title: Expression of MET in alveolar soft part sarcoma.
abstract: Alveolar soft part sarcoma (ASPS) is a rare soft tissue sarcoma which is characterized by the presence of a specific chromosomal translocation encoding the chimeric transcription factor (ASPL-TFE3) that activates expression of MET. We reviewed the clinical features and treatment outcome of 12 ASPS patients. The presence of ASPL-TFE3 fusion transcripts was assessed by reverse transcriptase polymerase chain reaction. In addition, we performed immunohistochemical studies for MET, TFE3, Ki-67, and EGFR expression. Lower extremity was the most commonly affected primary site (2 thigh, 3 lower leg, and 1 foot). Of four patients who received primary cytotoxic chemotherapy, no patient demonstrated treatment response. With follow-up duration of 94.4 months, median overall survival was 53.2 (95% C.I. 40.9-65.5) months. The immunohistochemical staining demonstrated 100% TFE3 positivity (8 of 8), 75% MET positivity (6 of 8) with a strong association between TFE3 expression and MET positivity with correlation coefficient of 0.808 (P = 0.02). The high expression of MET in ASPL-TFE3 (+) ASPS may further support the potential role of targeted agents against MET in this rare, chemoresistant tumor.
authors.affiliation: Division of Hematology-Oncology, Department of Medicine, Samsung Medical Center, Sungkyunkwan University School of Medicine, 50 Ilwon-dong, Gangnam-gu, Seoul 135-710, Korea.
authors.forename: Hyun Jung
authors.initials: HJ
authors.lastname: Jun
authors.forename: Jeeyun
authors.initials: J
authors.lastname: Lee
authors.forename: Do Hyoung
authors.initials: DH
authors.lastname: Lim
authors.forename: Joon Oh
authors.initials: JO
authors.lastname: Park
authors.forename: Geunghwan
authors.initials: G
authors.lastname: Ahn
authors.forename: Sung Wook
authors.initials: SW
authors.lastname: Seo
authors.forename: Ki-Sun
authors.initials: KS
authors.lastname: Sung
authors.forename: Do Hoon
authors.initials: DH
authors.lastname: Lim
authors.forename: Keon Hee
authors.initials: KH
authors.lastname: Yoo
authors.forename: Yoon-La
authors.initials: YL
authors.lastname: Choi
mesh_terms: D000293:Adolescent; D000328:Adult; D002648:Child; D005260:Female; D005528:Foot / Q000473:pathology; D015972:Gene Expression Regulation, Neoplastic; D006801:Humans; D007866:Leg / Q000473:pathology; D008297:Male; D008875:Middle Aged; D019859:Proto-Oncogene Proteins c-met / Q000096:biosynthesis* / Q000235:genetics; D017978:Receptors, Growth Factor / Q000096:biosynthesis* / Q000235:genetics; D012189:Retrospective Studies; D018234:Sarcoma, Alveolar Soft Part / Q000235:genetics / Q000378:metabolism / Q000473:pathology*; D013848:Thigh / Q000473:pathology; D055815:Young Adult
chemical_list: D017978:Receptors, Growth Factor; C491743:MET protein, human; D019859:Proto-Oncogene Proteins c-met