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. 2014:2014:680126.
doi: 10.1155/2014/680126. Epub 2014 Nov 5.

Incidence and outcomes of desmoplastic small round cell tumor: results from the surveillance, epidemiology, and end results database

Christina K Lettieri  1 , Pamela Garcia-Filion  2 , Pooja Hingorani  2
Affiliations

Incidence and outcomes of desmoplastic small round cell tumor: results from the surveillance, epidemiology, and end results database

Christina K Lettieri et al. J Cancer Epidemiol. 2014.

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.

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Figures

Figure 1
Figure 1
(a) Overall age-adjusted incidence of DSRCT. (b) Sex-based age-adjusted incidence of DSRCT. Males are more likely than females to get DSRCT (P < 0.001). (c) Race-based, age-adjusted incidence of DSRCT. Blacks are more likely than whites to get DSRCT (P = 0.037).
Figure 2
Figure 2
(a) Race-based survival of DSRCT. There may be a survival disadvantage for blacks compared to whites. Although it did not reach statistical significance, this analysis suggests that blacks are 33% more likely to succumb to DSRCT than are whites (P = 0.2). (b) Treatment-based survival of DSRCT, radiation versus no radiation. There was no statistically significant difference in survival amongst patients who received radiation therapy compared to those who did not. (c) Treatment-based survival of DSRCT, radiation after surgery versus no radiation. Patients who received radiation following surgery fared better than those patients who did not (P < 0.05).
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