Systematic Reviews
Copyright ©The Author(s) 2019. Published by Baishideng Publishing Group Inc. All rights reserved.
World J Gastroenterol. Aug 14, 2019; 25(30): 4261-4277
Published online Aug 14, 2019. doi: 10.3748/wjg.v25.i30.4261
Frequency, localization, and types of gastrointestinal stromal tumor-associated neoplasia
Johanna Waidhauser, Anne Bornemann, Martin Trepel, Bruno Märkl
Johanna Waidhauser, Anne Bornemann, Bruno Märkl, Institute of Pathology and Molecular Diagnostics, University Medical Center Augsburg, Augsburg 86156, Germany
Johanna Waidhauser, Martin Trepel, Department of Hematology and Clinical Oncology, University Medical Center Augsburg, Augsburg 86156, Germany
Author contributions: Waidhauser J performed literature research, collected and analyzed the data, performed statistical analysis and drafted the manuscript. Bornemann A performed literature research. Trepel M provided analytical oversight and revised the manuscript. Märkl B designed the review, was involved in literature research and statistical analysis and revised the manuscript.
Conflict-of-interest statement: All authors declare that no conflicting interests (including but not limited to commercial, personal, political, intellectual or religious interests) exist.
Data sharing statement: Statistical code, and dataset available from the corresponding author at Dryad repository, who will provide a permanent, citable and open-access home for the dataset.
PRISMA 2009 Checklist statement: The guidelines of the Prisma 2009 Statement have been adopted.
Open-Access: This is an open-access article that was selected by an in-house editor and fully peer-reviewed by external reviewers. It is distributed in accordance with the Creative Commons Attribution Non Commercial (CC BY-NC 4.0) license, which permits others to distribute, remix, adapt, build upon this work non-commercially, and license their derivative works on different terms, provided the original work is properly cited and the use is non-commercial. See: http://creativecommons.org/licenses/by-nc/4.0/
Corresponding author: Bruno Märkl, MD, Professor, Institute of Pathology and Molecular Diagnostics, University Medical Center Augsburg, Stenglinstrasse 2, Augsburg 86156, Germany. bruno.maerkl@outlook.de
Telephone: +49-821-4002150 Fax: +49-821-400173199
Received: April 19, 2019
Peer-review started: April 19, 2019
First decision: June 16, 2019
Revised: June 22, 2019
Accepted: July 5, 2019
Article in press: July 5, 2019
Published online: August 14, 2019
Processing time: 118 Days and 3.2 Hours
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.

Keywords: Gastrointestinal stromal tumor; Associated; Secondary; Neoplasia; Tumor

Core tip: Gastrointestinal stromal tumors (GIST) associated neoplasms have been previously reported to occur with a more than coincidental frequency. Numerous case reports, mostly retrospective studies and a few reviews have been published on this topic. In this, to our knowledge, first systematic review we analyzed 108 case reports and 22 retrospective and prospective studies with a total 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%).