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

Acta Neurochir (Wien). 2019 Feb;161(2):343-349. doi: 10.1007/s00701-019-03800-z. Epub 2019 Jan 17.


Background: 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.

Conclusion: 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.

Keywords: Central nervous system; Disseminated disease; Hemangioblastoma; Leptomeningeal carcinomatosis; Metastatic disease; von Hippel-Lindau disease.

Publication types

  • Case Reports
  • Review

MeSH terms

  • Adult
  • Cerebellar Neoplasms / pathology
  • Cerebellar Neoplasms / radiotherapy
  • Cerebellar Neoplasms / surgery*
  • Diagnosis, Differential
  • Female
  • Hemangioblastoma / pathology
  • Hemangioblastoma / radiotherapy
  • Hemangioblastoma / surgery*
  • Humans
  • Neoplasm Metastasis
  • von Hippel-Lindau Disease / genetics
  • von Hippel-Lindau Disease / pathology*