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Revista de otorrinolaringología y cirugía de cabeza y cuello

versión On-line ISSN 0718-4816

Resumen

PEREZ B, Sergio; VARELA U, Cecilia  y  DEL VILLAR U, Marcelo. Plexiform neurofibromatosis of nasal TIP. Rev. Otorrinolaringol. Cir. Cabeza Cuello [online]. 2009, vol.69, n.3, pp.255-258. ISSN 0718-4816.  http://dx.doi.org/10.4067/S0718-48162009000300008.

Neurofibromatosis is a genetic, autosomal dominant disease with high penetrance that belongs to the neurocutaneous syndrome group. There are several varieties of neurofibromatosis, of which the best known are neurofibromatosis type 1 (von Recklinghausen disease) and neurofibromatosis type 2 (schwannoma of the bilateral acoustic nerve). We present a case report of a 43 years old female patient, with no relevant family medical history, whose nasal tip size slowly enlarged, in association with considerable nasal obstruction and anosmia. The physical examination highlighted multiple brown, coffee and milk colored spots and nodules of different sizes distributed in the chest and the back. The imaging study of paranasal cavities with computerized tomography showed a son tissue nodule in the cartilaginous portion of the nasal septum. A total tumor resection and open rhinoplasty were performed. The histopatological diagnosis was plexiform neurofibroma of the nasal tip. We present the case given the unusual location of it and make a brief literature review of the topic.

Palabras clave : Neurofibromatosis; von Recklinghausen disease; plexiform neurofibroma.

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