Gastrointestinal Stromal Tumours (GIST) in Young Adult (18-40 Years) Patients: A Report from the Dutch GIST Registry

Nikki S IJzerman, Cas Drabbe, Dide den Hollander, Mahmoud Mohammadi, Hester van Boven, Ingrid M E Desar, Hans Gelderblom, Dirk J Grünhagen, An K L Reyners, Max M van Noesel, Ron H J Mathijssen, Neeltje Steeghs, Winette T A van der Graaf

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Abstract

Gastrointestinal stromal tumour (GIST) is a disease of older adults and is dominated by KIT/PDGFR mutations. In children, GIST is rare, predominantly occurs in girls, has a stomach location and generally lacks KIT/PDGFR mutations. For young adults (YA), aged 18 to 40 years, the typical phenotypic and genotypic patterns are unknown. We therefore aimed to describe the clinical, pathological and molecular characteristics of GIST in in YA. YA GIST patients registered in the Dutch GIST Registry (DGR) were included, and data were compared to those of older adults (OA). From 1010 patients in the DGR, 52 patients were YA (54% male). Main tumour locations were stomach (46%) and small intestine (46%). GIST genetic profiles were mutations in KIT (69%), PDGFRA (6%), SDH deficient (8%), NF1 associated (4%), ETV6-NTRK3 gene fusion (2%) or wildtype (10%). Statistically significant differences were found between the OA and YA patients (localisation, syndromic and mutational status). YA presented more often than OA in an emergency setting (18% vs. 9%). The overall five-year survival rate was 85%. In conclusion, YA GISTs are not similar to typical adult GISTs and also differ from paediatric GISTs, as described in the literature. In this series, we found a relatively high percentage of small intestine GIST, emergency presentation, 25% non-KIT/PDGFRA mutations and a relatively good survival.

Original languageEnglish
Article number730
JournalCancers
Volume12
Issue number3
DOIs
Publication statusPublished - 20 Mar 2020

Bibliographical note

Funding:
A research grant for the Dutch GIST Registry was received from Novartis (3017/13), Pfizer (WI189378),
Bayer (2013-MED-12005) and Deciphera (4EE9EEC-7F19-484D-86A4-646CFE0950A5). These funding sources did
not have any involvement in the conduction of this research

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