Neurofibromatosis Type I 환자에서 부인두강에 발생한 거대 신경섬유종증 1례

A Case of Huge Neurofibroma of the Parapharyngeal Space in the Neurofibromatosis Type I Patients

Neurofibromatosis type I or Von Recklinghausen's disease can occur at any site in the body. It is characterized by multiple spots on the skin-more than six spots greater than 1.5cm-, neurofibromas of the peripheral and centarl nervous system, and variety of other dysplastic abnormalities of the skin, bones, endocrine organs, nervous systems, and blood vessels. It is an autosomal dominant trait disease with a frequency of 1 of 3000. Neurofibromatosis is known to be complicated by malignancies. Neurofibromatosis is progressive disease and shows a marked variations in expression in affected individuals. In this report we describe a male patient with neurofibromatosis type I developed in the parapharyngeal space. The patient had huge mass at left parapharyngeal space and inguinal area. We successfully treated the patient with surgery without complication.