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Pax3-fkhr and pax7-fkhr fusion genes impact outcome of alveolar rhabdomyosarcoma in children

Kazanowska, Bernarda; Reich, Adam; Stegmaier, Sabine; Békássy, Albert LU ; Leuschner, Ivo; Chybicka, Alicja and Koscielniak, Ewa (2007) In Fetal and Pediatric Pathology 26(1). p.17-31
Abstract
Rhabdomyosarcoma is a highly malignant embryonic tumor of childhood. Two specific translocations t(2;13)(q35;q14) and t(1;13)(p36;q14) have been identified in about 75-80% of ARMS cells. The aim of this multicenter study was to analyze the relationships between the identified fusion transcripts and survival including some selected clinical parameters. The extent of disease was graded according to clinical staging system with following distribution: 3 children with stage I, 4 with stage II, 23 with stage III, and 18 with stage IV spread disease having distant metastases. PAX3-FKHR fusion genes were detected in 28 and PAX7-FKHR fusion genes in 7 tumor biopsy specimens. Children with PAX3-FKHR fusion gene had often distant metastases at... (More)
Rhabdomyosarcoma is a highly malignant embryonic tumor of childhood. Two specific translocations t(2;13)(q35;q14) and t(1;13)(p36;q14) have been identified in about 75-80% of ARMS cells. The aim of this multicenter study was to analyze the relationships between the identified fusion transcripts and survival including some selected clinical parameters. The extent of disease was graded according to clinical staging system with following distribution: 3 children with stage I, 4 with stage II, 23 with stage III, and 18 with stage IV spread disease having distant metastases. PAX3-FKHR fusion genes were detected in 28 and PAX7-FKHR fusion genes in 7 tumor biopsy specimens. Children with PAX3-FKHR fusion gene had often distant metastases at presentation (p = 0.03). PAX3-FKHR positive patients with locoregional disease had significantly poorer outcome compared with the ones with PAX7-FKHR positive tumors (p = 0.04). Although analyzed groups were small, significant differences in survival and clinical characteristics between PAX3-FKHR and PAX7-FKHR positive tumors were stated indicating their role in carcinogenesis. In addition, fusion gene analysis is a helpful tool in differential diagnosis of poorly differentiated soft tissue tumors. (Less)
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author
organization
publishing date
type
Contribution to journal
publication status
published
subject
keywords
prognosis, clinical characteristics, mutagenesis, survival, soft tissue malignant tumors
in
Fetal and Pediatric Pathology
volume
26
issue
1
pages
17 - 31
publisher
Taylor & Francis
external identifiers
  • pmid:17613043
  • wos:000247724800002
  • scopus:34447118189
ISSN
1551-3823
DOI
10.1080/15513810701394702
language
English
LU publication?
yes
id
784d25b2-a223-4da0-997a-378cb5f48765 (old id 1138472)
date added to LUP
2008-08-19 11:47:20
date last changed
2017-07-30 03:36:00
@article{784d25b2-a223-4da0-997a-378cb5f48765,
  abstract     = {Rhabdomyosarcoma is a highly malignant embryonic tumor of childhood. Two specific translocations t(2;13)(q35;q14) and t(1;13)(p36;q14) have been identified in about 75-80% of ARMS cells. The aim of this multicenter study was to analyze the relationships between the identified fusion transcripts and survival including some selected clinical parameters. The extent of disease was graded according to clinical staging system with following distribution: 3 children with stage I, 4 with stage II, 23 with stage III, and 18 with stage IV spread disease having distant metastases. PAX3-FKHR fusion genes were detected in 28 and PAX7-FKHR fusion genes in 7 tumor biopsy specimens. Children with PAX3-FKHR fusion gene had often distant metastases at presentation (p = 0.03). PAX3-FKHR positive patients with locoregional disease had significantly poorer outcome compared with the ones with PAX7-FKHR positive tumors (p = 0.04). Although analyzed groups were small, significant differences in survival and clinical characteristics between PAX3-FKHR and PAX7-FKHR positive tumors were stated indicating their role in carcinogenesis. In addition, fusion gene analysis is a helpful tool in differential diagnosis of poorly differentiated soft tissue tumors.},
  author       = {Kazanowska, Bernarda and Reich, Adam and Stegmaier, Sabine and Békássy, Albert and Leuschner, Ivo and Chybicka, Alicja and Koscielniak, Ewa},
  issn         = {1551-3823},
  keyword      = {prognosis,clinical characteristics,mutagenesis,survival,soft tissue malignant tumors},
  language     = {eng},
  number       = {1},
  pages        = {17--31},
  publisher    = {Taylor & Francis},
  series       = {Fetal and Pediatric Pathology},
  title        = {Pax3-fkhr and pax7-fkhr fusion genes impact outcome of alveolar rhabdomyosarcoma in children},
  url          = {http://dx.doi.org/10.1080/15513810701394702},
  volume       = {26},
  year         = {2007},
}