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

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.

<p>The Efficacy and Safety of Apatinib in Advanced Synovial Sarcoma: A Case Series of Twenty-One Patients in One Single Institution</p>

“…Synovial sarcoma (SS) is an aggressive malignancy of mesenchymal origin, accounting for approximately 8% to 10% of all soft-tissue sarcomas (STS). [1][2][3] SS is marked by the presence of a pathognomonic translocation between chromosomes X and 18, t(X;18)(p11.2;q11.2), 4 and its histological characteristics can be identified including monophasic, biphasic, and poorly differentiated SS. 4 Although SS frequently arises in the extremities, its histological feature is not related to synovial tissue.…”

“…12,13 However, controversy has still existed related to the most effective therapy regimen. 2 Once disease progression or chemo-resistance happened, no appropriate systemic agent is targeting the histologic or genomic characteristics of SS. Therefore, recently, several clinical trials on different approved palliative options have been reported, such as trabectedin, 14 pazopanib, 15 and regorafenib.…”

Synovial sarcoma mimicking a traumatic pseudoaneurysm in the upper extremity

“…A recent study combining two prospective trials has reported that for small, localised disease, a surgery-only approach can be a safe and viable treatment option 16. Additionally, neoadjuvant or adjuvant cytotoxic chemotherapy is the mainstay of treatment in cases of advanced disease5 15 17 with several studies showing improved overall patient survival 18–20. However, due to the relative rarity of this disease and its involvement of paediatric populations, there is limited prospective and randomised data analysing survival outcomes for different treatment options.…”

Calcium-Dependent Calpain Activation-Mediated Mitochondrial Dysfunction and Oxidative Stress Are Required for Cytotoxicity of Epinecidin-1 in Human Synovial Sarcoma SW982 Cells

“…Anthracycline-based regimes are the first-line therapy [3,4]. Second-line chemotherapeutic agents for synovial sarcoma include Pazopanib, Trabectedin, and Eribulin [4][5][6][7]. For metastatic cases, chemotherapy is the main treatment [2].…”