2113

ETS1

ETS-1|EWSR2;v-ets erythroblastosis virus E26 oncogene homolog 1 (avian);ETS1;v-ets erythroblastosis virus E26 oncogene homolog 1 (avian)

Avian erythroblastosis virus E26 (v-ets) oncogene homolog-1|ets protein|p54|protein C-ets-1|v-ets avian erythroblastosis virus E2 oncogene homolog 1

11q23.3

protein-coding

Ewing sarcoma-primitive neuroectodermal tumor (EWS/PNET) belongs to the group ofpediatric small round blue cell tumors; although EWS/PNET is classically a tumorof the soft tissue or bone in children and young adults, individual cases have been described in patients of ALL ages. A group of chromosomal translocations involving the EWS gene and a member of the Ets transcription factor family of genes has been detected in EWS/PNET, and heterogeneity in the precise breakpointof the translocation has been shown to generate a group of related fusion transcripts that may have prognostic significance. Within the last decade, the clinicopathologic spectrum of EWS/PNET has been markedly expanded by recognitionthat the tumor may also have a visceral origin. To determine whether visceral EWS/PNET has the same pattern of genetic alterations and range of fusion transcripts as EWS/PNET of bone and soft tissue, we performed reverse-transcription polymerase chain reaction-based testing of formalin-fixed,paraffin-embedded tissue from a series of visceral tumors for which the diagnosis of EWS/PNET was well established. Together with additional cases compiled from the literature, EWS-Fli1 (or a related fusion transcript) was present in 18 of 19 visceral EWS/PNET, with a distribution of transcript types not statistically different from EWS/PNET of soft tissue and bone (P >.05, chi(2) test). These results firmly establish the genetic relationship between EWS/PNET of visceral sites, soft tissue, and bone.#CI- Copyright 2001 by W.B. Saunders Company