Even Stocker raised doubts, if CPAM 4 can be differentiated from PPB-I: “I have seen one case of PPB presented as a ‘CPAM’ that was partially resected at age 2 only to recur as a solid nodule of PPB at age 4.” In a recent report he added that the presence of desmin-positive rhabdomyoblasts rules out CPAM 4 [16]. The gene discussed is DES; the disease is pleuropulmonary blastoma.