+ Site Statistics
+ Search Articles
+ PDF Full Text Service
How our service works
Request PDF Full Text
+ Follow Us
Follow on Facebook
Follow on Twitter
Follow on LinkedIn
+ Subscribe to Site Feeds
Most Shared
PDF Full Text
+ Translate
+ Recently Requested

Olfactory neuroblastoma is a peripheral primitive neuroectodermal tumor related to Ewing sarcoma



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): 1038-1043



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 FLI1 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 FLI1 genomic sequences. Moreover, both lines expressed EWS/FLI1 fusion transcripts with in-frame junctions between exon 7 of EWS and exon 6 of FLI1 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.

Please choose payment method:






(PDF emailed within 0-6 h: $19.90)

Accession: 009123419

Download citation: RISBibTeXText

PMID: 8577710

DOI: 10.2307/38743


Related references

Ewing sarcoma/peripheral primitive neuroectodermal tumor and related tumors. Pediatric and Developmental Pathology 15(1 Suppl): 108-126, 2012

The Ewing's sarcoma family of tumors: Ewing's sarcoma and peripheral primitive neuroectodermal tumor of bone and soft tissue. Cancer Treatment and Research 92: 253-308, 1997

Ewing sarcoma/peripheral primitive neuroectodermal tumor: adult abdominal tumors with an Ewing sarcoma gene rearrangement demonstrated by fluorescence in situ hybridization in paraffin sections. Applied Immunohistochemistry and Molecular Morphology 12(2): 160-165, 2004

Protocol for the examination of specimens from pediatric and adult patients with osseous and extraosseous ewing sarcoma family of tumors, including peripheral primitive neuroectodermal tumor and ewing sarcoma. Archives of Pathology and Laboratory Medicine 129(7): 866-873, 2005

Mic2 is a specific marker for ewing's sarcoma and peripheral primitive neuroectodermal tumors evidence for a common histogenesis of ewing's sarcoma and peripheral primitive neuroectodermal tumors from mic2 expression and specific chromosome aberration. Cancer 67(7): 1886-1893, 1991

Ewing's sarcoma and peripheral primitive neuroectodermal tumor. Current Opinion in Oncology 6(4): 391-396, 1994

Primary Ewing sarcoma/peripheral primitive neuroectodermal tumor of the vulva. Human Pathology 39(10): 1535-1539, 2008

Ewing's sarcoma and peripheral primitive neuroectodermal tumor: an interim report. Annals of Otology, Rhinology, and Laryngology 105(10): 838-843, 1996

Ewing's sarcoma and primitive neuroectodermal tumor in adults: are they different from Ewing's sarcoma and primitive neuroectodermal tumor in children?. Journal of Clinical Oncology 15(7): 2611-2621, 1997

Olfactory neuroblastoma is not a neuroblastoma but is related to primitive neuroectodermal tumor (PNET). Progress in Clinical and Biological Research 271: 463-473, 1988

Endometrial endometrioid carcinomas associated with Ewing sarcoma/peripheral primitive neuroectodermal tumor. International Journal of Gynecological Pathology 19(2): 127-132, 2000

Detection of circulating tumor cells in patients with Ewing's sarcoma and peripheral primitive neuroectodermal tumor. Journal of Clinical Oncology 15(2): 583-588, 1997

Extensive GI tract involvement of Ewing's sarcoma/peripheral primitive neuroectodermal tumor in an octogenarian. Gastrointestinal Endoscopy 67(7): 1196-1198, 2008