preview

Sarcoma

Decent Essays

Ewing’s sarcoma (ES), a malignant osteolytic tumor, characterized as small round cell tumors was first documented by James Ewing in the year 1921 as diffuse endothelioma of bone. Rarely, it also has extra osseous manifestations which resembles intraosseous ES. This extra osseous forms of ES was first described by Tefft in 1969.(1) Ewing’s sarcoma (ES) family of tumors include: classical ES (osseous origin), atypical ES (extra osseous), Primitive neuroectodermal tumor (PNET) and Askin tumor[2] . All these tumors have common morphology, immunophenotypic features and cytogenetics, hence included in the same family of tumors. These tumors were believed to be derived from a primitive cell. The primitive cell can be either the neural crest or mesenchymal …show more content…

Rarely, many other genes from the family members of ETS have also been identified as the fusion partners of EWS (7). According to Lin et al., 95% of cases of ES occur from EWS/FLI1 fusion gene formation as a result of transformation. Due to the difference in the locations of the EWS and FLI1 genomic breakpoints, it resulted in the existence of alternative forms of the chimeric gene. There are two most common forms, type 1 and type 2 accounting to 60% and 25% of the cases respectively. Type 1 consists of the first 7 exons of EWS joined to exons 6-9 of FLI1 and type 2 includes FLI1 exon 5 also.( 8) The prognosis of type 1 fusion is significantly better prognosis than the other fusion types as observed by Lin et al. (1999). A less active chimeric transcription factor is encoded by the type 1 fusion gene explaining the heterogeneous forms of ES at the molecular level. Particular chromosomal translocations is strongly associated with the development of PNET. The products of the fusion genes resulted from the translocations are specific to the type of tumors. In ES/PNETs, karyotype of t[11;22][q24;q12], which results from the EWS-FLII gene infusion and t[21;22][q22;q12], which results from EWS-ERG gene infusion account for 85% and 10% respectively (9-11). In reports of ES/PNETs, there are eight cases which chromosome translocation are t[11;22][q24; q12] …show more content…

The classic ES appears like that of a primitive, undifferentiated neoplasm morphologically. Histologically, there are small round blue cells in the form of monotonous sheets with hyperchromatic nuclei and scant cytoplasm (8). All the 24 cases in this review satiate these histologic criteria (Table 1). The tumor consists of extensive necrotic areas but viable tumor is usually preserved around blood vessels. Some tumor cells can also invade blood vessels. Some features are absent typically like nuclear atypia, palisading, and formation of rosettes (where the tumor cells are arranged in a circle about a central fibrillary space, indicative of neural differentiation or pseudo rosettes. The markers like P30/32MlC2 and at least two kinds of neuronal markers are the immunohistochemical criteria for the diagnosis of PNET. (12) Monoclonal antibodies like CD99, O13, HBA71, 12E7, RFB1 are also tested although none of them are actually specific for PNETs. (12) The neural markers like Neuron Specific Enolase(NSE), Chromogranin A(CgA), Synaptophysin(Syn) are usually positive but markers such as desmin, actin, S-100, insulin, glucagons, somatostatin are rarely

Get Access