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.