Background: Retroperitoneal liposarcoma is the commonest adult’s soft tissue sarcoma. It is a slowly growing tumor commonly presents >10cm & at advanced stage. CT is the preferred diagnostic tool. Biopsy is still controversial. Surgery with clear margins is the gold standard for management. Certain situations permit aggressive surgery without R0 resection resulting in inevitable recurrences requiring repeated resection again with improved DFS & OS. CTH protocols are given individually to high grade, failed R0 resections, recurrent & metastatic cases. Well differentiated type carries low metastatic potential but high local recurrence rate with about 90% 5-years OS. Undifferentiated type beside its high local recurrence rate carries high metastatic potential (lungs)
still low than expected (10–15%). Large tumor volume & wide anatomical extents causes’ poor outcome & morbidities with RT. Eventually repeated local recurrences become impossible for surgery with resultant mortality from its local destructive effects. Aims: To study clinicopathological features of all cases of RPLS, presentation, types, prognostic factors, management (surgery, RT & CTH) & outcome (DFS & OS). Materials & Methods: A single institution combined prospective & retrospective analysis of all cases presented to NCI-Cairo University with primary non-metastatic RPLS over period of 8 years from Jan 2012 to Dec 2019. Retrospective study is from Jan 2012 until Dec 2016 & prospective from Jan 2017 until Dec 2019. 52 cases were included. Data collected then analyzed. Results: Mean age was (50.6 years) with males/females ratio 1.2. Tumor sizes ranged (13-43cm) mean & median sizes 11.2 & 9cm respectively. Most tumors sized <20cm (30 cases, 57.6%). Median tumor burden (39cm) ranging (21-89cm). Commonest presentation was distension (31 cases, 57.6%) with mass palpated intra abdominally (41 cases, 78.8%). Preoperative image guided biopsy used only in (29 cases, 55.7%). PET-CT used (21 cases, 40.3%) mainly on follow-up. Median hospital stay was (11 days) ranging (8-31 days). Well differentiated type predominated (31 cases, 59.6%). High grade (III) found in (17 cases, 32.6%). Clear margins obtained in (31 cases, 59.6%) with nearby organs resection in (35 cases 67.3%). Commonest postoperative morbidity was SSI (29 cases, 55.7%). Postoperative mortality was in (6 cases, 11.5%). Postoperative CTH (38 cases, 73%) given to all high-grade, non R0 resection & as palliation in recurrent & metastatic cases (35 cases, 67.3%). Post-operative RT given (15 cases, 28.9%) mainly for non R0 resections unfit for CTH. Median follow up time was (63 months) ranging (0.06-9.31 years). OS at 1, 3 & 5 years were 96.2%, 72.4% & 43.9% respectively while corresponding DFS were 68.7%, 30.8% & 17% respectively.
Keywords: Retroperitoneal liposarcoma, Outcome, NCI, Cairo University