A Case of a Rapidly Growing Malignant Peripheral Nerve Sheath Tumor of the Small Intestine Presenting with Bowel Obstruction

Abstract

A 75-year-old woman presented to our hospital complaining of intermittent abdominal pain. Blood examination revealed anemia, and contrast-enhanced abdominal CT revealed a tumor measuring 9×4 cm in diameter in the small intestinal lumen causing dilatation of the proximal segment of the small intestine. The patient was diagnosed as having small bowel obstruction caused by a tumor, and laparotomy was performed. Intraoperatively, we visualized a small intestinal tumor growing into the lumen without exposure at the serosa, therefore, resection of the small intestinal segment bearing the tumor was performed. On histopathological examination, the tumor was found to be composed of spindle cells arranged in sweeping fascicles. We diagnosed the tumor as a malignant peripheral nerve sheath tumor of the small intestine. A year and five months after the operation, recurrence of the malignant peripheral nerve sheath tumor was suspected in the patient on the basis of worsening of her anemia. We performed a second laparotomy and resected a small intestinal tumor and tumor of the mesentery. Histopathology revealed recurrence of the tumor in the small intestine and peritoneal dissemination. A search of the current literature revealed few reports of cases of malignant peripheral nerve sheath tumor of the small intestine.