Soft tissue sarcoma– a rare tumor – accounting for less than 1% of all cancers – has the major unmet need in the form of limited treatment options for patients. Cytotoxic chemotherapy has been the mainstay of therapy for decades. Active drugs such as doxorubicin, ifosfamide, gemcitabine, taxanes, and several others are administered either as single agents or in combination regimens.

The most commonly used regimen is the combination of doxorubicin and ifosfamide.

After 40 years, the first drug Adriamycin to get approval for the Soft tissue sarcoma treatment, Lartruvo got approved in the STS market. However, in January 2019, the US FDA recommended that patients stop taking Lartruvo, a day after EU regulators issued a similar warning following the release of results from a failed late-stage phase III study ANNOUNCE study).

The year 2019 witnessed the approval of two more drugs Vitrakvi (larotrectinib) – Bayer and Rozlytrek (Entrectinib) – Roche for curing solid tumors testing positive for NTRK genes. The drugs are kinase inhibitors and are used to treat adult and pediatric patients 12 years of age and older.  

Similarly, Rozlytrek, FDA-approved in August last year, is also used to treat adult patients with metastatic non-small cell lung cancer (NSCLC) whose tumors are ROS1-positive. 

In a similar way, Trabectedin has been approved for L-sarcomas in Europe and a few other countries. Besides the therapies mentioned, other chemotherapeutic drugs such as dacarbazine, gemcitabine, docetaxel, and paclitaxel are also present in the Soft tissue sarcoma market, improving the lives of a certain set of patients.  

The European Society for Medical Oncology (ESMO) guidelines recommend as anthracyclines – as a single agent or in combination with ifosfamide or single-agent ifosfamide as a first-line Soft tissue sarcoma treatment if there are specific contra-indications. Second-line STS treatments include ifosfamide at standard doses if patients have not been previously treated with this agent during first-line treatment. Gemcitabine with or without docetaxel is commonly used in some specific sarcoma subsets. Pazopanib is the first non-chemotherapeutic anticancer agent approved by regulatory authorities for STS.

However, there have been many attempts to improve the treatment outcome of locally advanced or locally relapsed disease by combining local treatment (surgery, radiotherapy) with systemic therapy, such as neo-adjuvant (preoperative) or adjuvant (postoperative) chemotherapy. There are only limited data supporting the use of such perioperative treatments in sarcoma.

The current Soft tissue sarcoma treatment landscape is mainly dependent on targeted therapy, chemotherapy, anti-angiogenesis drugs, and radiation therapy, and supportive care has mostly opted for STS patients. The therapies currently being administered such as dacarbazine, gemcitabine, docetaxel, and paclitaxelare are not approved in the Soft tissue sarcoma treatment market. The approved ones are associated with significant side effects. 

However, pharma companies including Blueprint Medicines Corporation, Advenchen laboratories/Jiangsu Chia-Tai Tianqing Pharmaceutical, Karyopharm Therapeutics, Deciphera Pharmaceuticals, Philogen, GlaxoSmithKline, Epizyme, Eli Lilly and several others are advancing the Soft tissue sarcoma treatment market with novel therapies under trials in their pipeline, soon to be launched in the coming decade.