Surgical management and outcome of schwannomas in the craniocervical region

Clinical article

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Object

Schwannomas occupying the craniocervical junction (CCJ) are rare and usually originate from the jugular foramen, hypoglossal nerves, and C-1 and C-2 nerves. Although they may have different origins, they may share the same symptoms, surgical approaches, and complications. An extension of these lesions along the posterior fossa cisterns, foramina, and spinal canal—usually involving various cranial nerves (CNs) and the vertebral and cerebellar arteries—poses a surgical challenge. The primary goals of both surgical and radiosurgical management of schwannomas in the CCJ are the preservation and restoration of function of the lower CNs, and of hearing and facial nerve function. The origins of schwannomas in the CCJ and their clinical presentation, surgical management, adjuvant stereotactic radiosurgery, and outcomes in 36 patients treated at Barrow Neurological Institute (BNI) are presented.

Methods

Between 1989 and 2009, 36 patients (mean age 43.6 years, range 17–68 years) with craniocervical schwannomas underwent surgical resection at BNI. The records were reviewed retrospectively regarding clinical presentation, radiographic assessment, surgical approaches, adjuvant therapies, and follow-up outcomes.

Results

Headache or neck pain was present in 72.2% of patients. Cranial nerve impairments, mainly involving the vagus nerve, were present in 14 patients (38.9%). Motor deficits were found in 27.8% of the patients. Sixteen tumors were intra- and extradural, 15 were intradural, and 5 were extradural. Gross-total resection was achieved in 25 patients (69.4%). Adjunctive radiosurgery was used in the management of residual tumor in 8 patients; tumor control was ultimately obtained in all cases.

Conclusions

Surgical removal, which is the treatment of choice, is curative when schwannomas in the CCJ are excised completely. The far-lateral approach and its variations are our preferred approaches for managing these lesions. Most common complications involve deficits of the lower CNs, and their early recognition and rehabilitation are needed. Stereotactic radiosurgery, an important tool for the management of these tumors as adjuvant therapy, can help decrease morbidity rates.

Abbreviations used in this paper: BNI = Barrow Neurological Institute; CCJ = craniocervical junction; CN = cranial nerve; CPA = cerebellopontine angle; GTR = gross-total resection; KPS = Karnofsky Performance Scale; STR = subtotal resection.

Article Information

Address correspondence to: Robert F. Spetzler, M.D., c/o Neuroscience Publications, Barrow Neurological Institute, 350 West Thomas Road, Phoenix, Arizona 85013. email: neuropub@chw.edu.

Please include this information when citing this paper: published online July 9, 2010; DOI: 10.3171/2010.5.JNS0966.

© AANS, except where prohibited by US copyright law.

Headings

Figures

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    Origin of schwannomas in the CCJ. JF = jugular foramen.

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    Craniocervical schwannoma rising from the accessory nerve in a 47-year-old woman. Axial (A), coronal (B), and sagittal (C) MR images showing an enhancing mass in the CPA that extends within the jugular foramen.

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    Jugular foramen schwannoma in a 17-year-old male patient. Coronal (A), axial (B), and sagittal (C) MR images showing a 2-cm enhancing mass within the left jugular foramen.

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    Hypoglossal schwannoma in a 44-year-old man. A: Contrast-enhanced T1-weighted MR image showing a heterogeneously enhancing mass extending from a widened left hypoglossal canal toward the fourth ventricle. B: Coronal contrast-enhanced MR image through the brain showing the mass effect of this lesion on the brainstem and fourth ventricle. C: Postoperative axial T1-weighted MR image obtained after STR.

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    An intra- and extradural C-2 schwannoma in a 23-year-old woman. A and B: Sagittal (A) and coronal (B) T1-weighted MR images showing a homogeneously enhancing mass behind the C-1 and C-2 vertebral bodies. The mass extends from the level of the tip of the odontoid process to the posterior body of C-2. C: Postoperative sagittal T1-weighted MR image confirming complete tumor removal.

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    Postoperative outcome in patients treated for schwannomas in the CCJ.

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    Protocol for surgical management of schwannomas in the CCJ.

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    Kaplan-Meier plot showing recurrence-free survival in 23 patients treated for schwannomas in the CCJ and followed up more than 12 months.

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