Surgical resection improves overall survival of patients with small bowel leiomyosarcoma.

PURPOSE: Small bowel leiomyosarcoma (SB LMS) is a rare disease with few studies characterizing its outcomes. This study aims to evaluate surgical outcomes for patients with SB LMS. METHODS: The National Cancer Database was queried from 2004 to 2016 to identify patients with SB LMS who underwent surgical resection. The primary outcome was overall survival. RESULTS: A total of 288 patients with SB LMS who had undergone surgical resection were identified. The median age was 63, and the majority of patients were female (56%), White (82%), and had a Charlson comorbidity score of zero (76%). Eighty-one percent of patients had negative margins following surgical resection. Fourteen percent of patients had metastatic disease at the time of diagnosis. Nineteen percent of patients received chemotherapy and 3% of patients received radiation. One-year overall survival was 77% (95% CI: 72-82%) and 5-year overall survival was 43% (95% CI: 36-49%). Higher grade (HR: 1.98, 95% CI: 1.10-3.55, p = 0.02) and metastatic disease at diagnosis (HR: 2.57, 95% CI: 1.45-4.55, p = 0.001) were independently associated with higher risk of death. CONCLUSION: SB LMS is a rare disease entity, with treatment centering on complete surgical resection. Our results demonstrate that overall survival is higher than previously thought. Timely diagnosis to allow for complete surgical resection is key, and investigation into the possible role of chemotherapy or radiation therapy is needed.