title: A SDHB malignant paraganglioma with dramatic response to temozolomide-capecitabine.
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.
authors.affiliation: Hospices Civils de Lyon, Hôpital Edouard Herriot, Fédération des Spécialités Digestives, Place d'Arsonval, Lyon cedex 03, France.
authors.forename: Cécile
authors.initials: C
authors.lastname: Nozières
authors.forename: Thomas
authors.initials: T
authors.lastname: Walter
authors.forename: Marie-Odile
authors.initials: MO
authors.lastname: Joly
authors.forename: Sophie
authors.initials: S
authors.lastname: Giraud
authors.forename: Jean-Yves
authors.initials: JY
authors.lastname: Scoazec
authors.forename: Françoise
authors.initials: F
authors.lastname: Borson-Chazot
authors.forename: Chantal
authors.initials: C
authors.lastname: Simon
authors.forename: Jean-Paul
authors.initials: JP
authors.lastname: Riou
authors.forename: Catherine
authors.initials: C
authors.lastname: Lombard-Bohas
mesh_terms: D000328:Adult; D000971:Antineoplastic Combined Chemotherapy Protocols / Q000627:therapeutic use*; D014408:Biomarkers, Tumor / Q000378:metabolism; D000069287:Capecitabine; D003606:Dacarbazine / Q000008:administration & dosage / Q000031:analogs & derivatives; D003841:Deoxycytidine / Q000008:administration & dosage / Q000031:analogs & derivatives; D004357:Drug Synergism; D005472:Fluorouracil / Q000008:administration & dosage / Q000031:analogs & derivatives; D006801:Humans; D007150:Immunohistochemistry; D008297:Male; D010235:Paraganglioma / Q000188:drug therapy* / Q000201:enzymology* / Q000235:genetics / Q000378:metabolism / Q000601:surgery; D012186:Retroperitoneal Neoplasms / Q000188:drug therapy* / Q000201:enzymology* / Q000235:genetics / Q000378:metabolism / Q000601:surgery; D013385:Succinate Dehydrogenase / Q000235:genetics*; D000077204:Temozolomide; D016896:Treatment Outcome
chemical_list: D014408:Biomarkers, Tumor; D003841:Deoxycytidine; D000069287:Capecitabine; D003606:Dacarbazine; C108095:SDHB protein, human; D013385:Succinate Dehydrogenase; D005472:Fluorouracil; D000077204:Temozolomide