Pediatric sarcomas: Translating molecular pathogenesis of disease to novel therapeutic possibilities Journal Article
| Authors: | Anderson, J. L.; Denny, C. T.; Tap, W. D.; Federman, N. |
| Article Title: | Pediatric sarcomas: Translating molecular pathogenesis of disease to novel therapeutic possibilities |
| Abstract: | Pediatric sarcomas represent a diverse group of rare bone and soft tissue malignancies. Although the molecular mechanisms that propel the development of these cancers are not well understood, identification of tumor-specific translocations in many sarcomas has provided significant insight into their tumorigenesis. Each fusion protein resulting from these chromosomal translocations is thought to act as a driving force in the tumor, either as an aberrant transcription factor (TF), constitutively active growth factor, or ligand-independent receptor tyrosine kinase. Identification of transcriptional targets or signaling pathways modulated by these oncogenic fusions has led to the discovery of potential therapeutic targets. Some of these targets have shown considerable promise in preclinical models and are currently being tested in clinical trials. This review summarizes the molecular pathology of a subset of pediatric sarcomas with tumor-associated translocations and how increased understanding at the molecular level is being translated to novel therapeutic advances. © 2012 International Pediatric Research Foundation, Inc. |
| Keywords: | osteosarcoma; signal transduction; child; unclassified drug; gene translocation; review; sorafenib; bevacizumab; sunitinib; advanced cancer; gene targeting; imatinib; metastasis; models, biological; transcription initiation; dasatinib; angiosarcoma; cetuximab; childhood cancer; carcinogenesis; cixutumumab; r 1507; temsirolimus; ewing sarcoma; sarcoma; drug delivery systems; fibrosarcoma; gene fusion; oncogene proteins, fusion; cediranib; pazopanib; soft tissue sarcoma; translocation, genetic; lung alveolus cell carcinoma; hemangiopericytoma; leiomyosarcoma; rhabdomyosarcoma; synovial sarcoma; axitinib; malignant peripheral nerve sheath tumor; extraskeletal myxoid chondrosarcoma; chondrosarcoma; everolimus; oblimersen; epithelioid sarcoma; liposarcoma; clear cell sarcoma; valproic acid; sb 939; alveolar soft part sarcoma; desmoplastic small round cell tumor; myxosarcoma; dermatofibrosarcoma protuberans; transcription termination; figitumumab; plasma cell granuloma; gastrointestinal tumor; endometrium sarcoma; ganitumab; crizotinib; olaratumab; angiomatoid fibrous histiocytoma; congenital fibrosarcoma; pediatric sarcoma; nonrhabdomyosarcoma soft tissue sarcoma; retroperitoneal sarcoma; abexinostat; alisertib; conatumumab; imc c 225; teprotumumab; tivantinib; es like tumor; giant cell fibrosarcoma; low grade fibromyxoid; mesoblastic nephroma; osteoclastoma |
| Journal Title: | Pediatric Research |
| Volume: | 72 |
| Issue: | 2 |
| ISSN: | 0031-3998 |
| Publisher: | Int Pediatric Research Foundation, Inc |
| Date Published: | 2012-04-30 |
| Start Page: | 112 |
| End Page: | 121 |
| Language: | English |
| DOI: | 10.1038/pr.2012.54 |
| PROVIDER: | scopus |
| PUBMED: | 22546864 |
| PMCID: | PMC4283808 |
| DOI/URL: | |
| Notes: | --- - "Export Date: 4 September 2012" - "CODEN: PEREB" - "Source: Scopus" |
