Abstract
This study reports the treatment feasibility and efficacy of a novel multiagent intensive treatment program for young patients with desmoplastic small round cell tumor. This small series includes three patients and should be seen as a first suggestion of integration of the dose density and the maintenance chemotherapy concept. The IrIVA regimen (irinotecan, ifosfamide, vincristine, and actinomycin-D) is added-used at a short interval between chemotherapy administrations-at more classic intensive ifosfamide-based regimens. The vinorelbine and low-dose oral cyclophosphamide maintenance therapy is added at the end of conventional chemotherapy to achieve an antiangiogenic effect.
Keywords:
Desmoplastic small round cell tumor; IrIVA; chemotherapy; irinotecan; maintenance therapy; vinorelbine.
MeSH terms
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Adolescent
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Adult
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Antineoplastic Combined Chemotherapy Protocols / administration & dosage
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Antineoplastic Combined Chemotherapy Protocols / adverse effects
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Child
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Cyclophosphamide / administration & dosage
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Cyclophosphamide / adverse effects
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Dactinomycin / administration & dosage*
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Dactinomycin / adverse effects
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Desmoplastic Small Round Cell Tumor / drug therapy*
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Desmoplastic Small Round Cell Tumor / pathology
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Desmoplastic Small Round Cell Tumor / surgery
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Dose-Response Relationship, Drug
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Doxorubicin / administration & dosage
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Doxorubicin / adverse effects
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Humans
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Ifosfamide / administration & dosage*
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Ifosfamide / adverse effects
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Irinotecan / administration & dosage*
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Irinotecan / adverse effects
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Male
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Treatment Outcome
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Vincristine / administration & dosage*
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Vincristine / adverse effects
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Vinorelbine / administration & dosage
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Vinorelbine / adverse effects
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Young Adult
Substances
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Dactinomycin
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Vincristine
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Irinotecan
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Doxorubicin
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Cyclophosphamide
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Vinorelbine
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Ifosfamide