The Ewing’s sarcoma family of tumors: Ewing’s sarcoma and peripheral primitive neuroectodermal tumor of bone and soft tissue

Abstract

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

Treatment Effects in Pediatric Soft Tissue and Bone Tumors

“…Histologic subtypes do not have major prognostic significance, although the filigree growth pattern has an association with a poorer prognosis. [34][35][36] Recent studies of patients with EWS/PNET treated with contemporary regimens have documented a correlation between histopathologic degree of posttherapy tumor necrosis and patient survival, 20,33,36-49 but a number of other studies did not reveal a correlation between histologic tumor necrosis after treatment and survival, which might have been related to specific previous treatment protocols [50][51][52][53][54][55][56] ❚Table 3❚. Correlations between the radiographic and the pathologic responses to chemotherapy using computed tomography, magnetic resonance imaging, and other techniques have yielded variable results.…”

Biphenotypic sarcoma with characteristics of both a Ewing sarcoma and a desmoplastic small round cell tumor

“…Indeed, as many as 85-90% of fresh pathological specimens could be demonstrated to possess this chromosomal rearrangement by tumor cytogenetics [25,26]. Soon thereafter, it was recognized that another small round cell tumor, PPNET, though thought to be clinically distinct, also could be shown to have the same t(11;22)(q24;q12) [3,5,22]. This genetic similarity, coupled with evidence that they share a common neural histogenesis, led to the widely held notion that they should be grouped together, thus generating the term "Ewing family of tumors" [22].…”

“…Soon thereafter, it was recognized that another small round cell tumor, PPNET, though thought to be clinically distinct, also could be shown to have the same t(11;22)(q24;q12) [3,5,22]. This genetic similarity, coupled with evidence that they share a common neural histogenesis, led to the widely held notion that they should be grouped together, thus generating the term "Ewing family of tumors" [22]. From these initial cytogenetic observations came the discovery that this translocation resulted in the production of a hybrid RNA containing the 5Ј half of EWS fused to the 3Ј half of FLI1 (EWS/FLI1).…”

“…The initial response to therapy is often quite good, but complete responses are uncommon and relapse is the rule [20,21]. EWS typically occurs in a wider age range of children and adolescents, and primary lesions can appear in both osseous and extraosseous sites, including the abdomen [3,22]. Metastatic EWS confined to the abdomen as carcinomatosis is extremely uncommon.…”

Ewing`S Sarcoma/PNET of the Labia Majora an Unusual Presentation in 2 Year Old Girl

“…To our knowledge, this is the first reported case of extra skeletal Ewing`s sarcoma confirmed with immunocytochemistry and genetic analysis in our country. Histological features include small round hyperchromatic and little mitotic activity [1][2][3][4][5][6][7][8]. Because these findings are nonspecific, immunohistochemistry is frequently.…”