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Advanced and metastatic soft tissue sarcoma, a review of aajcc 8th edition staging and the use of olaratumab.

Hari A Deshpande, Ranjan Pathak, Fauzia Riaz

Soft tissue sarcomas represent a rare but histologically variable type of solid malignancy. Doxorubicin based regimens either alone or in combination with other agents had remained unchanged for decades as the standard first line treatment for metastatic disease. The definition of metastatic disease has been changed with the introduction of the American Joint Commission on Cancer (AJCC) 8th edition guidelines. The overall survival for patients with metastatic disease, despite the approval of 2 new agents, had been in the region of 12-19 months. Olaratumab is a monoclonal antibody directed against PDGFR alpha. The results of a randomized phase 2 study comparing olaratumab plus doxorubicin with doxorubicin alone, showed a statistically significant improvement in progression free survival (PFS) up to 6 months, and a more dramatic improvement in overall survival (OS) to 26.9 months. This was the first randomized trial to show a significant improvement in overall survival compared to doxorubicin alone. Olaratumab has been granted accelerated approval by the Food and Drug Administration of the United States of America. Ongoing trials are underway to further demonstrate the mechanism of action and also to confirm the benefit in a Phase III study.