Isolated hypoglossal schwannoma in a 9-year-old child

Case report

Thomas Santarius M.D., Ph.D., F.R.C.S. 1 , Srikanth Dakoji M.B.Ch.B., Ph.D. 1 , Fardad T. Afshari M.B.B.Chir., Ph.D. 1 , Frances L. Raymond M.D., Ph.D., F.R.C.P. 2 , Helen V. Firth D.M., F.R.C.P. 2 , Helen M. Fernandes M.D., F.R.C.S. 1 , and Matthew R. Garnett D.M., F.R.C.S. 1
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  • 1 Departments of Neurosurgery and
  • 2 Medical Genetics, Addenbrooke's Hospital, University of Cambridge, United Kingdom
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The authors report a case of an isolated schwannoma of left hypoglossal nerve in a 9-year-old girl. To the authors' knowledge, this is the first case report of hypoglossal nerve schwannoma in the pediatric population in the absence of neurofibromatosis Type 2.

The patient presented with a 2-month history of morning nausea and vomiting with occasional daytime headaches. Magnetic resonance imaging and subsequent CT scanning revealed a dumbbell tumor with a belly in the lower third of the posterior fossa and head underneath the left jugular foramen. Its neck protruded through an expanded hypoglossal canal. Although the lesion bore radiological characteristics of a hypoglossal schwannoma, the absence of hypoglossal palsy and the apparent lack of such tumors in the pediatric population the preoperative diagnosis was not certain.

The tumor was approached via a midline suboccipital craniotomy, and gross-total resection was achieved. Pathological examination confirmed the diagnosis of schwannoma. Blood and tumor tests for mutations in the NF2 gene were negative. Postoperative mild hypoglossal palsy recovered by the 3-month follow-up, and an MRI study obtained at 1 year did not show recurrence.

Abbreviation used in this paper:NF2 = neurofibromatosis Type 2.

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Contributor Notes

Address correspondence to: Thomas Santarius, M.D., Ph.D., F.R.C.S., Department of Neurosurgery, Addenbrooke's Hospital, Box 166, Hills Road, Cambridge, United Kingdom CB2 0QQ. email: thomas.santarius@cantab.net.

Please include this information when citing this paper: published online June 22, 2012; DOI: 10.3171/2012.3.PEDS11555.

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