Abstract | BACKGROUND: METHODS: This is a long-term retrospective analysis of a large observational study of an international open cohort of patients from a GIST research and patient advocacy's lifetime registry. Demographic and disease-specific data were voluntarily supplied by its members from May 2000-October 2010; the primary outcome was overall survival. Associations between survival and prognostic factors were evaluated by univariate Cox proportional hazard analyses, with backward selection at P < 0.05 used to identify independent factors. RESULTS: Inflections in gender ratios by age at diagnosis in years delineated two distinct groups: above and below age 35 at diagnosis. Closer analysis confirmed the above 35 age group as previously reported for adult-type GIST, typified by mixed primary tumor sites and gender, KIT or PDGFRα mutations, and shorter survival times. The pediatric group (< age 18 at diagnosis) was also as previously reported with predominantly stomach tumors, females, wild-type GIST or SDH mutations, and extended survival. "Young adults" however formed a third group aged 18-35 at diagnosis, and were a clear mix of these two previously reported distinct sub-types. CONCLUSIONS: Pediatric- and adult-type GIST have been previously characterized in clinical settings and these observations confirm significant prognostic factors for each from a diverse real-world cohort. Additionally, these findings suggest that extra diligence be taken with "young adults" (aged 18-35 at diagnosis) as pediatric-type GIST may present well beyond adolescence, particularly as these distinct sub-types have different causes, and consequently respond differently to treatments.
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Authors | Jerry Call, Christopher D Walentas, Jens C Eickhoff, Norman Scherzer |
Journal | BMC cancer
(BMC Cancer)
Vol. 12
Pg. 90
(Mar 19 2012)
ISSN: 1471-2407 [Electronic] England |
PMID | 22429770
(Publication Type: Journal Article)
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Chemical References |
- Antineoplastic Agents
- Benzamides
- Piperazines
- Pyrimidines
- Imatinib Mesylate
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Topics |
- Adolescent
- Adult
- Age Factors
- Age of Onset
- Aged
- Aged, 80 and over
- Antineoplastic Agents
(therapeutic use)
- Benzamides
- Child
- Child, Preschool
- DNA Mutational Analysis
- Female
- Gastrointestinal Neoplasms
(drug therapy, genetics, mortality)
- Gastrointestinal Stromal Tumors
(drug therapy, genetics, mortality)
- Humans
- Imatinib Mesylate
- Male
- Middle Aged
- Piperazines
(therapeutic use)
- Prognosis
- Proportional Hazards Models
- Pyrimidines
(therapeutic use)
- Retrospective Studies
- Sex Factors
- Survival Analysis
- Young Adult
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