[Rare case of the Recklinghausen disease in the head and neck region].

Otolaryngol Pol

Katedra i Klinika Otolaryngologii i Onkologii Laryngologicznej AM w Poznaniu.

Published: February 2007

Introduction: Neurofibromatosis type I (Recklinghausen disease) is one of the most common genetic disorders. Symptoms in head and neck region are present in nearly 30% of cases, but the most frequent are skin café-au-lait lesions or lesions localized in craniofacial region. Neurinomas of the last four cranial nerves are also described.

Material And Methods: We discuss a case of a 50-year woman admitted to our department because of chronic pain symptoms localized in the head and neck region. A rare case of neurinoma of the X and XII nerves in the neck region was stated. This patient was successfully treated surgically with removing tumors, which involved vagal and hypoglossal nerve on the right side.

Conclusions: The causal treatment is until now unknown and patients are carefully observed and scan with MRI. The possible treatment for patients are surgery when the lesion become painful or for cosmetic reasons. Also surgery with radiotherapy is require for tumors with malignant transformation which is possible in 5% of cases. The genetic guidance should be proposed to NF1 patients.

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