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Olfactory neuroblastoma is a peripheral primitive neuroectodermal tumor related to Ewing sarcoma.
Proc Natl Acad Sci U S A. 1996 Feb 06; 93(3):1038-43.PN

Abstract

Olfactory neuroblastoma (ONB) is a malignant tumor of the nasal mucosa whose histogenesis is unclear. A relationship to neuroblastoma (NB), a pediatric tumor of the sympathetic nervous system, is based on morphologic similarities and the expression of similar neural antigens. However, the clinical presentation of ONB differs from that of NB, and MYCN amplification characteristic of NB is not observed. We have therefore examined the relationship of this malignancy to other classes of neural tumors. In previous studies, two ONB cell lines demonstrated cytogenetic features and patterns of protooncogene expression suggestive of a relationship to the Ewing sarcoma family of childhood peripheral primitive neuroectodermal tumors (pPNETs). The pPNETs show t(11;22)(q24;q12) or t(21;22)(q22;q12) chromosomal translocations fusing the EWS gene from 22q12 with either the FL11 gene on 11q24 or the ERG gene on 21q22. We therefore analyzed ONBs for the presence of pPNET-associated gene fusions. Both cell lines showed rearrangement of the EWS gene, and fluorescence in situ hybridization (FISH) of each case demonstrated fusion of EWS and FL11 genomic sequences. Moreover, both lines expressed EWS/FL11 fusion transcripts with in-frame junctions between exon 7 of EWS and exon 6 of FL11 as described for pPNETs. We identified similar gene fusions in four of six primary ONB cases. None of the cases expressed tyrosine hydroxylase, a catecholamine biosynthetic enzyme widely expressed in NB. Our studies indicate that ONB is not a NB but is a member of the pPNET family.

Authors+Show Affiliations

Department of Pathology, British Columbia's Children's Hospital, Vancouver, Canada.No affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info available

Pub Type(s)

Journal Article
Research Support, Non-U.S. Gov't

Language

eng

PubMed ID

8577710

Citation

Sorensen, P H., et al. "Olfactory Neuroblastoma Is a Peripheral Primitive Neuroectodermal Tumor Related to Ewing Sarcoma." Proceedings of the National Academy of Sciences of the United States of America, vol. 93, no. 3, 1996, pp. 1038-43.
Sorensen PH, Wu JK, Berean KW, et al. Olfactory neuroblastoma is a peripheral primitive neuroectodermal tumor related to Ewing sarcoma. Proc Natl Acad Sci USA. 1996;93(3):1038-43.
Sorensen, P. H., Wu, J. K., Berean, K. W., Lim, J. F., Donn, W., Frierson, H. F., Reynolds, C. P., López-Terrada, D., & Triche, T. J. (1996). Olfactory neuroblastoma is a peripheral primitive neuroectodermal tumor related to Ewing sarcoma. Proceedings of the National Academy of Sciences of the United States of America, 93(3), 1038-43.
Sorensen PH, et al. Olfactory Neuroblastoma Is a Peripheral Primitive Neuroectodermal Tumor Related to Ewing Sarcoma. Proc Natl Acad Sci USA. 1996 Feb 6;93(3):1038-43. PubMed PMID: 8577710.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - Olfactory neuroblastoma is a peripheral primitive neuroectodermal tumor related to Ewing sarcoma. AU - Sorensen,P H, AU - Wu,J K, AU - Berean,K W, AU - Lim,J F, AU - Donn,W, AU - Frierson,H F, AU - Reynolds,C P, AU - López-Terrada,D, AU - Triche,T J, PY - 1996/2/6/pubmed PY - 1996/2/6/medline PY - 1996/2/6/entrez SP - 1038 EP - 43 JF - Proceedings of the National Academy of Sciences of the United States of America JO - Proc. Natl. Acad. Sci. U.S.A. VL - 93 IS - 3 N2 - Olfactory neuroblastoma (ONB) is a malignant tumor of the nasal mucosa whose histogenesis is unclear. A relationship to neuroblastoma (NB), a pediatric tumor of the sympathetic nervous system, is based on morphologic similarities and the expression of similar neural antigens. However, the clinical presentation of ONB differs from that of NB, and MYCN amplification characteristic of NB is not observed. We have therefore examined the relationship of this malignancy to other classes of neural tumors. In previous studies, two ONB cell lines demonstrated cytogenetic features and patterns of protooncogene expression suggestive of a relationship to the Ewing sarcoma family of childhood peripheral primitive neuroectodermal tumors (pPNETs). The pPNETs show t(11;22)(q24;q12) or t(21;22)(q22;q12) chromosomal translocations fusing the EWS gene from 22q12 with either the FL11 gene on 11q24 or the ERG gene on 21q22. We therefore analyzed ONBs for the presence of pPNET-associated gene fusions. Both cell lines showed rearrangement of the EWS gene, and fluorescence in situ hybridization (FISH) of each case demonstrated fusion of EWS and FL11 genomic sequences. Moreover, both lines expressed EWS/FL11 fusion transcripts with in-frame junctions between exon 7 of EWS and exon 6 of FL11 as described for pPNETs. We identified similar gene fusions in four of six primary ONB cases. None of the cases expressed tyrosine hydroxylase, a catecholamine biosynthetic enzyme widely expressed in NB. Our studies indicate that ONB is not a NB but is a member of the pPNET family. SN - 0027-8424 UR - https://www.unboundmedicine.com/medline/citation/8577710/Olfactory_neuroblastoma_is_a_peripheral_primitive_neuroectodermal_tumor_related_to_Ewing_sarcoma_ L2 - http://www.pnas.org/cgi/pmidlookup?view=long&pmid=8577710 DB - PRIME DP - Unbound Medicine ER -