Current treatment and outcomes of pediatric gastrointestinal stromal tumors (GIST): a systematic review of published studies



Raitio, Arimatias, Salim, Adeline, Mullassery, Dhanya and Losty, Paul D ORCID: 0000-0003-0841-5879
(2021) Current treatment and outcomes of pediatric gastrointestinal stromal tumors (GIST): a systematic review of published studies. PEDIATRIC SURGERY INTERNATIONAL, 37 (9). pp. 1161-1165.

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Abstract

Gastrointestinal stromal tumor (GIST) is a rare cancer of mesenchymal origin mostly seen in adult and elderly populations. Therefore, the prognostic and therapeutic features of pediatric GIST are not clearly defined. Clinical knowledge has been largely extrapolated from case series and adult studies. In this systematic review, we aimed to analyze the health outcome metrics of pediatric GIST. Medline and Embase databases were searched using relevant key terms. The original search retrieved 1,892 titles; 27 studies with 184 patients (68% female) were included for final review. The primary tumors were located in the stomach (165/184, 90%), small bowel (12/184, 7%), and elsewhere (7/184, 4%). Individual patient data were available in 125 cases with a median follow-up of 6.7 years. All patients underwent surgical resection, which varied from wide local excision to total gastrectomy. There were 12 deaths (10%), 65 (52%) patients were alive with no evidence of disease, and 31 cases (25%) were alive with disease. Tumor size > 5 cm, high mitotic index, and spindle morphology were predictive of mortality. Pediatric GIST has a more favorable prognosis and different characteristics versus adult tumors. There is a crucial need for international consensus and specific pediatric guidelines for the treatment of this rare tumor.

Item Type: Article
Uncontrolled Keywords: Gastrointestinal stromal tumor, GIST, Pediatric, Outcome, Survival
Divisions: Faculty of Health and Life Sciences
Faculty of Health and Life Sciences > Institute of Life Courses and Medical Sciences
Depositing User: Symplectic Admin
Date Deposited: 26 May 2021 10:38
Last Modified: 18 Jan 2023 22:44
DOI: 10.1007/s00383-021-04931-0
Related URLs:
URI: https://livrepository.liverpool.ac.uk/id/eprint/3124092