Impact of SYT-SSX fusion type on the clinical behavior of synovial sarcoma: a multi-institutional retrospective study of 243 patients

Marc Ladanyi, Cristina R. Antonescu, Denis H. Leung, James M. Woodruff, Akira Kawai, John H. Healey, Murray F. Brennan, Julia A. Bridge, James R. Neff, Frederic G. Barr, Jeffrey D. Goldsmith, John S. J. Brooks, John R. Goldblum, Syed Z. Ali, Janet Shipley, Colin S. Cooper, Cyril Fisher, Björn Skytting, Olle Larsson

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477 Citations (Scopus)

Abstract

Synovial sarcomas are aggressive spindle cell sarcomas containing in some cases areas of epithelial differentiation. They consistently show a specific t(X;18;p11;q11), which usually represents either of two gene fusions, SYT-SSX1 or SYT-SSX2, encoding putative transcriptional proteins differing at 13 amino acid positions. Previous studies have suggested that patients with SYT-SSX2 tumors do better than those with SYT-SSX1 tumors, but the study groups were too limited to be conclusive. To address this issue more definitively, we collected data on SYT-SSX fusion type, pathology, and clinical course in a retrospective multi-institutional study of 243 patients (age range, 6-82) with synovial sarcoma. SYT-SSX1 and SYT-SSX2 fusions were detected in 147 tumors (61%) and 91 tumors (37%), respectively. Histologically, 61 (25%) were classified as biphasic type and 180 (74%) as monophasic type based on the presence or absence of areas of glandular epithelial differentiation, respectively. Median and 5-year overall survivals for the SYT-SSX1 and SYT-SSX2 groups were 6.1 years and 53%, and 13.7 years and 73%, respectively. Overall survival was significantly better among SYT-SSX2 cases (P = 0.03), among cases localized at diagnosis (P
Original languageEnglish
Pages (from-to)135-140
Number of pages6
JournalCancer Research
Volume62
Issue number1
Publication statusPublished - 1 Jan 2002

Keywords

  • Adolescent
  • Adult
  • Aged
  • Aged, 80 and over
  • Cell Differentiation
  • Child
  • Chromosomes, Human, Pair 18
  • Female
  • Humans
  • Male
  • Middle Aged
  • Neoplasm Metastasis
  • Oncogene Proteins, Fusion
  • Regression Analysis
  • Retrospective Studies
  • Sarcoma, Synovial
  • Survival Rate
  • Translocation, Genetic
  • X Chromosome

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