An adenocarcinoma developed from the overlying mucosa of an ileal ganglioneuroma and an associated ileal stromal tumor in a patient with neurofibromatosis

Ileal resection of a 57 year old woman with Neurofibromatosis, showed a ganglioneuroma admixed with an adenocarcinoma. 10 cm away from this neoplasm, was another nodule of small intestinal stromal tumor (SIST) which was located in subserosa. Although intestinal ganglioneuromatosis was reported in patients with neurofibromatosis, it is quite rare to find it admixed with an adenocarcinoma. To date in these patients, an associated SIST was also rarely reported. Development of an adenocarcinoma from the overlying mucosa of an ileal ganglioneuroma and an additional SIST away from this neoplasm is an unusual association in a patient with Neurofibromatosis.