颅部与脊柱上皮样血管瘤:系统综述及示例性颅盖病例。
完整摘要 · 中文译文
背景/目的:颅骨和神经轴上皮样血管瘤(EH)极为罕见,且解剖学上具有异质性。我们系统评价了已报道的脑部、颅骨、原发性非骨性脊柱硬膜外及椎体起源且具有临床相关神经轴延伸的EH,并报告一例具有代表性的富血供颅盖病例。 材料与方法:通过PubMed对MEDLINE进行电子检索,并辅以Google Scholar、Crossref、Semantic Scholar、出版商档案、会议/增刊检索,以及截至2026年8月19日的前向和后向引文追踪。纳入标准为经组织学证实的累及脑实质、颅骨并伴相关颅内或颅椎延伸、原发性非骨性脊柱硬膜外间隙,或椎体骨并继发椎管内/椎间孔延伸的EH。 结果:共纳入22篇报告:14篇(63.6%)为椎体起源,8篇(36.4%)为脑部、颅盖-脑部联合、颅骨或原发性非骨性脊柱硬膜外。19/22篇出版物(86.4%)报告了手术,10/22篇(45.5%)报告了放疗,3/22篇(13.6%)报告了术前栓塞。三篇报告明确描述了复发、进展或治疗后新发疾病,但随访异质性妨碍了汇总复发率估计。所述代表性病例为32岁男性,经组织形态学诊断为富血供的矢状旁颅盖EH,压迫通畅的上矢状窦,接受了Onyx栓塞后切除及颅骨成形术。 结论:颅骨和神经轴EH罕见,且存在…… 已发表的证据以椎体疾病为主。治疗应根据解剖分区、神经压迫、结构不稳定性和血供情况个体化制定。在组织学上,必须与上皮样血管内皮瘤和血管肉瘤进行鉴别,而现有证据不足以确定放疗或栓塞治疗的相对疗效。
完整摘要 · 英文原文
BACKGROUND/AIM: Cranial and neuraxial epithelioid hemangioma (EH) is exceptionally rare and anatomically heterogeneous. We systematically reviewed reported cerebral, cranial osseous, primary non-osseous spinal epidural, and vertebral-origin EH with clinically relevant neuraxial extension and present an illustrative hypervascular calvarial case. MATERIALS AND METHODS: Electronic searches of MEDLINE via PubMed were supplemented by Google Scholar, Crossref, Semantic Scholar, publisher archives, conference/supplement searches, and backward and forward citation tracking through 19 August 2026. Histologically confirmed EH involving brain parenchyma, cranial bone with relevant intracranial or craniovertebral extension, primary non-osseous spinal epidural space, or vertebral bone with secondary intraspinal/foraminal extension was eligible. RESULTS: Twenty-two reports were included: 14 (63.6%) vertebral-origin and eight (36.4%) cerebral, combined calvarial-cerebral, cranial osseous, or primary non-osseous spinal epidural. Surgery was reported in 19/22 publications (86.4%), radiotherapy in 10/22 (45.5%), and preoperative embolization in 3/22 (13.6%). Recurrence, progression, or new post-treatment disease was explicitly described in three reports, but heterogeneous follow-up precluded pooled recurrence estimates. The illustrative 32-year-old man had a histomorphologically diagnosed hypervascular parasagittal calvarial EH compressing a patent superior sagittal sinus and underwent Onyx embolization followed by excision and cranioplasty. CONCLUSION: Cranial and neuraxial EH is rare, with published evidence dominated by vertebral disease. Management should be individualized according to anatomical compartment, neural compression, structural instability, and vascularity. Histological distinction from epithelioid hemangioendothelioma and angiosarcoma is essential, while the available evidence is insufficient to establish comparative efficacy of radiotherapy or embolization.