Primary iliocaval leiomyosarcomas: The path beyond surgery

Introduction Iliocaval leiomyosarcoma (ICLM) is a rare and aggressive form of sarcoma within the retroperitoneum. Surgery is the mainstay of treatment, with no consensus on the benefit of chemoradiotherapy in the neo/adjuvant setting. This study aims to describe the natural history of a chemotherapy-naive ICLM treated in a tertiary cancer centre and to explore potential directions to improve oncological outcome. Materials and methods A prospective database was used to identify patient demographics, clinicopathological variables and oncological outcomes in 30 patients who underwent surgical resection in our institution for primary non-metastatic ICLM between 2003 and 2018. Results There was no 90-day mortality. With a median follow-up time of 70.0 months (95% CI 52.6–87.4), 5/30 patients (16.7%) developed local recurrence while 11/30 (36.7%) developed distant metastatic disease. 1 patient (3.3%) developed both local and distant recurrence. Median overall survival of our cohort was 41.0 months (95% CI 33.6–48.4) and 5-year overall survival rate was 32.1%. Multivariate survival analysis using the Cox proportional hazard model identified tumour grade and blood loss of more than 600 mL as key prognostic factors in our model. Conclusion Management of ICLM should be centralised in high-volume sarcoma centres with expertise in the management of retroperitoneal sarcomas. Integration of tumour biology with a concerted effort to conduct conclusive multi-centre phase III in histological and molecularly defined sarcoma subgroups is necessary to improve patient outcome. We eagerly await the results of STRASS 2 study to gain more insights to the efficacy of neoadjuvant chemotherapy on patient prognosis.