Gastrointestinal Stromal Tumours (GIST) in Young Adult (18–40 Years) Patients: A Report from the Dutch GIST Registry
Gastrointestinal stromal tumour (GIST) is a disease of older adults and is dominated by <i>KIT</i>/<i>PDGFR</i> mutations. In children, GIST is rare, predominantly occurs in girls, has a stomach location and generally lacks <i>KIT</i>/<i>PDGFR</i> muta...
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| Format: | Article |
| Language: | English |
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MDPI AG
2020-03-01
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| Series: | Cancers |
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| Online Access: | https://www.mdpi.com/2072-6694/12/3/730 |
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| author | 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 |
| author_facet | 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 |
| author_sort | Nikki S. IJzerman |
| collection | DOAJ |
| description | Gastrointestinal stromal tumour (GIST) is a disease of older adults and is dominated by <i>KIT</i>/<i>PDGFR</i> mutations. In children, GIST is rare, predominantly occurs in girls, has a stomach location and generally lacks <i>KIT</i>/<i>PDGFR</i> 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 <i>KIT</i> (69%), <i>PDGFRA</i> (6%), SDH deficient (8%), NF1 associated (4%), <i>ETV6-NTRK3</i> 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-<i>KIT/PDGFRA</i> mutations and a relatively good survival. |
| first_indexed | 2024-03-12T09:49:35Z |
| format | Article |
| id | doaj.art-e430960d88584a68a95b4b45b537a72a |
| institution | Directory Open Access Journal |
| issn | 2072-6694 |
| language | English |
| last_indexed | 2024-03-12T09:49:35Z |
| publishDate | 2020-03-01 |
| publisher | MDPI AG |
| record_format | Article |
| series | Cancers |
| spelling | doaj.art-e430960d88584a68a95b4b45b537a72a2023-09-02T12:39:50ZengMDPI AGCancers2072-66942020-03-0112373010.3390/cancers12030730cancers12030730Gastrointestinal Stromal Tumours (GIST) in Young Adult (18–40 Years) Patients: A Report from the Dutch GIST RegistryNikki S. IJzerman0Cas Drabbe1Dide den Hollander2Mahmoud Mohammadi3Hester van Boven4Ingrid M.E. Desar5Hans Gelderblom6Dirk J. Grünhagen7An K.L. Reyners8Max M. van Noesel9Ron H.J. Mathijssen10Neeltje Steeghs11Winette T.A. van der Graaf12Department of Medical Oncology, Netherlands Cancer Institute, Plesmanlaan 121, 1066 CX Amsterdam, The NetherlandsDepartment of Medical Oncology, Netherlands Cancer Institute, Plesmanlaan 121, 1066 CX Amsterdam, The NetherlandsDepartment of Medical Oncology, Netherlands Cancer Institute, Plesmanlaan 121, 1066 CX Amsterdam, The NetherlandsDepartment of Medical Oncology, Leiden University Medical Center, Albinusdreef 2, 2300 RC Leiden, The NetherlandsDepartment of Pathology, Netherlands Cancer Institute, Plesmanlaan 121, 1066 CX Amsterdam, The NetherlandsDepartment of Medical Oncology, Radboud University Medical Center, Geert Grooteplein Zuid 10, 6525 GA Nijmegen, The NetherlandsDepartment of Medical Oncology, Leiden University Medical Center, Albinusdreef 2, 2300 RC Leiden, The NetherlandsDepartment of Surgical Oncology, Erasmus MC Cancer Institute, Erasmus University Medical Center, Doctor Molewaterplein 40, 3015 GD Rotterdam, The NetherlandsDepartment of Medical Oncology, University Medical Center Groningen, University of Groningen, Hanzeplein 1, 9713 GZ Groningen, The NetherlandsDepartment of Solid Tumors, Princess Maxima Center for Pediatric Oncology, Heidelberglaan 25, 3584 CS Utrecht, The NetherlandsDepartment of Medical Oncology, Erasmus MC Cancer Institute, Erasmus University Medical Center, Doctor Molewaterplein 40, 3015 GD Rotterdam, The NetherlandsDepartment of Medical Oncology, Netherlands Cancer Institute, Plesmanlaan 121, 1066 CX Amsterdam, The NetherlandsDepartment of Medical Oncology, Netherlands Cancer Institute, Plesmanlaan 121, 1066 CX Amsterdam, The NetherlandsGastrointestinal stromal tumour (GIST) is a disease of older adults and is dominated by <i>KIT</i>/<i>PDGFR</i> mutations. In children, GIST is rare, predominantly occurs in girls, has a stomach location and generally lacks <i>KIT</i>/<i>PDGFR</i> 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 <i>KIT</i> (69%), <i>PDGFRA</i> (6%), SDH deficient (8%), NF1 associated (4%), <i>ETV6-NTRK3</i> 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-<i>KIT/PDGFRA</i> mutations and a relatively good survival.https://www.mdpi.com/2072-6694/12/3/730gistyoung-adult patientsmutationstreatmentoutcome |
| spellingShingle | 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 Gastrointestinal Stromal Tumours (GIST) in Young Adult (18–40 Years) Patients: A Report from the Dutch GIST Registry Cancers gist young-adult patients mutations treatment outcome |
| title | Gastrointestinal Stromal Tumours (GIST) in Young Adult (18–40 Years) Patients: A Report from the Dutch GIST Registry |
| title_full | Gastrointestinal Stromal Tumours (GIST) in Young Adult (18–40 Years) Patients: A Report from the Dutch GIST Registry |
| title_fullStr | Gastrointestinal Stromal Tumours (GIST) in Young Adult (18–40 Years) Patients: A Report from the Dutch GIST Registry |
| title_full_unstemmed | Gastrointestinal Stromal Tumours (GIST) in Young Adult (18–40 Years) Patients: A Report from the Dutch GIST Registry |
| title_short | Gastrointestinal Stromal Tumours (GIST) in Young Adult (18–40 Years) Patients: A Report from the Dutch GIST Registry |
| title_sort | gastrointestinal stromal tumours gist in young adult 18 40 years patients a report from the dutch gist registry |
| topic | gist young-adult patients mutations treatment outcome |
| url | https://www.mdpi.com/2072-6694/12/3/730 |
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