one were to include the soft tissue variants, PNET, and older patients, the incidence would be slightly amplified. Horowitz and colleagues compiled data from 17 studies, performed over 10 years, which included Ewing's sarcoma, peripheral PNET and extraosseous Ewing's sarcoma, and found that among 1505 reported patients 87% had Ewing's sarcoma of bone, 8% extraosseous Ewing's sarcoma, and 5% peripheral PNET [6]. Among these patients, more than half the tumors occurred in the second decade of life, close to one third in the first decade, and less than 10% in the third decade. These data are very similar to those from the Children's Cancer Group (CCG)-Pediatric Oncology Group (POG) intergroup study (1988)(1989)(1990)(1991)(1992) of Ewing's sarcoma of bone and PNET, in which 30% of patients were younger than 10 years, 57% were age 10-17, and 13% were between 18 and 30 years of age [7,8]. Less commonly, ESFT occurs in patients over 30 years of age [9][10][11]. The spectrum of age of patients with peripheral neuroepitheliomas is said to include an increased number of younger patients. Ewing's sarcoma is rare among nonwhite populations [12][13][14][15]. In New York state, 22% of primary bone malignancies among white patients are Ewing's sarcoma, but only 7% of primary bone tumors diagnosed in African Americans are Ewing's sarcoma [12].Clear predisposing factors to Ewing tumors have not been identified. A recent report [16] from Canada linked an increased risk of death from bone cancer (osteosarcoma, Ewing's sarcoma, and chondrosarcoma) to elevated levels of radium in the drinking water of recorded birthplace, but this finding has not been confirmed in other reports. In an analysis from England, low birth weight was associated with Ewing's sarcoma [17]. A report for the National Cancer Institute [18] demonstrated an increased risk of stomach and neuroectodermal cancers in family members of Ewing's sarcoma patients; however, a similar (albeit smaller) study of patients ascertained from the Manchester bone tumor registry [19] found no excess of cancer in relatives. The risk of cancer in mothers of children with Ewing's sarcoma does not appear to be increased [20]. There have been reports of Ewing's sarcoma in siblings [21,22]; however, the Ewing family of tumors is not associated with the Li-Fraumeni familial cancer syndrome [23]. Although osteosarcoma following chemotherapy and radiation treatment for Ewing's sarcoma is well described, Ewing's sarcoma as a second malignancy is rare [24][25][26][27]. There is no convincing evidence that Ewing's sarcoma is associated with skeletal abnormalities, genitourinary abnormalities, or other congenital abnormalities, although case reports and associations have been reported.
Pathology