von Recklinghausen's disease with involvement of right parapharyngeal space: report of a case.
A 42-year-old patient of von Recklinghausen's disease suffered from odynophagia, trismus and prominent gag reflex for seven months. Physical examination revealed a firm, nonpulsatile submucosal mass in the right oropharynx. CT scan showed a huge, well-defined mass in the right parapharyngeal space and intracranial involvement was excluded. Pathological examination of the tumor after surgical excision demonstrated a neurofibroma about 7.5 x 4 x 3 cm in size, which was consistent with skin neurofibroma. After surgery the patient was free from the initial complaints and has remained well to the present. von Recklinghausen's disease, also known as neurofibromatosis type I, is characterized by numerous skin neurofibromas, cafe-au-lait spots, etc. It has quite diverse complications occurring in the head and neck region. Parapharyngeal space neurofibroma is one of the rare complications of neurofibromatosis type I, occurring in less than 5% of all parapharyngeal space neoplasia.
Department of Otolaryngology, Chang Gung Memorial Hospital, Taipei, Taiwan, R.O.C.
P T
PT
Yen
F P
FP
Lee
T S
TS
Huang
D000328:Adult; D006801:Humans; D008297:Male; D009456:Neurofibromatosis 1 / Q000473:pathology* / Q000601:surgery; D009960:Oropharynx; D010610:Pharyngeal Neoplasms / Q000473:pathology* / Q000601:surgery