Understanding Adult Central Nervous System Hemangioblastomas: A Systematic Review

Abstract

Background: Adult hemangioblastomas (HBs) are rare World Health Organization central nervous system (CNS) grade 1 tumors, particularly affecting the posterior cranial fossa. They exhibit a gender bias, impacting men in their fifth and sixth decades of life and manifesting sporadically or as part of von Hippel Lindau disease. Understanding the intricacies of CNS HBs is crucial for clinical decision-making.

Methods: A systematic review of 576 articles was conducted following the Preferred Reporting Items for Systematic Review and Meta-Analyses (PRISMA) guidelines. Eligibility criteria included 3189 adult cases of CNS HBs. Data on patient demographics, tumor characteristics, symptoms, treatment modalities, complications, and outcomes were systematically extracted and synthesized.

Results: The review revealed a heterogeneous demographic distribution, with a male predominance. The median age at diagnosis was 44.7 years. Cranial HBs were more commonly located in the infratentorial (73%) than supratentorial (27%) compartments. Spinal HBs were mostly located in the cervical spine (44.3%), followed by thoracic (36.7%) and lumbar spine (12%). Clinical symptoms varied by location, emphasizing the importance of anatomical considerations. Surgical intervention-total resection (82% of cases)-was the preferred treatment modality, while radiotherapy was less common. Histological examination and immunohistochemistry aided in accurate diagnosis. Complications were location-specific, with intracranial complications more common in infratentorial tumors. Overall, favorable outcomes were prevalent (78% of cases), with low mortality rates.

Conclusions: Adult CNS HBs present with diverse characteristics and clinical manifestations. Surgical intervention remains the mainstay treatment; ongoing research into genetic and molecular mechanisms may enhance our understanding of tumor pathology and lead to improved management strategies in the future.

Keywords: Adults; Hemangioblastoma; Infratentorial; Spinal; Supratentorial; Systematic review.