CLINICAL OUTCOMES OF RETROPERITONEAL SARCOMA: A CASE SERIES OF SIX PATIENTS

Abstract

Background: Retroperitoneal sarcomas (RPS) are rare malignancies arising in the retroperitoneum, accounting for a small fraction of adult cancers. They often present late due to their deep location and can reach large sizes, posing unique management challenges.

Methods: We retrospectively reviewed six patients with primary RPS treated at a tertiary center. Clinical data, imaging findings, surgical details, histopathology, and outcomes were collected. Overall survival (OS) was analyzed with Kaplan-Meier methods.

Results: The cohort had a median age of 56 years (range 45–68) and included 4 males and 2 females. Tumor size at presentation was large (median ~20 cm), with common symptoms being abdominal pain or a palpable mass. All patients underwent surgical resection; four required multivisceral en bloc resection of adjacent organs. Histologic subtypes included well-differentiated and dedifferentiated liposarcoma (50% of cases), leiomyosarcoma, undifferentiated pleomorphic sarcoma, and solitary fibrous tumor. Complete gross resection (R0/R1) was achieved in 5 of 6 patients; one had residual disease (R2 resection). After a median follow-up of 36 months, three patients were alive (two disease-free) and three had died of disease. The 3-year OS was ~50%, with a median OS of approximately 30 months.

Conclusion: This case series highlights the aggressive behavior and management complexity of RPS. Complete surgical resection at specialized centers remains the cornerstone of therapy, often necessitating removal of adjacent organs to achieve clear margins. Despite aggressive surgery, recurrence rates are high and overall survival is guarded, consistent with literature. Tailored multidisciplinary strategies and continued research into adjunct therapies are needed to improve outcomes.