Dataset Information

Population-Based Epidemiology and Mortality of Small Malignant Gastrointestinal Stromal Tumors in the USA.

ABSTRACT: Gastrointestinal stromal tumors (GISTs) have significant variability in size and malignant behavior. Our current understanding is limited to pathological analyses, autopsy studies, and small case series. The aim of the current study is to define the risk factors, incidence, and mortality rates of GIST <2 cm in the National Cancer Institute's Surveillance, Epidemiology, and End Results database.Patients with histologically confirmed malignant GIST <2 cm were studied from 2001 to 2011. GIST was defined by GI tumor site codes and GIST-specific histology codes.We identified 378 patients with GIST <2 cm. The average age at diagnosis was 64.0 years with equal sex distribution. The most common tumor location was the stomach (62.2 %), followed by the small intestine (23.3 %), colon (5.6 %), and rectum (3.4 %). Most patients had localized disease (79.4 %), but 11.4 % had regional/distant metastatic disease. The annual incidence rate was 4.2 per 10,000,000 (10M). This was the highest among Blacks (7.6 per 10M). Among patients with GIST and no additional cancers, the 5-year GIST-specific mortality was 12.9 %. Moreover, there was a significantly increased 5-year GIST-specific mortality in those patients who had regionally advanced (34.0 %) or metastatic GIST (34.3 %), as compared to those patients with localized GIST (5.6 %).This study represents the first population-based analysis of malignant GIST <2 cm. While quite rare, these tumors have an underappreciated disease-specific mortality. Further studies are needed to define the underlying reasons for the identified racial differences, to develop novel risk assessment schema for patients with these small tumors, and to determine appropriate indications for resection and/or medical therapy.

SUBMITTER: Coe TM

PROVIDER: S-EPMC4880518 | biostudies-literature | 2016 Jun

REPOSITORIES: biostudies-literature

ACCESS DATA

Publications

Population-Based Epidemiology and Mortality of Small Malignant Gastrointestinal Stromal Tumors in the USA.

Coe Taylor M TM Fero Katherine E KE Fanta Paul T PT Mallory Robert J RJ Tang Chih-Min CM Murphy James D JD Sicklick Jason K JK

Journal of gastrointestinal surgery : official journal of the Society for Surgery of the Alimentary Tract 20160329 6

<h4>Background and aims</h4>Gastrointestinal stromal tumors (GISTs) have significant variability in size and malignant behavior. Our current understanding is limited to pathological analyses, autopsy studies, and small case series. The aim of the current study is to define the risk factors, incidence, and mortality rates of GIST <2 cm in the National Cancer Institute's Surveillance, Epidemiology, and End Results database.<h4>Methods</h4>Patients with histologically confirmed malignant GIST <2 cm ...[more]

PMID: 27025710

Similar Datasets

Project description:BackgroundMost gastrointestinal stromal tumors (GISTs) are considered nonhereditary or sporadic. However, single-institution studies suggest that GIST patients develop additional malignancies at increased frequencies. It was hypothesized that greater insight could be gained into possible associations between GISTs and other malignancies with a national cancer database inquiry.MethodsPatients diagnosed with GISTs (2001-2011) in the Surveillance, Epidemiology, and End Results database were included. Standardized prevalence ratios (SPRs) and standardized incidence ratios (SIRs) were used to quantify cancer risks incurred by GIST patients before and after GIST diagnoses, respectively, in comparison with the general US population.ResultsThere were 6112 GIST patients, and 1047 (17.1%) had additional cancers. There were significant increases in overall cancer rates: 44% (SPR, 1.44) before the GIST diagnosis and 66% (SIR, 1.66) after the GIST diagnosis. Malignancies with significantly increased occurrence both before and after diagnoses included other sarcomas (SPR, 5.24; SIR, 4.02), neuroendocrine-carcinoid tumors (SPR, 3.56; SIR, 4.79), non-Hodgkin lymphoma (SPR, 1.69; SIR, 1.76), and colorectal adenocarcinoma (SPR, 1.51; SIR, 2.16). Esophageal adenocarcinoma (SPR, 12.0), bladder adenocarcinoma (SPR, 7.51), melanoma (SPR, 1.46), and prostate adenocarcinoma (SPR, 1.20) were significantly more common only before the GIST diagnosis. Ovarian carcinoma (SIR, 8.72), small intestine adenocarcinoma (SIR, 5.89), papillary thyroid cancer (SIR, 5.16), renal cell carcinoma (SIR, 4.46), hepatobiliary adenocarcinoma (SIR, 3.10), gastric adenocarcinoma (SIR, 2.70), pancreatic adenocarcinoma (SIR, 2.03), uterine adenocarcinoma (SIR, 1.96), non-small cell lung cancer (SIR, 1.74), and transitional cell carcinoma of the bladder (SIR, 1.65) were significantly more common only after the GIST diagnosis.ConclusionsThis is the first population-based study to characterize the associations and temporal relations between GISTs and other cancers by both site and histological type. These associations may carry important clinical implications for future cancer screening and treatment strategies.

Project description: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.

Dataset Information

Population-Based Epidemiology and Mortality of Small Malignant Gastrointestinal Stromal Tumors in the USA.

Publications

Population-Based Epidemiology and Mortality of Small Malignant Gastrointestinal Stromal Tumors in the USA.

Similar Datasets

OmicsDI is part of the ELIXIR infrastructure

Tweets