Abstract

A consistent t(2;13) (q35;q14) translocation in alveolar RMS results in fusion between PAX3 or PAX7 genes with the FKHR factors. Using reverse transcription (RT)-PCR and fluorescence in situ hybridization (FISH) technique, we will investigate the presence of PAX3 (PAX7)/FKHR transcripts in a large number of alveolar RMS. Embryonal RMS on the other hand exhibits a reproducible loss of chromosomal material on chromosome 11p15.5. We have used oligonucleotide repeats at the tyrosine hydroxylase (TH) locus and investigated loss of heterozygosity (LOH) in 11 RMS and 13 non-RMS sarcomas in children and young adults. We found consistent LOH at the TH locus in all but one (botryoid) RMS. However, LOH was also detected in 2 nerve sheath tumors and one fibrosarcoma. We would like to extend our studies to include a larger sample of RMS and non-RMS tumors. Our preliminary findings however suggest, that although the RT-PCR detected fusion transcripts are specific for alveolar RMS, LOH at the 11p15.5 locus is present not only in embryonal RMS, but other tumors as well.

  Funding Agency

Agency
National Institute of Health (NIH)
Institute
National Cancer Institute (NCI)
Type
Intramural Research (Z01)
Project #
1Z01SC009431-02
Application #
2464541
Study Section
Special Emphasis Panel (LP)
Project Start
Project End
Budget Start
Budget End
Support Year
2
Fiscal Year
1996
Total Cost
Indirect Cost

  Institution

Name
National Cancer Institute Division of Clinical Sciences
Department
Type
DUNS #
City
State
Country
United States
Zip Code

  Publications

Cortes, Lizette M; Mattapallil, Mary J; Silver, Phyllis B et al. (2008) Repertoire analysis and new pathogenic epitopes of IRBP in C57BL/6 (H-2b) and B10.RIII (H-2r) mice. Invest Ophthalmol Vis Sci 49:1946-56
Ahmed, Atif Ali; Tsokos, Maria (2007) Sinonasal rhabdomyosarcoma in children and young adults. Int J Surg Pathol 15:160-5
Petricoin 3rd, Emanuel F; Espina, Virginia; Araujo, Robyn P et al. (2007) Phosphoprotein pathway mapping: Akt/mammalian target of rapamycin activation is negatively associated with childhood rhabdomyosarcoma survival. Cancer Res 67:3431-40
Schinstine, Malcolm; Abati, Andrea; Tsokos, Maria et al. (2006) Cytological identification of metastatic epithelial nephroblastoma in pleural fluid: report of a case and review of literature. Diagn Cytopathol 34:621-5
Nathan, Paul C; Tsokos, Maria; Long, Lauren et al. (2005) Adjuvant chemotherapy for the treatment of advanced pediatric nonrhabdomyosarcoma soft tissue sarcoma: the National Cancer Institute experience. Pediatr Blood Cancer 44:449-54
Barenboim-Stapleton, Linda; Yang, Xuezhong; Tsokos, Maria et al. (2005) Pediatric pancreatoblastoma: histopathologic and cytogenetic characterization of tumor and derived cell line. Cancer Genet Cytogenet 157:109-17
Uren, A; Merchant, M S; Sun, C J et al. (2003) Beta-platelet-derived growth factor receptor mediates motility and growth of Ewing's sarcoma cells. Oncogene 22:2334-42
Debelenko, Larisa V; Arthur, Diane C; Pack, Svetlana D et al. (2003) Identification of CARS-ALK fusion in primary and metastatic lesions of an inflammatory myofibroblastic tumor. Lab Invest 83:1255-65
Gorlick, Richard; Anderson, Peter; Andrulis, Irene et al. (2003) Biology of childhood osteogenic sarcoma and potential targets for therapeutic development: meeting summary. Clin Cancer Res 9:5442-53
Wexler, L H; Meyer, W H; Parham, D M et al. (2000) Neural differentiation and prognosis in peripheral primitive neuroectodermal tumor. J Clin Oncol 18:2187-8

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