step-wise phenotypic pattern.

Conclusions: These findings can explain the highly selective distribution of hemangioblastomas through the CNS: During normal embryonic development, Scl-expressing cells are confined to those locations that give rise to the spinal cord, cerebellum and retina, which exactly matches the distribution of hemangioblastomas.