Racial Differences in the Incidence of Mesenchymal Tumors Associated with EWSR1 Translocation

Abstract

Background: The incidence of Ewing sarcoma varies by race, with very low rates among persons of African and East Asian ancestry. The incidence by race of other mesenchymal tumors that also harbor EWSR1 translocations has not been studied.Methods: The SEER database was queried to find cases of mesenchymal tumors associated with EWSR1 translocations: Ewing sarcoma; clear cell sarcoma; extraskeletal myxoid chondrosarcoma; myxoid liposarcoma; desmoplastic small round cell tumor; and myoepithelial tumor. Age-adjusted incidence rates were calculated for white, African American, and Asian/Native American populations and compared statistically.Results: Ewing sarcoma was significantly less common in the African American and Asian/Native American populations compared with the white population, with incidence rate ratios of 0.12 (95% CI, 0.08-0.20; P < 0.001) and 0.54 (95% CI, 0.41-0.69; P < 0.001), respectively. Desmoplastic small round cell tumor was significantly more common in the African American population compared with the white population (incidence rate ratio ¼ 3.0; 95% CI, 1.62-5.49; P < 0.001). Myxoid liposarcoma was significantly less common in the Asian/Native American population compared with the white population (incidence rate ratio ¼ 0.72; 95% CI, 0.56-0.92; P ¼ 0.006). The incidence rates for extraskeletal myxoid chondrosarcoma, myoepithelial tumors, and clear cell sarcoma did not differ significantly by race.Conclusions: Tumors associated with EWSR1 translocation are not uniformly more common in people of European ancestry.Impact: The relationship between race and EWSR1 somatic translocation is complex. Future studies investigating the genetic epidemiology of EWSR1 translocated tumors are required. Cancer Epidemiol Biomarkers Prev; 20(3); 449-53. Ó2011 AACR.

Desmoplastic Small Round Cell Tumor of the Pleura Successfully Treated with a Lower Dose of Pazopanib

“…DSRCT is a rare malignant tumor with an annual incident rate of 0.1/1,000,000 (9). The pleura is a rare site of presentation (2); thus far, less than ten cases have been reported in the English literature (10-13).…”

Predictors of acute chemotherapy‐associated toxicity in patients with Ewing sarcoma

“…Ethnicity was classified as either Latino or non-Latino based on ethnicity noted in the patient record, typically based on patient reported ethnicity. Patient race was also collected though not analyzed further due to small numbers of non-white patients, consistent with the known epidemiology of this disease [23]. Data for a proxy for low family income (eligibility for California’s state program for low income patients ≤ 21 years of age) were also collected for all patients under 21 years of age at diagnosis.…”

CD99 polymorphisms significantly influence the probability to develop Ewing sarcoma in earlier age and patient disease progression

“…EWS arises abruptly in pediatric age, with the peak of incidence in the second decade of life, and its natural history is still mostly unknown. The tumor is more common in Caucasians while it rarely appears in individuals of African or Asian heritage [2–6]. This observation together with reports indicating EWS in siblings or cousins [7, 8], suggests that genetic susceptibility factors may exist for this tumor, particularly among European population.…”