Cranial and Spinal Epithelioid Hemangioma: Systematic Review and Illustrative Calvarial Case

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.