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A Journal on Otorhinolaryngology, Head and Neck Surgery,
Plastic Reconstructive Surgery, Otoneurosurgery
Indexed/Abstracted in: EMBASE, Scopus
Otorinolaringologia 2007 September;57(3):171-3
Uncommon site of Neurofibromatosis type 1 (NF1) involving the external auditory canal (EAC). Case report
Gargano R., Gallina S., Raineri R., Speciale R., Restivo S.
Department of Otorhinolaryngology University of Palermo, Palermo, Italy
Tumors of neurogenic origin are a small but significant portion of head and neck region masses in children. We report a case of a fifteen-year-old female patient with an uncommon site of Neurofibromatosis type 1 (NF1) involving the external auditory canal. This disease, which is also called Recklinghausen’s disease, is among the most common genetically transmitted diseases, being found in one out of 3000 births. The genetic abnormality of NF1 patients has been localized on the long arm of chromosome 17. This gene is a tumor suppressor gene, the malfunction of which results in part in benign and occasionally malignant sensory nerve tumors. The decision to operate on the NF1 patient rests limited number of surgical principles. Surgical treatment in these patients should serve as a palliative function. In the present case, the tumor was surgically removed without complications.