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.
Highlights
IntroductionHigh-grade pleomorphic malignant soft tissue tumors that do not differentiate into specific histological features are classified as undifferentiated pleomorphic sarcoma (UPS), previously known as malignant fibrous histiocytoma (MFH)
This paper reports the case of a longterm survivor of primary undifferentiated pleomorphic sarcoma (UPS) of the jejunum with lymph node metastases and a skip lesion in the jejunum
High-grade pleomorphic malignant soft tissue tumors that do not differentiate into specific histological features are classified as undifferentiated pleomorphic sarcoma (UPS), previously known as malignant fibrous histiocytoma (MFH)
Summary
High-grade pleomorphic malignant soft tissue tumors that do not differentiate into specific histological features are classified as undifferentiated pleomorphic sarcoma (UPS), previously known as malignant fibrous histiocytoma (MFH). UPS usually occurs in the soft tissues of the extremities. Metastasis occurs via the haematogenous route, with the lung being the most common site of metastatic disease. The prognosis of UPS is basically poor, when accompanied with metastatic lesions is further poorer.
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