Accurate diagnosis of solid pediatric tumors requires a combination of diagnostic techniques including reverse transcription polymerase chain reaction (RT-PCR). Many pediatric solid tumors exhibit fundamental cytogenetic abnormalities that have implications in their pathogenesis. The Ewing's sarcoma family of tumors (ESFT) and alveolar rhabdomyosarcoma (RMS) are characterized by consistent chromosomal translocations which result in the fusion of genes and subsequent formation of novel chimeric genes. These molecular markers can be detected by RT-PCR or fluorescence in situ hybridization (FISH) and can be used not only to establish the diagnosis in difficult cases, but also to understand the pathogenesis of these tumors. Recently, the products of these fusion genes have become the target of vaccine therapies in newly established protocols in the Pediatric Oncology Branch (POB) at the NCI. The objective of this project is: (1) to provide state of the art diagnosis on tissue specimens from pediatric tumor patients participating in POB clinical trials (2) to evaluate the molecular pathology of protocol-related pediatric tumors (presence or absence of specific fusion transcripts) (3) to evaluate the significance of molecular markers in the diagnosis, classification and pathogenesis of pediatric sarcomas. The following accomplishments have been made in the last year: (1) A total of 120 pediatric tumor pathology reports were issued. (2) A total of 35 pediatric tumor molecular pathology reports were issued. (3) Six pediatric tumor pathology studies were published.

Agency
National Institute of Health (NIH)
Institute
Division of Clinical Sciences - NCI (NCI)
Type
Intramural Research (Z01)
Project #
1Z01SC009431-08
Application #
6756956
Study Section
(LP)
Project Start
Project End
Budget Start
Budget End
Support Year
8
Fiscal Year
2002
Total Cost
Indirect Cost
Name
Clinical Sciences
Department
Type
DUNS #
City
State
Country
United States
Zip Code
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
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
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
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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