Systemic Anti-Cancer Therapy in Synovial Sarcoma: A Systematic Review.

Richard Riedel,Robin Jones,Kerstin Mueller,Antoine Italiano,Juliette Thompson,Brian Van Tine,Seth Pollack,Zaeem Khan,Chet Bohac

doi:10.3390/cancers10110417

Abstract

Synovial sarcoma (SS) is an aggressive malignancy which accounts for approximately 5–10% of all soft-tissue sarcomas. SS has pathologic and genomic characteristics that define it as a distinct subtype of soft tissue sarcoma (STS). STS subtypes continue to be recognized as distinct entities with specific characteristics, including differential chemo-sensitivity. The objective of this study was to conduct a descriptive review of current data on survival outcomes of systemic anti-cancer therapy specific to SS. A systematic literature review was conducted, using a custom search strategy to search EMBASE, Medline and CENTRAL for clinical trials and observational studies reporting overall survival (OS), progression-free survival (PFS) and/or response for cohorts of at least 50 SS patients. We identified 28 studies meeting these criteria, 25 of which were retrospective studies. Only three prospective studies were identified. Survival reports varied widely between studies based on the population, in particular on the disease stage, and reporting was heterogeneous in terms of the time points reported on. For patients with localized disease, reports of five-year PFS ranged from 26% to 80.7% and five-year OS from 40% to 90.7%, whereas five-year OS for patients with metastatic disease was very low at around 10%; and in one case, 0% was reported. Only four of the included publications reported outcomes by type of systemic anti-cancer therapy received. Our study draws attention to the fact that additional prospective studies to better define the most appropriate treatment for SS in all stages and lines of therapy are still needed.

Highlights

There is limited differentiation in treatment guidelines between soft tissue sarcoma (STS)subtypes [1,2]
A total of 3198 abstracts were identified in the search, and 28 met the inclusion criteria for this review (Figure 1)
The objectives of the included publications are listed in the supplementary material (Table S2)

Summary

Introduction

There is limited differentiation in treatment guidelines between soft tissue sarcoma (STS)subtypes [1,2]. There is limited differentiation in treatment guidelines between soft tissue sarcoma (STS). Distinct subtypes with specific characteristics are being recognized; features. Cancers 2018, 10, 417 that distinguish subtypes include morphology, immunohistochemistry, genomics, molecular profile and clinical features [3]. Identifying histology-specific treatment is important where STS subtypes are being classified and treated based on molecular and genetic characteristics as well as differential natural history and chemosensitivity [3,4]. The estimated incidence of SS is 1.5 per 1,000,000 in the US and 1.4 per 1,000,000 in the UK [7,9,10,11] It is the most common non-rhabdomyosarcoma STS in children and young adults [8]. SS frequently arises in the extremities, but can develop from serosal surfaces of almost any part of the body such as the pleura or pericardium [7,8]

Objectives

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Results

Discussion

Conclusion