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.
MeSH terms
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Abdominal Neoplasms / pathology*
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Antineoplastic Agents / therapeutic use*
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Humans
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Liver Neoplasms / drug therapy
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Liver Neoplasms / secondary
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Lung Neoplasms / drug therapy
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Lung Neoplasms / secondary
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Lymphatic Metastasis
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Male
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Protein Kinase Inhibitors / therapeutic use*
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Sirolimus / analogs & derivatives*
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Sirolimus / therapeutic use
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TOR Serine-Threonine Kinases / antagonists & inhibitors*
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Young Adult
Substances
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Antineoplastic Agents
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Protein Kinase Inhibitors
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temsirolimus
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TOR Serine-Threonine Kinases
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Sirolimus