Cerebral Hemangioblastoma Without Von Hippel-Lindau Syndrome: A Report of 6 Cases

Int J Surg Pathol. 2021 Apr;29(2):129-134. doi: 10.1177/1066896920933998. Epub 2020 Jun 14.


Background. Hemangioblastoma occurs mainly in the cerebellum and rarely in the cerebrum. Objective. The present study aimed to analyze the clinical manifestations and radiological and pathological features of cerebral hemangioblastoma, and to improve the recognition of this tumor and avoid misdiagnosis. Methods. The characteristics of 6 patients with cerebral hemangioblastoma were analyzed, and a retrospective review of cerebral hemangioblastoma reported in the literature was performed. Results. All 6 patients were female, aged from 22 to 70 years (55 years on average), and all cases were wild-type sporadic, in which 4 cases occurred in the frontal lobe and 2 cases occurred in the parietal lobe. Imaging revealed a solid tumor in 4 cases, a cystic tumor in 1 case, and a mixed tumor in 1 case. Microscopically, the morphology and immunophenotype of tumor cells were not different from those of classical hemangioblastoma. All 6 patients survived tumor free during the follow-up period. Conclusions. Cerebral hemangioblastoma often simulates the imaging characteristics of meningioma or glioma. Enough attention should be paid to differential diagnosis before the operation, and exact diagnosis relies on the pathological examination.

Keywords: cerebellum; cerebrum; clinicopathology; hemangioblastoma; radiology.

Publication types

  • Review

MeSH terms

  • Aged
  • Cerebellar Neoplasms / diagnosis*
  • Cerebellar Neoplasms / mortality
  • Cerebellar Neoplasms / pathology
  • Cerebellar Neoplasms / surgery
  • Cerebellum / diagnostic imaging
  • Cerebellum / pathology*
  • Cerebellum / surgery
  • Diagnosis, Differential
  • Disease-Free Survival
  • Female
  • Follow-Up Studies
  • Glioma / diagnosis
  • Glioma / pathology
  • Hemangioblastoma / diagnosis*
  • Hemangioblastoma / mortality
  • Hemangioblastoma / pathology
  • Hemangioblastoma / surgery
  • Humans
  • Magnetic Resonance Imaging
  • Meningioma / diagnosis
  • Meningioma / pathology
  • Middle Aged
  • Neurosurgical Procedures
  • Retrospective Studies
  • Young Adult