Expression of MET in alveolar soft part sarcoma

Med Oncol. 2010 Jun;27(2):459-65. doi: 10.1007/s12032-009-9234-8. Epub 2009 May 27.


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.

Publication types

  • Comparative Study

MeSH terms

  • Adolescent
  • Adult
  • Child
  • Female
  • Foot / pathology
  • Gene Expression Regulation, Neoplastic*
  • Humans
  • Leg / pathology
  • Male
  • Middle Aged
  • Proto-Oncogene Proteins c-met / biosynthesis*
  • Proto-Oncogene Proteins c-met / genetics
  • Receptors, Growth Factor / biosynthesis*
  • Receptors, Growth Factor / genetics
  • Retrospective Studies
  • Sarcoma, Alveolar Soft Part / genetics
  • Sarcoma, Alveolar Soft Part / metabolism
  • Sarcoma, Alveolar Soft Part / pathology*
  • Thigh / pathology
  • Young Adult


  • Receptors, Growth Factor
  • MET protein, human
  • Proto-Oncogene Proteins c-met