Previously published cases on the use of targeted agents in DICER1-associated malignancies include a young adult patient with an anaplastic sarcoma of the kidney with biallelic somatic DICER1 mutations and concurrent activating PDGFRA p.D842V mutation, treated with avapritinib, resulting in significant response but intolerable toxicities14. Here, PDGFRA is linked to kidney sarcoma.