title: A clinicopathologic study on SS18 fusion positive head and neck synovial sarcomas.
abstract: To determine clinicopathologic factors on survival in patients with head and neck synovial sarcoma.
  
  
  
  We retrospectively identified patients with molecularly confirmed synovial sarcomas of the head and neck (SS-HN), either by the presence of SS18-SSX fusion transcript by RT-PCR or SS18 gene rearrangement by FISH, who were managed at our institution over a 20-year period (1996-2015). Kaplan-Meier survival analysis and log-rank test were performed to evaluate variables related to disease specific survival (DSS). Fisher exact test was performed to evaluate variables related to local recurrence.
  
  
  
  Thirty-four patients (20 males and 14 females, mean of 31years) with SS18-SSX fusion-positive SS-HN were identified. The parapharyngeal region of the neck was the most common site. The mean tumor size was 4.8cm (0.8-10cm). Two-thirds (n=23) of cases had a monophasic histology. The 2, 5 and 10-year DSS rates were 97%, 79% and 68%. The 5-year DSS rates for the adult/pediatric cohort were 74%/88%. Recurrence showed significant effect on DSS (p=0.021). There was no significant effect on DSS with age, therapy modality, tumor site, surgical margin, tumor size (⩽5cm vs. >5cm) and histopathologic subtype. Tumor site (i.e. skull base/paranasal sinus region) was associated with local recurrence (p=0.003).
  
  
  
  In our cohort DSS rate was associated with recurrence. Tumors located in the skull base/paranasal sinus region were associated with a higher rate of local recurrence. Thus appropriate selection of high risk patients who can benefit from multimodality therapies might improve survival.
authors.affiliation: Department of Surgery, Memorial Sloan Kettering Cancer Center, New York, United States.
authors.forename: Adepitan A
authors.initials: AA
authors.lastname: Owosho
authors.affiliation: Department of Surgery, Memorial Sloan Kettering Cancer Center, New York, United States.
authors.forename: Cherry L
authors.initials: CL
authors.lastname: Estilo
authors.affiliation: Department of Surgery, Memorial Sloan Kettering Cancer Center, New York, United States.
authors.forename: Evan B
authors.initials: EB
authors.lastname: Rosen
authors.affiliation: Department of Surgery, Memorial Sloan Kettering Cancer Center, New York, United States.
authors.forename: SaeHee K
authors.initials: SK
authors.lastname: Yom
authors.affiliation: Department of Surgery, Memorial Sloan Kettering Cancer Center, New York, United States.
authors.forename: Joseph M
authors.initials: JM
authors.lastname: Huryn
authors.affiliation: Department of Pathology, Memorial Sloan Kettering Cancer Center, New York, United States. Electronic address: antonesc@mskcc.org.
authors.forename: Cristina R
authors.initials: CR
authors.lastname: Antonescu
mesh_terms: D000328:Adult; D000368:Aged; D003131:Combined Modality Therapy; D005260:Female; D006258:Head and Neck Neoplasms / Q000000981:diagnostic imaging / Q000235:genetics / Q000473:pathology* / Q000628:therapy; D006801:Humans; D008279:Magnetic Resonance Imaging; D008297:Male; D008875:Middle Aged; D015514:Oncogene Proteins, Fusion / Q000235:genetics*; D011518:Proto-Oncogene Proteins / Q000235:genetics*; D012008:Recurrence; D012097:Repressor Proteins / Q000235:genetics*; D012189:Retrospective Studies; D013584:Sarcoma, Synovial / Q000000981:diagnostic imaging / Q000235:genetics / Q000473:pathology* / Q000628:therapy; D016019:Survival Analysis
chemical_list: D015514:Oncogene Proteins, Fusion; D011518:Proto-Oncogene Proteins; D012097:Repressor Proteins; C093961:SS18 protein, human
keywords: Head and neck sarcomas; SS18-SSX; Synovial sarcoma; t(X;18)