Synovial Sarcoma Dr. Michelle Ghert Dr. Rajiv Gandhi
Synovial Sarcoma Young adult population (15-40yrs) 5-10% of all soft tissue sarcomas mainly found in the extremities 5 year survival only 60% at presentation; mets to lung, nodes, and bone Treatment historically surgery + radiation(effective after wide and marginal excision)
Synovial Sarcoma Aggressive spindle cell sarcoma with two histological subtypes: monophasic and biphasic(glandular epithelial differentiation along with mesenchymal spindle cells) Specific chromosomal translocation t(x;18) SYT gene of 18 fuses with SSX1 or SSX2 of X Present in 95% of SS
Staging-Enneking Grade IA-low grade, single compartment IB-low grade, extra compartmental IIA-high grade, intra compartmental IIB- high grade, extra compartmental III distant mets
What about Chemo? Rhabdomyosarcoma is known to be very chemo sensitive in the pediatric population
Cancer 2004 Ferrari et al. Retrospective, cohort study with MUCH SUBGROUP ANALYSIS 271 patients treated over a 30 year period, age range(5-87 years) 255/271 had localized disease 215/255 had surgical resection the other 40 deemed un-resectable
Treatment Overall 40% of the patients got chemotherapy, corresponding to 76% of patients < 16 years and <20% of older patients( some got systemic(single vs double agent and some got local perfusion chemo)
Results 5 year survival for entire cohort was 37% Broken down by age: Age(years) survival <16 66% 17-30 40% >30 31%
Results(cont d) Of all patients treated with surgical resection, the Mets free survival at 5 years was: 60% for those treated with chemo and 48% for those not tx with chemo
Conclusions Adjuvant chemo should be considered for the young adult SS with tumor size >5cm No mention of complications in the paper
Ann of Onco Spurrell et al 2004 Retrospective review of 104 patients with advanced disease(12 did not have metastatic disease) Mean age at diagnosis 33 years Some patients were treated with surgery + radiation, some had single agent chemo added, and some with double agent chemo(number of cycles varied from 1-7!) 1
Results Median survival was 22 months Predictors for survival were age<35 year (p=0.03) and response to first line tx with chemo(p=0.05) Survival was better for double agent vs single agent chemo
Tissue of Origin Nagayama et al Cancer Res 2002 Allander et al AJP 2002 These two studies indicate thru gene expression and microarray testing, that the tissue origin maybe neural crest rather than mesenchyme (used to analyze info on a genome. Consists of different nucleic acid probes that are chemicallly attached to a substrate)
DOES FUSION TYPE AFFECT CLINICAL OUTCOMES? SYT-SSX1 SSX1 VS SYT-SSX2 SSX2
NEJM Kawai et al. 1998 Retrospectively reviewed 45 SS 29 SSX1 and 16 SSX2 All 12 biphasic tumors were of the SSX1 fusion type and all 16 of the SSX2 were monophasic(p=0.003) Mets free survival was better in the SSX2 group than SSX1(p=0.03) (Some patients had local disease, local recurrence and some had mets at diagnosis; no uniformity in treatment)
Cancer Research Ladany et al. 2002 238 patients retrospectively reviewed- 147 SSX1 and 91 SSX2 Median 5 year survival rate was 53% and 73% respectively Overall survival also better for those with localized disease (p<0.0001), tumor <5cm (p<0.01) SSX1 tumors showed mostly biphasic morphology(presence of glandular differentiation)
J Clin Oncology Guillou et al. 2004 To assess prognostic value of SYT- SSX fusion in SS 141 patients with localized disease reviewed retrospectively Factors were age at diagnosis, sex, tumor size and site, histologic grade, necrosis, mitotic count, and fusion type SYT-SSX1 SSX1 vs SYT-SSX2. SSX2.
Results Histologic Grade was the most prognostic of all factors size >7cm and high mitotic count were also significant SYT-SSX SSX fusion type was not predictive
Take Home Points Synovial Sarcoma is an aggressive tumor with poor survivorship Although studies are limited, chemo may be beneficial in the treatment regiment Why this sarcoma is chemo sensitive and most are not, might be due to it s origin being in neural crest tissue rather than mesenchymal tissue