Surgical resection improves overall survival of patients with small bowel leiomyosarcoma
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ORIGINAL ARTICLE
Surgical resection improves overall survival of patients with small bowel leiomyosarcoma Vanessa M. Welten 1 & Adam C. Fields 1 & Pamela W. Lu 1,2 & James Yoo 1 & Joel E. Goldberg 1 & Jennifer Irani 1 & Ronald Bleday 1 & Nelya Melnitchouk 1,2 Accepted: 14 August 2020 # Springer-Verlag GmbH Germany, part of Springer Nature 2020
Abstract 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. Keywords Leiomyosarcoma . Small bowel . outcomes . Survival
Introduction Small bowel leiomyosarcoma (SB LMS) is a rare smooth muscle malignancy that has been poorly characterized [1, 2]. Primary small bowel sarcomas account for less than 10% of cancers in the small intestine [3–5]. The most common small bowel tumor histologies are adenocarcinoma and carcinoid, Vanessa M. Welten and Adam C. Fields contributed equally to this work. Vanessa M. Welten and Adam C. Fields are Co-first authors * Vanessa M. Welten [email protected] 1
Division of General and GI Surgery, Department of Surgery, Brigham and Women’s Hospital, Harvard Medical School, Boston, MA, USA
2
Center for Surgery and Public Health, Brigham and Women’s Hospital, Harvard Medical School, Boston, MA, USA
both accounting for approximately one third of cases, followed by lymphomas, gastrointestinal stromal tumors (GISTs), and leiomyosarcomas (LMS) [6–8]. LMSs account for an estimated 2-3% of small bowel cancers [9]. The advent of immunohistochemical techniques has allowed for the classification of SB LMSs as distinct clinicopathologic entities from GISTs, when previously they had both been classified as LMSs [1, 9, 10]. Specifically, GISTs express c-KIT (CD117),
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