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. 2018 Feb;472(2):221-229.
doi: 10.1007/s00428-017-2285-x. Epub 2018 Jan 8.

The incidence, mutational status, risk classification and referral pattern of gastro-intestinal stromal tumours in the Netherlands: a nationwide pathology registry (PALGA) study

Arie J Verschoor  1 , J V M G Bovée  2 , L I H Overbeek  3 ; PALGA group; P C W Hogendoorn  2 , H Gelderblom  4
Collaborators, Affiliations

The incidence, mutational status, risk classification and referral pattern of gastro-intestinal stromal tumours in the Netherlands: a nationwide pathology registry (PALGA) study

Arie J Verschoor et al. Virchows Arch. 2018 Feb.

Abstract

Symptomatic gastrointestinal stromal tumours (GIST) are infrequent with an incidence of 12.7 per million inhabitants in the western population. We studied whether the incidence of GIST has further increased between 2003 and 2012 and assessed the frequency of mutations, risk groups, histological subtypes and immunohistochemistry results. From PALGA, the nationwide Dutch Pathology Registry, pathology excerpts from all patients with a GIST or GIST-like tumour between 2003 and 2012 were retrieved to calculate incidence rates. Full pathology reports were retrieved of resections in 2011 and 2012 to study the frequency of mutations, risk groups, histological subtypes and immunohistochemistry results. The incidence of GIST increased to 17.7 per million inhabitants in 2012 with a median age of 67 years. Mutational analysis was performed in 33.9% of patients with a resection between 2011 and 2012 (KIT mutation 67.5%, PDGFRA 16.3%, wild-type 11.4%). The percentage of high risk patients in the different risk classifications varied from 19.9% to 38.0% depending on the used classification. Only 35.9% of patients had diagnosis or revision of pathology diagnosis within three months in a designated GIST referral centre. No increase in proportion of central pathology reviews was found. Proportion of patients with mutational analysis increased over the years. The registered incidence of GIST, 17.7 per million inhabitants in 2012 in the Netherlands, is still rising. Despite incorporation in the ESMO GIST guidelines since 2008 for mutational testing and since 2010 for central review of pathology, both are performed in a minority of patients.

Keywords: Epidemiology; Gastrointestinal stromal tumours; Incidence; Mutation; Pathology; Soft tissue neoplasms.

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Conflict of interest statement

Research involving human participants

Not applicable.

Informed consent

Because fully anonymized pathology reports were used, no informed consent was obtained.

Conflict of interest statement

Dr. Gelderblom received a grant from Novartis and Pfizer. Dr. Verschoor received a grant from Novartis. Dr. Hogendoorn, Dr Bovée and Dr Overbeek declare that they have no conflicts of interest to declare.

Figures

Fig. 1
Fig. 1
Diagram of inclusion and exclusion of patients
Fig. 2
Fig. 2
a Incidence of GIST standardised for the Dutch population of 2012. b Relative incidence of the four tumour diameter groups
See this image and copyright information in PMC

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