A nonrandom association of gastrointestinal stromal tumor (GIST) and desmoid tumor (deep fibromatosis): Case series of 28 patients Journal Article
| Authors: | Dumont, A. G.; Rink, L.; Godwin, A. K.; Miettinen, M.; Joensuu, H.; Strosberg, J. R.; Gronchi, A.; Corless, C. L.; Goldstein, D.; Rubin, B. P.; Maki, R. G.; Lazar, A. J.; Lev, D.; Trent, J. C.; von Mehren, M. |
| Article Title: | A nonrandom association of gastrointestinal stromal tumor (GIST) and desmoid tumor (deep fibromatosis): Case series of 28 patients |
| Abstract: | Background: Gastrointestinal stromal tumors (GISTs) and desmoid tumors (DTs) are two rare mesenchymal tumor. Anecdotal reports of individuals with both diseases led us to make the hypothesis that the association is a nonrandom event as the probability would be extremely low to observe such cases if they were independent events. Patients and methods: We evaluated the existence of patients with GIST and DT in a large multicenter cohort at 10 institutions in the United States, Australia and Europe. Data on gender, age at diagnosis, KIT, PDGFRA, CTNNB1 mutation status and follow-up time after diagnosis were collected. Results: We identified 28 patients diagnosed with both tumors. DT was diagnosed after GIST in 75% of patients and concomitantly in 21%. In only one case (4%), GIST was diagnosed after DT. KIT or PDGFRA mutations were detected in 12 of 14 GIST, 9 in KIT exon 11, 2 in KIT exon 9 and 1 in PDGFRA. Conclusion: A statistical analysis of these 28 cases suggests a nonrandom association between GIST and DT. Further studies may be able to elucidate the underlying biology responsible for this association. © The Author 2011. Published by Oxford University Press on behalf of the European Society for Medical Oncology. All rights reserved. |
| Keywords: | adult; cancer survival; clinical article; controlled study; aged; aged, 80 and over; middle aged; retrospective studies; gene mutation; overall survival; exon; cancer localization; dose response; united states; follow up; follow-up studies; gastrointestinal stromal tumor; imatinib; disease association; gastrointestinal stromal tumors; cohort studies; incidence; genetic association; mutational analysis; retrospective study; carcinogenesis; europe; oncogene; clinical evaluation; australia; kit; gist; desmoid tumor; beta-catenin; kit gene; fibromatosis, aggressive; deep fibromatosis; pdgfra gene; beta catenin 1 gene |
| Journal Title: | Annals of Oncology |
| Volume: | 23 |
| Issue: | 5 |
| ISSN: | 0923-7534 |
| Publisher: | Oxford University Press |
| Date Published: | 2012-05-01 |
| Start Page: | 1335 |
| End Page: | 1340 |
| Language: | English |
| DOI: | 10.1093/annonc/mdr442 |
| PROVIDER: | scopus |
| PUBMED: | 21994214 |
| PMCID: | PMC3493136 |
| DOI/URL: | |
| Notes: | --- - "Export Date: 4 June 2012" - "CODEN: ANONE" - "Source: Scopus" |
