Long-Term Survival after Resection for Primary Undifferentiated Pleomorphic Sarcoma of the Jejunum with Lymph Node Metastases: Case Report

Abstract

Primary undifferentiated pleomorphic sarcoma (UPS) of the small intestine is extremely rare. The prognosis of UPS is basically poor, and particular, when accompanied with metastatic lesions. This paper reports the case of a long-term survivor of primary UPS of the jejunum with lymph node metastases and a skip lesion in the jejunum. The patient was a 50-year-old Japanese man who presented with a chief complaint of breathlessness. Small bowel X-ray series revealed an approximately 4-cm size with protruded lesion (image shows a filling defect) in the proximal jejunum. Based on a presumptive diagnosis of the hemorrhagic small bowel tumor, he underwent a laparotomy. A tumor was observed in the jejunum at approximately 90-cm from the Treitz ligament; some swollen mesenteric lymph nodules were also observed. Segmental resection of the jejunum was performed 20-140 cm from the Treitz ligament. A complete surgical excision with en-bloc regional lymph node dissection was performed. The final histopathological diagnosis was UPS of the jejunum with metastatic lymph nodes and a skip lesion in the jejunum. The postoperative course was uneventful. The patient was not given adjuvant chemotherapy and was discharged on postoperative day 16. He is currently well without any evidence of recurrence for >10 years after the operation.