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Peter D. Costantino, MD; Craig D. Friedman, MD; Harold J. Pelzer, DDS, MD

Arch Otolaryngol Head Neck Surg. 1989;115(3):380-383.

Abstract
• Neurofibromatosis type II (NF-II) has been traditionally referred to as "acoustic" neurofibromatosis and is not known to be genetically distinct from classic von Recklinghausen's disease (NF-I). Neurofibromatosis type II is due to a lesion on chromosome 22q, while von Recklinghausen's neurofibromatosis is from a defect on chromosome 17. The approximate incidence of NF-II is one in 50000, with bilateral acoustic neuromas occurring in over 90% of those with the abnormal gene. We studied a 17-year-old boy with NF-II who presented with bilateral acoustic neuromas and a concomitant primary nasopharyngeal meningioma. Less than 30 nasopharyngeal meningiomas have been documented, and the presence of both tumor types in the same individual is unique in the literature.

(Arch Otolaryngol Head Neck Surg 1989;115:380-383)

Author Affiliations
From the Department of Otolaryngology–Head and Neck Surgery, Northwestern University Medical School, Chicago.

Footnotes
Accepted for publication Sept 9, 1988.

Presented as a poster at the Second International Conference on Head and Neck Cancer, Boston, July 31 to Aug 4, 1988.

Reprint requests to Department of Otolaryngology–Head and Neck Surgery, Northwestern University Medical School, Searle Bldg 12-561, 303 E Chicago Ave, Chicago, IL 60611 (Dr Costantino).

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