Intestinal leiomyosarcoma and gastroparesis associated with von Recklinghausen's disease

The rare association between intestinal leiomyosarcoma, von Recklinghausen's disease (type-1 neurofibromatosis) and gastroparesis is described. A 20-year-old male, diagnosed 12 years earlier as having pelvic von Recklinghausen's disease, presented with nausea and vomiting. A gastric scintigraphy demonstrated an extremely slow gastric emptying time in the absence of obvious causes for gastroparesis. A small ileal leiomyosarcoma was later found and removed by surgery. The latter was followed by a marked improvement in the clinical condition of the patient.