J Neurol Surg A Cent Eur Neurosurg 2013; 74(01): 054-058
DOI: 10.1055/s-0032-1324802
Case Report
Georg Thieme Verlag KG Stuttgart · New York

Giant Dumbbell-Shaped Intra- and Extracranial Nerve Schwannoma in a Child Presenting with Glossopharyngeal Neuralgia Syncope Syndrome: A Case Report and Review of the Literature

Sevgi Sarikaya-Seiwert
1   Department of Neurosurgery, Heinrich-Heine-University, Düsseldorf, Germany
Thomas Klenzner
2   Department of Otorhinolaryngology, Heinrich-Heine-University, Düsseldorf, Germany
Jörg Schipper
2   Department of Otorhinolaryngology, Heinrich-Heine-University, Düsseldorf, Germany
Hans-Jakob Steiger
1   Department of Neurosurgery, Heinrich-Heine-University, Düsseldorf, Germany
Daniel Haenggi
1   Department of Neurosurgery, Heinrich-Heine-University, Düsseldorf, Germany
› Author Affiliations
Further Information

Publication History

11 June 2011

17 April 2012

Publication Date:
26 October 2012 (online)


Lower cranial nerve schwannomas are benign tumors of the neurolemmocytes of the cranial nerves. Among children, cranial nerve schwannomas are extremely rare and are predominantly associated with neurofibromatosis (NF) type 2. The purpose of the current case report is to describe a unique giant extra- and intracranial foramen jugular schwannoma in a young boy with lower cranial nerve deficits and glossopharyngeal neuralgia syncope syndrome and to review the pertinent literature. In the current case report, we illustrate the course of disease in a 14-year-old boy with a 4-month history of recurrent syncope and a big bulge on the left side of his neck. Audiometry showed deafness of the left ear. Magnetic resonance imaging (MRI) demonstrated a giant unilateral dumbbell-shaped intra- and extracranial foramen jugular schwannoma with a volume of 156 cm3 causing severe brain stem compression and obstructive hydrocephalus. The tumor was removed completely in a two-step surgery. The tumor was confirmed during surgery to originate from the glossopharyngeal nerve. The histological examination revealed the characteristic features of a schwannoma. The MRI 3 months after the second surgery confirmed complete tumor removal. The genetic examination for NF was negative. Review of literature showed that dumbbell-shaped lower cranial nerve schwannomas in the childhood population are rare.

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