Increased risk of additional cancers among patients with gastrointestinal stromal tumors: A population‐based study

Abstract

Purpose Most gastrointestinal stromal tumors (GIST) are considered non-hereditary or sporadic. However, single-institution studies suggest that GIST patients develop additional malignancies with increased frequencies. We hypothesized that we could gain greater insight into possible associations between GIST and other malignancies using a national cancer database inquiry. Methods Patients diagnosed with GIST (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, when compared with the general U.S. population. Results Of 6,112 GIST patients, 1,047 (17.1%) had additional cancers. There were significant increases in overall cancer rates: 44% (SPR=1.44) before diagnosis and 66% (SIR=1.66) after GIST diagnoses. Malignancies with significantly increased occurrence both before/after diagnoses included other sarcomas (SPR=5.24/SIR=4.02), neuroendocrine-carcinoid tumors (SPR=3.56/SIR=4.79), non-Hodgkin’s 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 GIST. Ovarian carcinoma (SIR=8.72), small intestine adenocarcinoma (SIR=5.89), papillary thyroid cancer (SIR=5.16), renal cell carcinoma (SIR=4.46), hepatobiliary adenocarcinomas (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 GIST. Conclusion This is the first population-based study to characterize the associations and temporal relationships between GIST and other cancers, both by site and histological type. These associations may carry important clinical implications for future cancer screening and treatment strategies.

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

“…Given these high mortality rates and our recent publication describing higher incidences of additional cancers in GIST patients, we queried whether the presence of additional cancers may explain this unexpected finding. 20 Kaplan-Meier analysis, stratified by the presence of additional cancers, demonstrated a significant difference in overall mortality between these groups, with a 5-year mortality of 20.0 % in those with GIST only and 33.8 % in those with GIST and additional cancers ( P = 0.001) (Fig. 1b).…”

“…Agaimy et al estimated that 20–30 % of older adults have GIST <2 cm based upon autopsy cases and surgical pathology analyses. 12 However, this study was limited by several biases, namely (1) it only included gastric GIST cases, (2) it was derived from a single institution, and (3) it analyzed two subsets of patients known to be at higher risk for GIST (i.e., older patients 4 and patients undergoing surgery for other diseases or tumors 20 ). Additionally, Chan et al studied the prevalence of concurrent small GIST in 207 patients undergoing esophagectomy or gastrectomy.…”

“…4 Given this high mortality rate and our previous findings demonstrating a higher incidence of additional cancers among patients with GIST, 20 we excluded patients with additional cancer diagnoses from our analyses. This revealed that the 5-year GIST-specific mortality rate was 12.9 %.…”

A Case of a Gastrointestinal Stromal Tumor Diagnosed at the Postpartum Period

“…Our patient is on 400 mg/day of imatinib mesylate treatment after the surgery and she did not show any sign of disease progression 15 months postoperatively. Murphy et al reported that in their community-based research of patients with the diagnosis of GIST also showed predisposition against other malignancies [18]. Among these cancers the most common ones are genitourinary, breast, respiratory, and hematologic malignancies.…”

Pathologie von neuroendokrinen Neoplasien (NEN) und gastrointestinalen Stromatumoren (GIST)

“…pädiatrischen GIST sind überwiegend im Magen lokalisiert, zeigen oft eine epitheloide Morphologie, sind in bis zu 10 % der Fälle mit Lymphknotenmetastasen assoziiert und haben keine KIT-oder PDGFRA-Mutationen, sondern einen Funktionsverlust ("lossof-function") des Succinatdehydrogenase (SDH)-Komplexes [38]. Interessanterweise sind GIST in bis zu 30 % der Fälle mit anderen, synchron oder metachron auftretenden Neoplasien mit Hauptlokalisation im GI-Trakt assoziiert [41]. In < 5 % der Fälle gehören GIST zum Spektrum von hereditären Tumorsyndromen und in diesem Kontext sind sie auch mit NEN assoziiert.…”