Sigmoid mesenteric tumour as a manifestation of von Recklinghausen's disease

The gastrointestinal tract may be involved in up to 25% of cases of von Recklinghausen's disease. The authors report such a case in which an 18-year-old girl presented with gastrointestinal symptoms secondary to a tumour in the sigmoid mesentery. There was no family history so it was presumed to be a case of spontaneous mutation. The authors discuss the protein manifestations of neurofibromatosis emphasizing the gastrointestinal presentations and the need for careful surgical management.