Surgical Management of Craniovertebral Junction Schwannomas: A Systematic Review

background: craniovertebral junction (CVJ) schwannomas are rare, with surgery and stereotactic radiosurgery (SRS) being effective yet challenging options. we systematically reviewed the literature on CVJ schwannomas. methods: pubmed, scopus, web-of-science, and cochrane were searched following the PRISMA statement to include studies reporting CVJ schwannomas. clinical features, management, and outcomes were analyzed. Results: we collected 353 patients from 101 included articles. presenting symptoms were mostly neck pain (30.3%) and headache (26.3%), with most cranial neuropathies involving the XII (31.2%) and X (24.4%) nerves. most tumors originated from C2 (30.9%) and XII (29.4%) nerves, being extracranial (45.1%) and intradural-extradural (44.2%). erosion of C1–C2 vertebrae (37.1%), the hypoglossal canal (28.3%), and/or jugular foramen (20.1%) were noted. all tumors were operated, preferably with the retrosigmoid approach (36.5%), with the far-lateral approach (29.7%) or with the posterior approach and cervical laminectomy (26.9%), far-lateral approaches (14.2%), or suboccipital craniotomy with concurrent cervical laminectomy (14.2%). complete tumor resection was obtained most frequently (61.5%). adjuvant post-surgery stereotactic radiosurgery was delivered in 5.9% patients. median follow-up was 27 months (range, 12–252). symptom improvement was noted in 88.1% of cases, and cranial neuropathies showed improvement in 10.2%. post-surgical complications occurred in 83 patients (23.5%), mostly dysphagia (7.4%), new cranial neuropathies (6.2%), and cerebrospinal fluid leak (5.9%). a total of 16 patients (4.5%) had tumor recurrence and 7 died (2%), with median overall survival of 2.7 months (range, 0.1–252). conclusions: microsurgical resection is safe and effective for CVJ schwannomas. data on SRS efficacy and indications are still lacking, and its role deserves further evaluation.