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Visceral primitive peripheral neuroectodermal tumors: a clinicopathologic and molecular study.
Hum Pathol. 2001 Oct; 32(10):1109-15.HP

Abstract

Ewing sarcoma-primitive neuroectodermal tumor (EWS/PNET) belongs to the group of pediatric small round blue cell tumors; although EWS/PNET is classically a tumor of 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 breakpoint of 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 recognition that 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.

Authors+Show Affiliations

Lauren V. Ackerman Laboratory of Surgical Pathology, Washington University Medical Center, St Louis, MO 63110, USA.No affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info available

Pub Type(s)

Case Reports
Journal Article

Language

eng

PubMed ID

11679946

Citation

O'Sullivan, M J., et al. "Visceral Primitive Peripheral Neuroectodermal Tumors: a Clinicopathologic and Molecular Study." Human Pathology, vol. 32, no. 10, 2001, pp. 1109-15.
O'Sullivan MJ, Perlman EJ, Furman J, et al. Visceral primitive peripheral neuroectodermal tumors: a clinicopathologic and molecular study. Hum Pathol. 2001;32(10):1109-15.
O'Sullivan, M. J., Perlman, E. J., Furman, J., Humphrey, P. A., Dehner, L. P., & Pfeifer, J. D. (2001). Visceral primitive peripheral neuroectodermal tumors: a clinicopathologic and molecular study. Human Pathology, 32(10), 1109-15.
O'Sullivan MJ, et al. Visceral Primitive Peripheral Neuroectodermal Tumors: a Clinicopathologic and Molecular Study. Hum Pathol. 2001;32(10):1109-15. PubMed PMID: 11679946.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - Visceral primitive peripheral neuroectodermal tumors: a clinicopathologic and molecular study. AU - O'Sullivan,M J, AU - Perlman,E J, AU - Furman,J, AU - Humphrey,P A, AU - Dehner,L P, AU - Pfeifer,J D, PY - 2001/10/27/pubmed PY - 2002/1/5/medline PY - 2001/10/27/entrez SP - 1109 EP - 15 JF - Human pathology JO - Hum Pathol VL - 32 IS - 10 N2 - Ewing sarcoma-primitive neuroectodermal tumor (EWS/PNET) belongs to the group of pediatric small round blue cell tumors; although EWS/PNET is classically a tumor of 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 breakpoint of 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 recognition that 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. SN - 0046-8177 UR - https://www.unboundmedicine.com/medline/citation/11679946/Visceral_primitive_peripheral_neuroectodermal_tumors:_a_clinicopathologic_and_molecular_study_ DB - PRIME DP - Unbound Medicine ER -