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Cancer Immunol Res. 2018 Sep;6(9):1001-1007. doi: 10.1158/2326-6066.CIR-18-0037. Epub 2018 Jul 17.

Response to Immune Checkpoint Inhibition in Two Patients with Alveolar Soft-Part Sarcoma.

Author information

1
Department of Medical Oncology and Hematology, Princess Margaret Cancer Centre, Toronto, Canada.
2
Program in Genetics and Genome Biology, The Hospital for Sick Children, Toronto, Canada.
3
Department of Paediatric Laboratory Medicine, The Hospital for Sick Children, Toronto, Canada.
4
Department of Pathology and Laboratory Medicine, Mount Sinai Hospital, Toronto, Canada.
5
The Arthur and Sonia Labatt Brain Tumour Research Centre, The Hospital for Sick Children, Toronto, Ontario, Canada.
6
Department of Medical Biophysics, Faculty of Medicine, University of Toronto, Toronto, Ontario, Canada.
7
Division of Hematology/Oncology, The Hospital for Sick Children, Toronto, Ontario, Canada.
8
Department of Medical Oncology and Hematology, Princess Margaret Cancer Centre, Toronto, Canada. albiruni.razak@uhn.ca.

Abstract

Alveolar soft-part sarcoma (ASPS) is a morphologically distinctive mesenchymal tumor characterized by a canonical ASPL-TFE3 fusion product. In the metastatic setting, standard cytotoxic chemotherapies are typically ineffective. Studies have suggested modest clinical response to multitargeted receptor tyrosine kinase inhibitors. Here, we report sustained partial responses in two patients with immune checkpoint inhibition treated with either durvalumab (anti-PD-L1) alone or in combination with tremelimumab (anti-CTLA-4), which appeared unrelated to tumor immune infiltrates or mutational burden. Genomic analysis of these patients, and other cases of ASPS, demonstrated molecular mismatch-repair deficiency signatures. These findings suggest that immune checkpoint blockade may be a useful therapeutic strategy for ASPS. Cancer Immunol Res; 6(9); 1001-7. ©2018 AACR.

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