1. Temsirolimus for metastatic desmoplastic small round cell tumor.
- Author
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Thijs AM, van der Graaf WT, and van Herpen CM
- Subjects
- Humans, Liver Neoplasms drug therapy, Liver Neoplasms secondary, Lung Neoplasms drug therapy, Lung Neoplasms secondary, Lymphatic Metastasis, Male, Sirolimus therapeutic use, Young Adult, Abdominal Neoplasms pathology, Antineoplastic Agents therapeutic use, Protein Kinase Inhibitors therapeutic use, Sirolimus analogs & derivatives, TOR Serine-Threonine Kinases antagonists & inhibitors
- Abstract
Desmoplastic small round cell tumor (DSRCT) is a rare and aggressive neoplasm that mostly occurs in young males. No curative treatment options currently exist for this type of tumor and long-term survival remains poor. In vitro rapamycin induces apoptotic death of JN-DSRCT-1 cells, a possible model for desmoplastic small round cell tumors in which the EWS gene is fused to the WT1 gene. We therefore demonstrate the prolonged activity of temsirolimus, an mTOR-inhibitor, in a patient with DSRCT.
- Published
- 2010
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