Clinical features of patients bearing central nervous system hemangioblastoma in von Hippel-Lindau disease

Abstract

Central nervous system (CNS) hemangioblastoma (HB) is one of the most common manifestations in von Hippel-Lindau disease (VHL), but large-scale studies on clinical features of CNS HB in VHL are scarce. On the basis of the results of a questionnaire, we collected data of VHL patients with CNS HB. The total number of CNS HBs in 111 VHL patients (male 59, female 52) was 264 with the following distributions: cerebellar, 65.4%; brainstem, 9.9%; spinal cord, 23.9%; and pituitary, 1. 1%. The follow-up period was 0.6 to 39.2years, with the mean 12.5years. Patients bearing brainstem or spinal cord HB also had another HB significantly more frequently than those bearing cerebellar HBs (P < 0.05). The mean onset age of CNS HB was 29.1years, and that of patients bearing a single HB (mean 34.4years) was significantly greater than that of multiple HBs (mean 25.7years). Patients with multiple HBs under 40years are more dominant than those with a single HB. The distribution rate of brainstem HB is significantly smaller in patients below 30years than patients above 29years. Although ECOG PS score increased along with number of operations, the onset age decreased with increasing number of operations. The mean ECOG PS score of patients below 20years is significantly smaller than patients above 19years. When the onset age of CNS HB is under 40years, and CNS HB is located at the brainstem or spinal cord HB, the probability of multiple occurrence can be predicted. Since patients with an onset age under 20years old preserve a high performance status, early detection of CNS HB would be important. In addition, since a multiple operations aggravate performance status, number of operations should be reduced.