Ileal ganglioneuromatosis with adenocarcinoma in a patient with multiple neurofibromatosis.

Von Recklinghausen's disease or type 1 neurofibromatosis is an autosomal dominant disease characterized by mucocutaneous neurofibromas, café-au-lait spots and involves many organs including gastrointestinal tract. Gastrointestinal involvement in neurofibromatosis is uncommon and is seen 25% patients and nearly 5% of them are only symptomatic (Shekitka and Sobin, Am J Surg Pathol 18:250-257, 1994; Tomita et al., Int J Colorectal Dis 21: 89-91, 2006).