Surgical Management of Craniovertebral Junction Schwannomas: A Systematic Review

Abstract

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.

18F-FDG PET/CT revealed sporadic schwannomatosis involving the lumbar spinal canal and both lower limbs: a case report

“…The main treatment for schwannomatosis is surgical resection (43,44). However, not all schwannomas necessitate surgery; it is typically reserved for patients with symptoms such as bleeding, pain, neurological dysfunction, and a tendency to malignant transformation (45)(46)(47). The main problem facing neurosurgeons today is to achieve complete tumor resection and prevent recurrence while maximizing the protection of neurological function under the premise of protecting nerve function (47).…”

Outcomes after Surgical Resection of Jugular Foramen Schwannomas: Systematic Review and Meta-Analysis

“…15 The pooled recurrence rate was 10% (95% CI 6 -16, I 2 = 54.6%). Out of 51 patients with recurrence management was described in 33 (64.7%), which included repeat resection (13), SRS (12), and observation (8).…”

“…1 Other analyses have included JFSs with schwannomas of other locations. 8 Thus, we sought to analyze the surgical series for JFSs to describe rates of GTR, postoperative morbidity, and tumor recurrence. These results could guide preoperative patient counseling, serve as a benchmark for surgeons' own outcomes, and provide a comparison for future surgical series of JFS.…”

Incidental craniovertebral junction schwannoma: Surgical or radiosurgical management?

“…Craniovertebral junction (CVJ) schwannomas are rare tumors located at the jugular foramen, hypoglossal canal, or C1‐C2 foramina, showing direct involvement of the atlanto‐occipital and atlanto‐axial joints 1,2 . Symptoms are often misleading, and include neuropathies and neurological dysfunction, while radiological investigation typically document contrast‐enhancing dumbbell‐shaped lesions 3 .…”

“…Craniovertebral junction (CVJ) schwannomas are rare tumors located at the jugular foramen, hypoglossal canal, or C1‐C2 foramina, showing direct involvement of the atlanto‐occipital and atlanto‐axial joints. 1 , 2 Symptoms are often misleading, and include neuropathies and neurological dysfunction, while radiological investigation typically document contrast‐enhancing dumbbell‐shaped lesions. 3 Microsurgical removal is the standard of care to improve symptoms and local control, but stereotactic radiosurgery (SRS) may be considered in patients not eligible to undergo surgery or that refuse it and for treating postsurgical tumor remnants or recurrences.…”