Unmasking the uncommon: retroperitoneal Leiomyosarcoma case report

Leiomyosarcoma is a rare subtype of soft tissue sarcoma originating from smooth muscle cells. The clinical presentation varies based on the tumor's location. We report the case of a 63-year-old woman with no significant medical history, who presented with persistent epigastric pain. A suspected lymphadenopathy was found on a CT scan. Following surgical excision, histopathology and immunohistochemistry confirmed the diagnosis of leiomyosarcoma, negative for CD117 and Dog1, but positive for H-Caldesmon. Retroperitoneal leiomyosarcoma is aggressive and rare, making diagnosis difficult prior to histopathology. Complete surgical resection with negative margins is the gold standard of treatment, though it can be challenging to achieve. A multidisciplinary approach is crucial to improve survival and quality of life. The patient is under regular follow-up and remains free of recurrence six months post-operatively.