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

[Johannes]

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.

[D.ap]

Hello Johannes and Elisa,
Thank you for the article .
If I might , I’d like to link this discussion to your post .

viewtopic.php?f=3&t=1633&p=14419&hilit= ... nal#p14419

As well as full text —

https://cancerimmunolres.aacrjournals.o ... t/6/9/1001

We hope to hear an update , soon .
Love ,