Hemangioblastomas of the central nervous system. A 10-year study with special reference to von Hippel-Lindau syndrome

J Neurosurg. 1989 Jan;70(1):24-30. doi: 10.3171/jns.1989.70.1.0024.


The findings of a 10-year study (1976 to 1986) conducted in southwest Germany on hemangioblastomas (HBL's) of the central nervous system (CNS) are presented. During that period, 47 HBL's were diagnosed and surgically removed in 44 patients, with a good postoperative survival rate and prognosis. The majority (83%) of these tumors were located in the cerebellum. By thorough clinical examination of the patients and careful evaluation of their family background, it was found that 23% of the HBL patients were afflicted with von Hippel-Lindau syndrome. In addition to the CNS tumors, 14 neoplastic or similar lesions were detected in other tissues. These included angiomatosis of the retinae, pheochromocytomas, pancreatic cysts, renal cysts, and renal carcinoma. The diagnosis of von Hippel-Lindau syndrome was thus established in seven families. The authors suggest the need for a screening program for patients with HBL of the CNS which is designed to confirm or exclude ocular or visceral lesions associated with von Hippel-Lindau syndrome.

MeSH terms

  • Adolescent
  • Adult
  • Aged
  • Angiomatosis / surgery*
  • Brain Neoplasms / complications
  • Brain Neoplasms / diagnostic imaging
  • Brain Neoplasms / surgery*
  • Cerebellar Neoplasms / complications
  • Cerebellar Neoplasms / diagnostic imaging
  • Cerebellar Neoplasms / surgery
  • Child
  • Eye Neoplasms / complications
  • Female
  • Hemangiosarcoma / complications
  • Hemangiosarcoma / diagnostic imaging
  • Hemangiosarcoma / surgery*
  • Humans
  • Kidney Neoplasms / complications
  • Male
  • Microsurgery
  • Middle Aged
  • Pancreatic Cyst / complications
  • Pedigree
  • Retinal Diseases / complications
  • Spinal Neoplasms / complications
  • Spinal Neoplasms / diagnostic imaging
  • Spinal Neoplasms / surgery*
  • Tomography, X-Ray Computed
  • von Hippel-Lindau Disease / diagnostic imaging
  • von Hippel-Lindau Disease / genetics
  • von Hippel-Lindau Disease / surgery*