Population-based study of islet cell carcinoma.
BACKGROUND
We examine the epidemiology, natural history, and prognostic factors that affect the duration of survival for islet cell carcinoma by using population-based registries.


METHODS
The Surveillance, Epidemiology, and End Results (SEER) Program database (1973-2003 release, April 2006) was used to identify cases of islet cell carcinoma by histology codes and tumor site.


RESULTS
A total of 1310 (619 women and 691 men) cases with a median age of 59 years were identified. The annual age-adjusted incidence in the periods covered by SEER 9 (1973-1991), SEER 13 (1992-1999), and SEER 17 (2000-2003) were .16, .14, and .12 per 100,000, respectively. The estimated 28-year limited duration prevalence on January 1, 2003, in the United States was 2705 cases. Classified by SEER stage, localized, regional, and distant stages corresponded to 14%, 23%, and 54% of cases. The median survival was 38 months. By stage, median survival for patients with localized, regional, and distant disease were 124 (95% CI, 80-168) months, 70 (95% CI, 54-86) months, and 23 (95% CI, 20-26) months, respectively. By multivariate Cox proportional modeling, stage (P < .001), primary tumor location (P = .04), and age at diagnosis (P < .001) were found to be significant predictors of survival.


CONCLUSIONS
Islet cell carcinomas account for approximately 1.3% of cancers arising in the pancreas. Most patients have advanced disease at the time of diagnosis. Despite the disease's reputation of being indolent, survival of patients with advanced disease remains only 2 years. Development of novel therapeutic approaches is needed.
Yao
James C
JC
Department of Gastrointestinal Medical Oncology, The University of Texas M. D. Anderson Cancer Center, 1515 Holcombe Boulevard, Houston, Texas 77030, USA. jyao@mdanderson.org
Eisner
Milton P
MP
Leary
Colleen
C
Dagohoy
Cecile
C
Phan
Alexandria
A
Rashid
Asif
A
Hassan
Manal
M
Evans
Douglas B
DB
D000293:Adolescent; D000328:Adult; D017677:Age Distribution; D000368:Aged; D000369:Aged, 80 and over; D018273:Carcinoma, Islet Cell / Q000453:epidemiology* / Q000473:pathology; D002648:Child; D002675:Child, Preschool; D005260:Female; D015408:Gastrinoma / Q000453:epidemiology / Q000473:pathology; D005935:Glucagonoma / Q000453:epidemiology / Q000473:pathology; D006801:Humans; D015994:Incidence; D007223:Infant; D007231:Infant, Newborn; D007340:Insulinoma / Q000453:epidemiology / Q000473:pathology; D008297:Male; D008875:Middle Aged; D009367:Neoplasm Staging; D010190:Pancreatic Neoplasms / Q000453:epidemiology* / Q000473:pathology; D011379:Prognosis; D012042:Registries; D018426:SEER Program; D015996:Survival Rate; D014481:United States / Q000453:epidemiology; D003969:Vipoma / Q000453:epidemiology / Q000473:pathology