Clinical presentation, management, and maternal-fetal outcomes in gestational hemangioblastoma: a systematic review of case reports and case series

Abstract

Hemangioblastoma is a highly vascular benign tumor of the central nervous system. Its presentation during pregnancy poses diagnostic and management challenges, while the evidence base consists largely of case reports and small case series. We aimed to describe the clinical presentation, management, and maternal-fetal outcomes of hemangioblastoma during pregnancy and to compare sporadic with VHL-associated disease. We searched PubMed, Scopus, Web of Science, EBSCO, and Embase from inception to May 2026 for case reports and case series describing pregnant women with central nervous system hemangioblastoma, including retinal lesions, documented during pregnancy. Continuous variables were summarized as mean ± SD or median (IQR). Comparisons were exploratory and unadjusted for multiplicity. Fifty-three reports contributed data on 80 pregnant women. Mean maternal age was 29.6 ± 5.5 years, and median gestational age at diagnosis was 22 weeks (IQR 13–30). The cerebellum was the most common site (36/80, 45%), followed by the spinal cord (21/80, 26.3%), multiple sites (9/80, 11.3%), and brainstem (7/80, 8.8%). Median maximum lesion dimension was 3.5 cm (IQR 2.5–4.7). Obstructive hydrocephalus occurred in 28/80 patients (35%). Progressive neurological deficits were documented in 69 patients (86.2%). Tumor resection was performed in 48 (60%), conservative management in 29 (36.2%), cerebrospinal fluid diversion in 16 (20%), and embolization in 5 (6.2%). Genetic status was reported in 56 patients (70%). Multiple lesions occurred in 23/24 patients with VHL-associated disease versus 2/25 with sporadic disease ( p < 0.001). Pregnancy was maintained in 72/79 patients (91.1%). Among 70 documented fetal outcomes, there were 41 term and 21 preterm live births, 2 fetal or neonatal deaths, and 6 terminations. Gestational hemangioblastoma is most often reported with posterior fossa or spinal lesions, with neurological symptoms frequently emerging or progressing during pregnancy. VHL-associated disease was characterized by younger age, multiple lesions, and broader anatomical distribution. Findings are hypothesis-generating given heterogeneous reporting and reliance on case reports. Management should be individualized through multidisciplinary neurosurgical and maternal-fetal care.