DICER1 and cancer: There has not been a prospective clinical study to determine the optimal management of non-PPB DICER1-associated sarcomas, given the rarity and heterogeneity of these cancers; however, regimens used for PPB, such as VAIA (vincristine, actinomycin-D, ifosfamide, doxorubicin) and IVADo, are often used for DICER1-associated sarcomas.