Advances in systemic treatment of advanced soft tissue sarcomas

Abstract

Systemic treatment in soft tissue sarcoma (STS) is an important element of therapy in a disease eligible for combined treatment with a radical intention and is the basis for the treatment of an unresectable or metastatic disease. The possibilities of treating STS are limited and for many years progress in this area was minor and the main drugs used in this indication were and still remain anthracyclines and alkylating agents. Clinical trials with new drugs are difficult for STS due to the heterogeneity of these tumours and due to their rare occurrence. Over the past two decades, there have been tested many substances in this indication, including molecularly targeted drugs. Great success was imatinib in the treatment of gastrointestinal stromal tumours (GIST). Other drugs in STS have been tested and approved for use, e.g. sunitinib and regorafenib in the treatment of GIST, pazopanib in the treatment of non-GIST STS, trabectedin and olaratumab in the treatment of STS. First reports on the effectiveness of immunotherapy in the treatment of rare subtypes of STS are also available.

Epithelioid sarcoma

“…Most recently, tazemetostat showed activity in advanced ES with loss of INI1/SMARCB1. Tazemetostat therapy is a new treatment option for patients with ES approved by the FDA [90,91].…”

Giant cell tumor of tendon sheath in the pediatric population – review

“…The ENLIVEN study published in 2016 demonstrated that oral doses of pexidartinib (inhibitor of the colony stimulating factor 1 receptor, CSF1R) is effective in treating GCTTS. It is noteworthy that this study included only patients above 18 years of age and at this moment there are no published articles regarding its use in the pediatric population [12,14,[23][24].…”

“…The global incidence rate of GCTTS located in the digits, limb and the diffuse-type are respectively 29, 10, and 4 per million person-years [11]. GCTTS is even less common in the pediatric population, with an incidence rate of 2.42 and 1.09 per million person-years in case of localized and diffuse tumors, respectively [12]. The knowledge about pediatric GCTTS is scattered across case reports and case series [7,13].…”