NEUROFIBROMATOSIS INVOLVING THE SMALL-BOWEL ASSOCIATED WITH ADENOCARCINOMA OF THE ILEUM WITH A NEUROENDOCRINE COMPONENT

A patient with neurofibromatosis involving the small bowel who developed adenocarcinoma in an area of the ileum showing diffuse involvement of the disease is presented. Unlike the five cases with this association previously described, the present case shows a neuroendocrine component within the adenocarcinoma. The relation between neurofibromatosis of the bowel wall and adenocarcinoma may be explained by a decreased intestinal transit time in this patient. The presence of a carcinoid tumor in the ileum is not unusual in a patient with von Recklinghausen's disease, although the location of the carcinoid in the duodenum is described more frequently in these patients. The appearance of endocrine/neuroendocrine features within an adenocarcinoma already involved by diffuse neurofibromatosis is exceptional and may not be a chance event.