The cERMS typically has the features of the favorable botryoid ERMS with a cambium layer and in almost 50% of the cases foci of cartilage are identified, a seemingly unique feature of ERMS within the DICER1 setting unlike the absence of cartilage in the sporadic ERMS (Fig. 9A, B); this same combination of ERMS and cartilage is present in PPB as well as other DICER1-associated neoplasms. This evidence concerns the gene DICER1 and pleuropulmonary blastoma.