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

Research output: Contribution to journalArticlepeer-review

411 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)135-40
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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