A case of jejunal primitive neuroectodermal tumor in an adult female

Primitive neuroectodermal tumor (PNET) is frequent in children and adolescents, but rare in adults. Most of the extraosseous Ewing's sarcoma or PNET occur in the soft tissues of the extremities, the paravertebral region, and the pelvic cavity. PNET in the gastrointestinal tract is uncommon. We report herein a case of PNET arising from the jejunum in a 38-year-old woman. She presented with anorexia, nausea, vomiting, and weight loss. Abdominal computed tomography revealed a mass in the small bowel with obstruction. She had a small bowel segmental resection, from which PNET was diagnosed. Multi-agent chemotherapy comprised of vincristine, adriamycin, cyclophophamide, ifosfamide, and etoposide (VAC/IE) was administered for 1 year. The treatment was well-tolerated. She remains alive and continues to be disease free 30 months postoperatively. (Korean J Med 75:237-241, 2008)