Dr. Dehner discusses his four decades journey with an entity that he first described in 1977. Since his initial description of Pleuropulmonary Blastoma (PPB), the neoplasm has become one of the defining entities of DICER1 Syndrome, a rare autosomal dominant familial tumor predisposition disorder with heterozygous DICER1 germline mutation. A fascinating historical perspective and most informative ModPath Chat episode on the wide spectrum of neoplasms that can be encountered in the syndrome awaits the listener. Study by Dehner et al.DICER1 tumor predisposition syndrome: an evolving story initiated with the pleuropulmonary blastoma. Modern Pathology (2021). https://www.nature.com/articles/s41379-021-00905-8.