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57. Jahrestagung der Deutschen Gesellschaft für Neuropathologie und Neuroanatomie (DGNN)

Deutsche Gesellschaft für Neuropathologie und Neuroanatomie

12. - 15.09.2012, Erlangen

Banner: 57. Jahrestagung der Deutschen Gesellschaft für Neuropathologie und Neuroanatomie

Rhabdomyoma of the Internal Auditory Canal – Case Report

Meeting Abstract

Suche in Medline nach

  • presenting/speaker Ahmed Rizk - Uniklik Tuebingen, Neurosurgery, Tuebingen, Germany; Benha University, Neurosurgery, Tuebingen, Egypt
  • Florian Ebner - Uniklik Tuebingen, Neurochirurgie, Tuebingen, Germany
  • Martin Schuhmann - Uniklik Tuebingen, Neurochirurgie, Tuebingen, Germany
  • Marcos Tatagiba - Uniklik Tuebingen, Neurochirurgie, Tuebingen, Germany

Deutsche Gesellschaft für Neuropathologie und Neuroanatomie. 57th Annual Meeting of the German Society for Neuropathology and Neuroanatomy (DGNN). Erlangen, 12.-15.09.2012. Düsseldorf: German Medical Science GMS Publishing House; 2012. Doc12dgnnPP3.28

DOI: 10.3205/12dgnn072, URN: urn:nbn:de:0183-12dgnn0727

Veröffentlicht: 11. September 2012

© 2012 Rizk et al.
Dieser Artikel ist ein Open Access-Artikel und steht unter den Creative Commons Lizenzbedingungen (http://creativecommons.org/licenses/by-nc-nd/3.0/deed.de). Er darf vervielfältigt, verbreitet und öffentlich zugänglich gemacht werden, vorausgesetzt dass Autor und Quelle genannt werden.


Gliederung

Text

The case of a five-year-old child is presented, who had left sided sensorineural hearing loss, lower motor neuron facial weakness on the left side (HB grade 3), and gait ataxia. A brain MRI with gadolinium showed a homogenously enhancing lesion that fills the internal auditory canal (IAC) and extends into the cerebellopontine angle with brain stem compression and associated edema in the brain stem. CT scans of the brain with bone window showed widening and erosion of the left IAC. Open biopsy performed in another hospital resulted in neuropathological diagnosis of a teratoma. Microsurgical tumor excision was performed through a retrosigmoid approach. The tumor was hardly dissected from the brain stem. Since both facial and cochlear nerves were infiltrated by the tumor, they could not be preserved. The proximal and distal ends of the facial nerve were not available; hence early hypoglossal facial anastmosis was performed 2 weeks later. Pathology showed well-differentiated skeletal muscle fibers and individual ganglion cells, with no evidence of malignant changes. Muscle fibers showed diffuse immunoreactivity for Desmin, and ganglion cells stained for Neurofilament. The diagnosis of rhabdomyoma was made.

Rhabdomyoma is an extremely rare pathological entity in the CPA. There are several case reports of hamrtomas and choristomas in the CPA, but so fare only another case of rhabdomyoma has been described in literature. We suggest that early exploration of this congenital tumor increases the chance of successful radical surgery.