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Original Investigation
January 18, 2017

Surgical Management of Adolescents and Young Adults With Gastrointestinal Stromal TumorsA US Population-Based Analysis

Author Affiliations
  • 1School of Medicine, University of California, San Diego, La Jolla
  • 2Department of Surgery, Massachusetts General Hospital, Harvard Medical School, Boston
  • 3Moores Cancer Center, Division of Medical Oncology, Department of Medicine, University of California, San Diego, La Jolla
  • 4Moores Cancer Center, Division of Surgical Oncology, Department of Surgery, University of California, San Diego, La Jolla
  • 5Moores Cancer Center, Department of Radiation Medicine and Applied Sciences, University of California, San Diego, La Jolla
JAMA Surg. Published online January 18, 2017. doi:10.1001/jamasurg.2016.5047
Key Points

Question  What is the association of operative management with overall and gastrointestinal stromal tumor–specific survival in adolescent and young adult patients with gastrointestinal stromal tumors?

Findings  In this retrospective cohort study of 392 adolescent and young adult patients and 5373 older adult patients with histologically diagnosed gastrointestinal stromal tumors in the Surveillance, Epidemiology, and End Results database, the adolescent and young adult patients were more likely to undergo surgical management than the older adult patients.

Meaning  Additional studies are needed to understand the long-term outcomes of adolescent and young adult patients with gastrointestinal stromal tumors and to further define the role of operative management among patients with metastatic disease in a more personalized manner.

Abstract

Importance  There is a dearth of population-based evidence regarding outcomes of the adolescent and young adult (AYA) population with gastrointestinal stromal tumors (GISTs).

Objectives  To describe a large cohort of AYA patients with GISTs and investigate the effect of surgery on GIST-specific survival (GSS) and overall survival (OS).

Design, Setting, and Participants  This retrospective cohort study of 392 AYA patients and 5373 older adult (OA) patients in the Surveillance, Epidemiology, and End Results (SEER) database with GISTs histologically diagnosed from January 1, 2001, through December 31, 2013, with follow-up through December 31, 2015, compared the baseline characteristics of AYA (13-39 years old) and OA (≥40 years old) patients and among AYA patients stratified by operative management. Kaplan-Meier estimates were used for OS analyses. Cumulative incidence functions were used for GSS analysis. The effect of surgery on survival was evaluated with a multivariable Fine-Gray regression model.

Exposure  Tumor resection.

Main Outcomes and Measures  GIST-specific survival and OS.

Results  This study included 392 AYA and 5373 OA patients diagnosed with GISTs (207 [52.8%] male AYA patients, 2767 [51.5%] male OA patients, 277 [70.7%] white AYA patients, and 3661 [68.1%] white OA patients). Compared with the OA patients, more AYA patients had small-intestine GISTs (139 [35.5%] vs 1465 [27.3%], P = .008) and were managed operatively (332 [84.7%] vs 4212 [78.4%], P = .003). Multivariable analysis of AYA patients found that nonoperative management was associated with a more than 2-fold increased risk of death from GISTs (subdistribution hazard ratio, 2.27; 95% CI, 1.21-2.25; P = .01). On subset analysis of 349 AYA patients with tumors of the stomach and small intestine, small-intestine location was associated with improved survival (OS: 91.1% vs 77.2%, P = .01; GSS: 91.8% vs 78.0%, P = .008). On subset analysis of 91 AYA patients with metastatic disease, operative management was associated with improved survival (OS: 69.5% vs 53.7%, P = .04; GSS: 71.5% vs 56.7%, P = .03).

Conclusions and Relevance  This study found that AYA patients are more likely to undergo surgical management than OA patients. Operative management is associated with improved OS and GSS in AYA patients, including those with metastatic disease.

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