Abstract
Ten percent of paragangliomas are malignant and one-third occurs in a genetic background. We report a case of succinate dehydrogenase subunit B (SDHB)-related malignant paraganglioma with dramatic response to temozolomide and capecitabine regimen (decrease in tumor size of 70% with RECIST criteria). Tumor cells harbored a new mutation in SDHB gene and showed aberrant hypermethylation of O6-methylguanine-DNA-methyltransferase promoter. Our report suggests the importance of molecular predictive factors of response for the selection of chemotherapeutic as well as targeted agents. This observation points to a possible genotype response to treatment relationships, which could help to design tailor-made treatments in the future.
MeSH terms
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Adult
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Antineoplastic Combined Chemotherapy Protocols / therapeutic use*
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Biomarkers, Tumor / metabolism
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Capecitabine
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Dacarbazine / administration & dosage
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Dacarbazine / analogs & derivatives
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Deoxycytidine / administration & dosage
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Deoxycytidine / analogs & derivatives
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Drug Synergism
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Fluorouracil / administration & dosage
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Fluorouracil / analogs & derivatives
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Humans
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Immunohistochemistry
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Male
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Paraganglioma / drug therapy*
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Paraganglioma / enzymology*
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Paraganglioma / genetics
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Paraganglioma / metabolism
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Paraganglioma / surgery
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Retroperitoneal Neoplasms / drug therapy*
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Retroperitoneal Neoplasms / enzymology*
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Retroperitoneal Neoplasms / genetics
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Retroperitoneal Neoplasms / metabolism
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Retroperitoneal Neoplasms / surgery
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Succinate Dehydrogenase / genetics*
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Temozolomide
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Treatment Outcome
Substances
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Biomarkers, Tumor
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Deoxycytidine
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Capecitabine
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Dacarbazine
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SDHB protein, human
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Succinate Dehydrogenase
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Fluorouracil
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Temozolomide