title: Lack of response of a metastatic renal perivascular epithelial cell tumor (PEComa) to successive courses of DTIC based-therapy and imatinib mesylate.
abstract: An 11 year-old girl presented with two large abdominal masses in the left flank and epigastrium and left supraclavicular lymphadenopathy. Subsequent investigations led to the diagnosis of metastatic perivascular epithelioid cell tumor (PEComa) arising from the left kidney. Effective treatment for this rare tumor is not yet known. The tumor did not respond to an initial treatment of two cycles of a dacarbazine (DTIC) based regimen. She was placed on a trial of imatinib mesylate based on tumor expression of c-KIT, a tyrosine kinase targeted by this drug. This report highlights the first documented case of the use of imatinib for PEComa. Lack of response and adverse effects of the drug required discontinuation of therapy.
authors.affiliation: Dalhousie Medical School, Dalhousie University, Halifax, Nova Scotia, Canada.
authors.forename: Heather
authors.initials: H
authors.lastname: Rigby
authors.forename: Weiming
authors.initials: W
authors.lastname: Yu
authors.forename: Matthias H
authors.initials: MH
authors.lastname: Schmidt
authors.forename: Conrad V
authors.initials: CV
authors.lastname: Fernandez
mesh_terms: D018207:Angiomyolipoma / Q000188:drug therapy* / Q000473:pathology; D000970:Antineoplastic Agents / Q000494:pharmacology / Q000627:therapeutic use*; D001549:Benzamides; D002648:Child; D003606:Dacarbazine / Q000627:therapeutic use; D015622:Epithelioid Cells / Q000473:pathology*; D017809:Fatal Outcome; D005260:Female; D006801:Humans; D000068877:Imatinib Mesylate; D007680:Kidney Neoplasms / Q000188:drug therapy* / Q000473:pathology; D009362:Neoplasm Metastasis; D010879:Piperazines / Q000494:pharmacology / Q000627:therapeutic use*; D011743:Pyrimidines / Q000494:pharmacology / Q000627:therapeutic use*; D017211:Treatment Failure
chemical_list: D000970:Antineoplastic Agents; D001549:Benzamides; D010879:Piperazines; D011743:Pyrimidines; D003606:Dacarbazine; D000068877:Imatinib Mesylate