Abstract
A 3-year-old male presented with a large retroperitoneal mass and multiple metastases. Biopsy results suggested alveolar rhabdomyosarcoma bearing a methylated O6-methylguanine-DNA methyltransferase (MGMT) gene promoter. Serum microRNA-206 levels were elevated and remained high after three cycles of vincristine, dactinomycin, and cyclophosphamide (VAC). Replacement of vincristine, irinotecan, and temozolomide (VIT) for VAC induced a marked tumor reduction and normalization of the miR-206 levels. The patient completed 14 cycles of VIT with local radiotherapy and has been in remission for 31 months. Temozolomide could be effective for tumors with a methylated MGMT gene promoter. Individualized therapy is warranted for such patients.
Keywords:
MGMT gene; methylation; rhabdomyosarcoma; temozolomide.
© 2017 Wiley Periodicals, Inc.
MeSH terms
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Antineoplastic Combined Chemotherapy Protocols / administration & dosage*
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Camptothecin / administration & dosage
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Camptothecin / analogs & derivatives
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Chemoradiotherapy*
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Child, Preschool
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Cyclophosphamide / administration & dosage
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DNA Methylation*
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DNA Modification Methylases / metabolism*
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DNA Repair Enzymes / metabolism*
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DNA, Neoplasm / metabolism*
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Dacarbazine / administration & dosage
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Dacarbazine / analogs & derivatives
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Dactinomycin / administration & dosage
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Humans
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Irinotecan
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Male
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MicroRNAs / metabolism
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Neoplasm Metastasis
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Promoter Regions, Genetic*
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RNA, Neoplasm / metabolism
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Rhabdomyosarcoma, Alveolar* / metabolism
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Rhabdomyosarcoma, Alveolar* / pathology
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Rhabdomyosarcoma, Alveolar* / therapy
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Temozolomide
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Tumor Suppressor Proteins / metabolism*
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Vincristine / administration & dosage
Substances
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DNA, Neoplasm
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MIRN206 microRNA, human
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MicroRNAs
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RNA, Neoplasm
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Tumor Suppressor Proteins
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Dactinomycin
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Vincristine
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Irinotecan
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Dacarbazine
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Cyclophosphamide
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DNA Modification Methylases
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MGMT protein, human
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DNA Repair Enzymes
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Camptothecin
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Temozolomide