The following are examples of recent translational studies which we have either initiated or played a major collaborative role and which have been completed and are at the stage of preparation of a manuscript : - We studied nestin expression in benign and malignant nerve sheath tumors from patients with neurofibromatosis 1 and found its correlation with a malignant phenotype. We also found increased expression of nestin in histologically atypical areas of otherwise benign tumors that correlate with increased PET uptake, suggesting that it may serve as a marker of tumor progression. - We evaluated expression of the ganglioside GD2 in large number of solid pediatric tumors and found high expression in osteosarcoma and Ewing sarcoma. Our data will help design a clinical trial targeting GD2 in these tumors. - We evaluated NY-ESO expression pediatric synovial sarcomas and other solid tumors in this age group (manuscript in preparation), which will lead to a new clinical trial at the NCI. - We found that c-kit negative gastrointestinal stromal tumors (GIST) with mutation in the succinate dehydrogenase (SDH)B gene, which lack expression of SDHB, show high expression of EGFR, but lack EGFR gene amplification or mutation. These data suggest that c-kit negative (pediatric) GIST may be yet another tumor example of translational upregulation of EGFR by tumor hypoxia.- We collaborate on a study that seeks to identify molecular therapeutic targets in pediatric pontine gliomas, by performing immunohistochemical staining and evaluation of a panel of proteins

National Institute of Health (NIH)
National Cancer Institute (NCI)
Scientific Cores Intramural Research (ZIC)
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National Cancer Institute Division of Clinical Sciences
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Tsokos, Maria; Alaggio, Rita D; Dehner, Louis P et al. (2012) Ewing sarcoma/peripheral primitive neuroectodermal tumor and related tumors. Pediatr Dev Pathol 15:108-26
Meadors, Joanna L; Cui, Yonghzi; Chen, Qing-Rong et al. (2011) Murine rhabdomyosarcoma is immunogenic and responsive to T-cell-based immunotherapy. Pediatr Blood Cancer 57:921-9
Maxhimer, Justin B; Soto-Pantoja, David R; Ridnour, Lisa A et al. (2009) Radioprotection in normal tissue and delayed tumor growth by blockade of CD47 signaling. Sci Transl Med 1:3ra7
Fitzhugh, Courtney D; Wise, Barbara; Baird, Kristin et al. (2009) Secondary supratentorial primitive neuroectodermal tumor following treatment of childhood osteosarcoma. Pediatr Blood Cancer 53:496-8
Kim, Su Young; Tsokos, Maria; Helman, Lee J (2008) Dilemmas associated with congenital ewing sarcoma family tumors. J Pediatr Hematol Oncol 30:4-7
Mackall, Crystal L; Rhee, Eunice H; Read, Elizabeth J et al. (2008) A pilot study of consolidative immunotherapy in patients with high-risk pediatric sarcomas. Clin Cancer Res 14:4850-8