The most common site of involvement of leiomyosarcoma is the retroperitoneum, accounting for approximately 50% of all cases. The case study presented herein describes the journey of a man with a grade 2 retroperitoneal leiomyosarcoma at the time of diagnosis. The patient received first-line doxorubicin (six cycles) and evofosfamide (11 cycles) during participation in the phase III, randomized, double-blind SARC021 trial and achieved stable disease. Upon progression, he received 24 cycles of second-line trabectedin with stable disease, then third-line pazopanib for 14 months with stable disease. Finally, he received fourth-line gemcitabine monotherapy for 5 months until disease progression, which was followed by death. Notably, trabectedin provided long-term disease control and maintained the patient's functional performance throughout treatment.
Keywords: disease control; evofosfamide; retroperitoneal leiomyosarcoma; trabectedin; treatment sequence.