Incidence and Outcomes of Desmoplastic Small Round Cell Tumor: Results from the Surveillance, Epidemiology, and End Results Database

Christina K. Lettieri, Pamela Garcia-Filion, Pooja Hingorani

Research output: Contribution to journalArticle

24 Citations (Scopus)

Abstract

Desmoplastic small round cell tumor (DSRCT) is a rare but highly fatal malignancy. Due to the rarity of this neoplasm, no large population based studies exist. Procedure. This is a retrospective cohort analysis. Incidence rates were calculated based on sex and ethnicity and compared statistically. Gender-, ethnicity-, and treatment-based survival were calculated using the Kaplan-Meier method. Results. A total of 192 cases of DSRCT were identified. Peak incidence age was between 20 and 24 years. Age-adjusted incidence rate for blacks was 0.5 cases/million and for whites was 0.2 cases/million (P = 0.037). There was no statistically significant difference in survival based on gender or ethnicity. When adjusted for age, there was no statistically significant difference in survival amongst patients who received radiation therapy compared to those who did not (HRadj = 0.73; 95% CI 0.49, 1.11). There was a statistically significant survival advantage for patients who received radiation after surgery compared to those who did not (HR 0.49; 95% CI 0.30, 0.79). Conclusion. DSRCT is more common in males and in people of African-American descent. Although overall survival remains poor, radiation therapy following surgery seems to improve outcome in these patients.

Original languageEnglish (US)
Article number680126
JournalJournal of Cancer Epidemiology
Volume2014
DOIs
StatePublished - Jan 1 2014

Fingerprint

Desmoplastic Small Round Cell Tumor
Epidemiology
Databases
Survival
Incidence
Radiotherapy
African Americans
Neoplasms
Cohort Studies
Radiation
Population

ASJC Scopus subject areas

  • Epidemiology
  • Genetics
  • Public Health, Environmental and Occupational Health

Cite this

Incidence and Outcomes of Desmoplastic Small Round Cell Tumor : Results from the Surveillance, Epidemiology, and End Results Database. / Lettieri, Christina K.; Garcia-Filion, Pamela; Hingorani, Pooja.

In: Journal of Cancer Epidemiology, Vol. 2014, 680126, 01.01.2014.

Research output: Contribution to journalArticle

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