Understanding Adult Central Nervous System Hemangioblastomas: meta-analysis of meta-analysis.

Adult hemangioblastomas are rare WHO 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 (VHL) disease. Understanding the intricacies of CNS hemangioblastomas is crucial for clinical decision-making. A systematic review of 576 articles was conducted following PRISMA guidelines. Eligibility criteria included 3189 adult cases of CNS hemangioblastomas. Data on patient demographics, tumor characteristics, symptoms, treatment modalities, complications, and outcomes were systematically extracted and synthesized. The review revealed a heterogeneous demographic distribution, with a male predominance. Median age at diagnosis was 44.7 years. Cranial hemangioblastomas were more commonly located in the infratentorial (73 %) than supratentorial (27%) compartments. Spinal hemangioblastomas 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. Adult CNS hemangioblastomas 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.

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