Hypopharyngeal Carcinoma and Intestinal Leiomyosarcoma Associated with von Recklinghausen's Disease: A Case Report

We encountered a case having cutaneous neurofibromatosis associated with intestinal leiomyosarcoma and hypopharyngeal carcinoma. The patient was a 57-year-old female, who was hospitalized at Dokkyo University Hospital in September 1993 complaining of dysphagia. Various tests were made, and a tumor was found at the posterior wall of the right piriformis sinus. As a result of a punch biopsy from the hypopharynx, the patient was diagnosed as having “well-differentiated squamous cell carcinoma. ”By radiotherapy of the hypopharynx, her general condition was temporarily improved, but she underwent an operation for a recurrence of the dysphagia on May 30, 1994. Histopathologically, a three-tumor duplication was found after the operation. This consisted of cutaneous neurofibromatosis, leiomyosarcoma of the small intestine and well-differentiated squamous cell carcinoma of the hypopharynx. Tumor duplication is often seen in neurofibromatosis. Careful observation of patients with von Recklinghausen's disease is necessary.