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Journal Scan
Sarcoma

Combined checkpoint inhibition therapy shows potential in epithelioid sarcoma

Posted on November 8, 2020

Regardless of prior therapy, extent of disease and performance status, patients with epithelioid and other INI1 protein-deficient sarcomas may benefit from combined checkpoint inhibition therapy, according to a study in the Journal of Immunotherapy.

In patients with epithelioid sarcoma, SMARCB1 inactivation is common. This results in a loss of INI1 protein expression and overexpression of the cancer cell growth, which promotes methyltransferase enzyme, EZH2.

In this case report, a 19-year-old man with IV SMARCB1 inactivated epithelioid sarcoma with recurrent end-stage rapidly progressing bulky disease who had failed standard therapy and treatment with an EZH2 inhibitor, was treated with combination ipilimumab and nivolumab.

The patient underwent complete clinical regression of a large (16.1×18.6 cm) soft tissue back mass within 2 weeks of the first cycle of combination checkpoint inhibition therapy. A PET scan 5 months later showed negative complete remission. Checkpoint inhibition therapy was discontinued after a second negative PET/CT scan 3 months after the first.

Reference

Pecora A, Halpern S, Weber M, et al. Rapid and complete response to combination anti-CTLA-4 and anti-PD-1 checkpoint inhibitor therapy in a patient with stage IV refractory end-stage epithelioid sarcoma: A case report. J Immunother. 2020;43(9):286-290. DOI: 10.1097/CJI.0000000000000332.

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