Hemangioblastomas of the central nervous system

TLDR

The authors recommend a screening program for patients with CNS hemangioblastomas to detect or exclude ocular or visceral lesions associated with von Hippel‑Lindau syndrome. A 10‑year German study of 47 hemangioblastomas in 44 patients showed a high cerebellar prevalence (83 %), good postoperative survival, and a 23 % incidence of von Hippel‑Lindau syndrome with associated extra‑neural lesions in seven families.

Abstract

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.