Background: Esophageal gastrointestinal stromal tumors (E-GIST) and leiomyosarcoma (E-LMS) are rare tumors. Previous studies are limited to small number of patients. We sought to study these two tumors using a large national database. Methods: The National Cancer Data Base 2004–2014 was queried for patients with E-GIST and E-LMS. The primary outcome was overall survival (OS). Univariate and multivariable Cox regression models were used to investigate OS predictors. Results: We found 141 E-GIST and 38 E-LMS patients, with esophagectomy and systemic treatment rate of 55% and 49% for E-GIST and 50% and 26% for E-LMS. The 5-year OS of E-GIST and E-LMS were 62% and 23%, respectively, p < 0.001. In multivariable analysis, young age, tumor <10 cm, esophagectomy, and E-GIST were associated with superior OS. There was a higher median and mean OS with neoadjuvant vs. upfront surgery for E-GIST group (98 and 111 vs 79 and 80 months). Conclusion: E E-GIST has superior OS compared to E-LMS. Esophagectomy is the cornerstone treatment modality. Further studies are needed to evaluate the role of neoadjuvant therapy in E-GIST patients.
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