Frequency, localization, and types of gastrointestinal stromal tumor-associated neoplasia

Abstract

BACKGROUND In recent years, increasing evidence of second neoplasms associated with gastrointestinal stromal tumors (GIST) has been found. Numerous case reports, mostly retrospective studies and a few reviews, have been published. To our knowledge, however, no systematic review or meta-analysis of the existing data has been performed so far. AIM To prepare a compilation, as complete as possible, of all reported second tumor entities that have been described in association with GIST and to systematically analyze the published studies with regard to frequency, localization, and types of GIST-associated neoplasms. METHODS The MEDLINE and EBSCO databases were searched for a combination of the keywords GIST/secondary, synchronous, coincident/tumor, neoplasm, and relevant publications were selected by two independent authors. RESULTS Initially, 3042 publications were found. After deletion of duplicates, 1631 remained, and 130 papers were selected; 22 of these were original studies with a minimum of 20 patients, and 108 were case reports. In the 22 selected studies, comprising a total number of 12050 patients, an overall rate of GIST-associated neoplasias of 20% could be calculated. Most second neoplasias were found in the gastrointestinal tract (32%) and in the male and female urogenital tract (30%). The specific risk scores of GISTs associated with other tumors were significantly lower than those without associated neoplasias. CONCLUSION In this first systematic review, we could confirm previously reported findings of a more than coincidental association between GIST and other neoplasias. The question whether there is an underlying causal association will need further investigation. Our data suggest that even GIST with a very low risk of disease progression should prompt screening for second neoplasia and subsequent frequent controls or extended staging.

Gastrointestinal stromal tumors (GISTs) and synchronous intra-abdominal malignancies: case series of a single institution’s experience

“…GIST-associated sarcomas are rarely reported in the literature considering that soft tissue tumors comprise only 0.5–3% of all GIST-associated neoplasms [ 12 , 14 ]. Cases of synchronous, GIST-associated sarcomas are even less frequently reported and they are usually liposarcomas [ 27 ], angiosarcomas [ 28 ], angiomyoliposarcomas [ 24 ], and leiomyosarcomas [ 29 , 30 ].…”

“…In comparison, the rate of a second malignancy in GIST patients is higher than expected. GISTs with synchronous malignancy of the GI tract are described as ranging from 10% to 35%, with the majority of them being adenocarcinomas [ 1 , 9 – 11 , 12 ].…”

A gastrointestinal stromal tumor of stomach presenting with an intratumoral abscess: A case report

“…GIST are considered to develop from interstitial cells of Cajal (ICC), which play an important role in autonomous gastrointestinal movement. The most common localization of GIST is the stomach, followed by other gastrointestinal tract localizations [ 1 ]. GISTs of the stomach presenting as an intratumoral abscess are extremely rare, which necessitates emergency surgery, we report a case of a stomach GIST developing an intratumoral abscess, in whom emergency surgery was performed.…”

Second Primary Tumors in Patients with Gastrointestinal Stromal Tumors: A Single-Center Experience

“…There are publications in the literature showing that the association is mostly synchronous [ 9 , 10 , 11 , 12 , 13 , 14 , 15 , 16 ]. Synchronous associations often coexist with gastrointestinal cancers [ 11 , 17 ]. The association of GIST with secondary tumors ranges from 4.5% to 33% in different series (average, 13%) [ 9 ].…”

“…This rate was reported to be 21% in a retrospective monocentric analysis by Comandini et al [ 19 ]. Although varying rates have been reported in literature, the association of GISTs with secondary tumors was reported to be 20% in a meta-analysis performed in 2019 [ 11 , 12 ]. Most studies have shown that GISTs are most commonly associated with gastrointestinal and genitourinary system tumors [ 11 , 12 , 14 , 18 , 20 ].…”