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.
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.
摘要：
背景：成人血管母细胞瘤是罕见的WHO中枢神经系统（CNS）1级肿瘤，尤其影响后颅窝。他们表现出性别偏见，影响男性的第五个和第六个十年的生活，并偶尔表现为vonHippelLindau（VHL）疾病的一部分。了解中枢神经系统血管母细胞瘤的复杂性对于临床决策至关重要。
方法：根据PRISMA指南对576篇文章进行了系统评价。合格标准包括3189例成人中枢神经系统血管母细胞瘤。患者人口统计数据，肿瘤特征，症状,治疗方式，并发症,并对结果进行了系统的提取和综合。
结果：这篇综述揭示了不同的人口统计学分布，男性占主导地位。诊断时的中位年龄为44.7岁。颅骨血管母细胞瘤更常见于幕下（73％），而不是幕上（27％）。脊髓血管母细胞瘤主要位于颈椎（44.3%），其次是胸椎(36.7%)和腰椎(12%)。临床症状因位置而异，强调解剖学考虑的重要性。手术干预-全切除（82％的病例）-是首选的治疗方式，而放疗不太常见。组织学检查和免疫组化有助于准确诊断。并发症是特定于地点的，以颅内并发症多见于幕下肿瘤。总的来说,有利的结果是普遍的（78%的病例），死亡率低。
结论：成人中枢神经系统血管母细胞瘤具有不同的特征和临床表现。手术干预仍然是主要的治疗方法；正在进行的遗传和分子机制研究可能会增强我们对肿瘤病理学的理解，并导致未来改进的管理策略。
方法：根据PRISMA指南对576篇文章进行了系统评价。合格标准包括3189例成人中枢神经系统血管母细胞瘤。患者人口统计数据，肿瘤特征，症状,治疗方式，并发症,并对结果进行了系统的提取和综合。
结果：这篇综述揭示了不同的人口统计学分布，男性占主导地位。诊断时的中位年龄为44.7岁。颅骨血管母细胞瘤更常见于幕下（73％），而不是幕上（27％）。脊髓血管母细胞瘤主要位于颈椎（44.3%），其次是胸椎(36.7%)和腰椎(12%)。临床症状因位置而异，强调解剖学考虑的重要性。手术干预-全切除（82％的病例）-是首选的治疗方式，而放疗不太常见。组织学检查和免疫组化有助于准确诊断。并发症是特定于地点的，以颅内并发症多见于幕下肿瘤。总的来说,有利的结果是普遍的（78%的病例），死亡率低。
结论：成人中枢神经系统血管母细胞瘤具有不同的特征和临床表现。手术干预仍然是主要的治疗方法；正在进行的遗传和分子机制研究可能会增强我们对肿瘤病理学的理解，并导致未来改进的管理策略。
