Desmoplastic small round cell tumor 20 years after its discovery Journal Article
| Authors: | Mora, J.; Modak, S.; Cheung, N. K.; Meyers, P.; de Alava, E.; Kushner, B.; Magnan, H.; Tirado, O. M.; Laquaglia, M.; Ladanyi, M.; Rosai, J. |
| Article Title: | Desmoplastic small round cell tumor 20 years after its discovery |
| Abstract: | Desmoplastic small round cell tumor (DSRCT) was proposed as a distinct disease entity by William L Gerald and Juan Rosai in 1991. Over 850 patients have been reported in the medical literature. A specific translocation, t(11;22)(p13;q12), is seen in almost all cases, juxtaposing the EWS gene to the WT1 tumor suppressor gene. DSRCT is composed of nests of small round cells with polyphenotypic differentiation, typically a mixture of epithelial, mesenchymal and neural features, surrounded by a prominent desmoplastic stroma. DSRCT has a predilection for adolescent and young adult males, and primarily involves the abdominal cavity and pelvis. Survival is low despite their initial response to multimodal treatment. Most patients relapse with disseminated disease that is unresponsive to further therapy. © 2015 Future Medicine Ltd. |
| Keywords: | protein kinase b; cancer survival; event free survival; treatment response; overall survival; review; intensity modulated radiation therapy; bevacizumab; cisplatin; sunitinib; cancer combination chemotherapy; multimodality cancer therapy; nonhuman; gemcitabine; paclitaxel; temozolomide; imatinib; quality of life; apoptosis; gene expression; cytogenetics; cyclophosphamide; protein bcl xl; angiogenesis; phosphatidylinositol 3 kinase; tumor antigen; sex ratio; thiotepa; docetaxel; irinotecan; somatomedin c receptor; temsirolimus; ewing sarcoma; mammalian target of rapamycin; pazopanib; chromosome translocation; sex difference; upregulation; wt1 protein; hyperthermic therapy; trabectedin; navelbine; dna binding; radioimmunotherapy; rna binding protein ews; rapamycin; somatomedin b; diploidy; history of medicine; desmoplastic small round cell tumor; protein bax; phase 2 clinical trial (topic); phase 1 clinical trial (topic); tumor invasion; ganitumab; dna content; ews gene; leflunomide; wt1 gene; human; priority journal; adolescent and young adult oncology; desmoplastic tumors; developmental cancer; small round cell tumors; william l gerald |
| Journal Title: | Future Oncology |
| Volume: | 11 |
| Issue: | 7 |
| ISSN: | 1479-6694 |
| Publisher: | Future Medicine |
| Date Published: | 2015-01-01 |
| Start Page: | 1071 |
| End Page: | 1081 |
| Language: | English |
| DOI: | 10.2217/fon.15.32 |
| PROVIDER: | scopus |
| PUBMED: | 25804122 |
| DOI/URL: | |
| Notes: | Export Date: 4 May 2015 -- Source: Scopus |
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