# A Rare Case of High-Grade Undifferentiated Pleomorphic Sarcoma of the Terminal Ileum in a 40-Year-Old Man: Successful Outcome and Prolonged Survival After Surgical Resection and Adjuvant Chemotherapy

## TL;DR

A 40-year-old man with a rare intestinal cancer achieved long-term remission after surgery and chemotherapy.

## Contribution

Reports a successful treatment outcome for high-grade undifferentiated pleomorphic sarcoma in a young patient.

## Key findings

- Complete surgical resection with negative margins was achieved in a 40-year-old patient with UPS of the terminal ileum.
- Adjuvant chemotherapy with the AIM regimen contributed to prolonged disease-free survival at 30 months.
- The case suggests that combined surgical and chemotherapy treatment can lead to favorable outcomes in rare UPS cases.

## Abstract

Undifferentiated pleomorphic sarcoma (UPS) of the small intestine is an exceedingly rare malignancy associated with a poor prognosis, characterized by a high risk of recurrence and metastasis. The typical age of onset is between 61 and 70 years. Only a few cases of UPS in the terminal ileum have been successfully treated with complete surgical resection, followed by adjuvant chemotherapy, leading to favorable outcomes and prolonged disease-free survival. Currently, there are no standardized treatment guidelines for UPS due to the tumor’s rarity, and the role of adjuvant chemotherapy in the management of UPS in the small intestine remains inadequately explored in the literature. We present the case of a 40-year-old man diagnosed with high-grade UPS of the terminal ileum. The patient underwent successful surgical resection with negative margins, followed by adjuvant chemotherapy with the Adriamycin, ifosfamide, and mesna (AIM) regimen. At his 30-month follow-up, the patient remains disease-free. In conclusion, the complete surgical resection of UPS of the small intestine, combined with adjuvant chemotherapy, can lead to favorable outcomes and long-term disease-free survival.