title: A case of adult metastatic Xp11 translocation renal cell carcinoma treated successfully with sunitinib.
abstract: Renal cell carcinomas (RCC) associated with Xp11.2 translocations (transcription factor E3 gene fusions) are rare tumors predominantly reported in children. Although only a small number of adult Xp11.2 translocation RCC cases have been described, this is likely due to the possibility of this tumor being unrecognized because of its histologic similarity to either clear cell or papillary subtypes based on hematoxylin and eosin staining. Herein, we describe the first successful case of systemic treatment of metastatic Xp11 RCC with sunitinib, a currently accepted standard of care in metastatic RCC, and attempt to elucidate potential mechanisms behind sunitinib activity in this histologic subtype.
authors.affiliation: Lank Center for Genitourinary Oncology, Dana-Farber Cancer Institute, Boston, MA 02115, USA. Toni_Choueiri@dfci.harvard.edu
authors.forename: Toni K
authors.initials: TK
authors.lastname: Choueiri
authors.forename: Juan-Miguel
authors.initials: JM
authors.lastname: Mosquera
authors.forename: Michelle S
authors.initials: MS
authors.lastname: Hirsch
mesh_terms: D000970:Antineoplastic Agents / Q000627:therapeutic use*; D002292:Carcinoma, Renal Cell / Q000188:drug therapy* / Q000235:genetics / Q000473:pathology; D002880:Chromosomes, Human, Pair 11 / Q000235:genetics*; D041321:Chromosomes, Human, X / Q000235:genetics*; D005260:Female; D015972:Gene Expression Regulation, Neoplastic; D006801:Humans; D017404:In Situ Hybridization, Fluorescence; D007211:Indoles / Q000627:therapeutic use*; D007621:Karyotyping; D007680:Kidney Neoplasms / Q000188:drug therapy* / Q000235:genetics / Q000473:pathology; D008875:Middle Aged; D009362:Neoplasm Metastasis / Q000188:drug therapy; D011758:Pyrroles / Q000627:therapeutic use*; D000077210:Sunitinib; D014178:Translocation, Genetic
chemical_list: D000970:Antineoplastic Agents; D007211:Indoles; D011758:Pyrroles; D000077210:Sunitinib