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RESEARCH PRODUCT

Novel clinical insights into spinal hemangioblastoma in adults: a systematic review

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ABSTRACT Background Hemangioblastomas are well vascularized, benign CNS tumors and the third most common primary spinal cord tumor after astrocytoma/ependymoma, occurring sporadically or as a part of an autosomal dominant von Hippel-Lindau disease in which tumors are often multiple and prone to relapse. Spinal hemangioblastomas are commonly located in the cervical cord and associated with a syrinx formation. Due to location and growth trends, they may cause significant neurological deficit, impairing patient quality of life. We conducted a systematic review to understand better clinical insights of spinal hemangioblastoma in adults and compare spinal hemangioblastoma versus posterior cranial fossa hemangioblastoma. Methods Followed PRISMA guidelines for conducting systematic reviews, we performed a review of the English literature on adult spinal hemangioblastoma in the MEDLINE/PubMed database over the last 40 years. Results We reviewed 237 total scientific articles on adult spinal hemangioblastoma and analyzed national and continental distribution, clinical symptomatology, tumor location and presence of syringomyelia, treatment strategies and postoperative complications, histology and immunochemistry, as well as treatment outcomes. We also compared individual characteristics in both sporadic and von Hippel-Lindau Disease spinal hemangioblastomas. Finally, we compared features of posterior cranial fossa and spinal hemangioblastomas. Conclusion Spinal cord hemangioblastomas are most commonly located dorsally and intramedullary. Total surgical tumor resection is the first treatment option, while preoperative embolization may be performed to reduce intraoperative bleeding and surgical time. Hemangioblastomas located in the spine have decreased mortality and rate of infection, but increased rates of cardiopulmonary complications compared with those found in the posterior cranial fossa.