A G Dumont1, L Rink2, A K Godwin3, M Miettinen4, H Joensuu5, J R Strosberg6, A Gronchi7, C L Corless8, D Goldstein9, B P Rubin10, R G Maki11, A J Lazar12, D Lev13, J C Trent14, M von Mehren2. 1. Department of Sarcoma Medical Oncology, The University of Texas M. D. Anderson Cancer Center, Houston. 2. Department of Medical Oncology, Fox Chase Cancer Center, Philadelphia. 3. Department of Medical Oncology, Fox Chase Cancer Center, Philadelphia; Department of Pathology and Laboratory Medicine, University of Kansas Medical Center. 4. Department of Soft Tissue Pathology, Armed Forces Institute of Pathology, Washington, USA. 5. Department of Oncology, Helsinki University Central Hospital, Helsinki, Finland. 6. Department of Gastrointestinal Oncology, H. Lee Moffitt Cancer Center, Tampa, USA. 7. Department of Surgery, National Tumor Institute, Milan, Italy. 8. Department of Pathology and Knight Cancer Institute, Oregon Health & Science University, Portland, USA. 9. Department of Medical Oncology, Prince of Wales Hospital, Sydney, Australia. 10. Department of Molecular Genetics, Lerner Research Institute; Department of Anatomic Pathology, Taussig Cancer Center, Cleveland Clinic, Cleveland. 11. Department of Medicine, Memorial Sloan-Kettering Cancer Center, New York. 12. Department of Pathology, The University of Texas M. D. Anderson Cancer Center, Houston. 13. Department of Cancer Biology, The University of Texas M. D. Anderson Cancer Center, Houston, USA. 14. Department of Sarcoma Medical Oncology, The University of Texas M. D. Anderson Cancer Center, Houston. Electronic address: jonctrent@gmail.com.
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.
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.
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