Description:   Pleuropulmonary blastoma (PPB) is a neoplasm of early childhood like most of the other dysembryonic neoplasms. This tumor is the most recently described member of the latter group of neoplasms. Its initial reporting in 1988 was followed by the recognition of its pathologic evolution from a multicystic lesion in the lung to a solid multipatterned primitive sarcoma, of its familial predisposition, of its association with a family of extrapulmonary neoplasms and of its heterozygous germline mutation of DICER1 in those with PPB. It was a lesson for me that a great deal can be learned by turning on the microscope.
 

Louis P. “Pepper” Dehner, MD is Professor of Pathology and Immunology and of Pathology in Pediatrics at Washington University School of Medicine, St. Louis, MO. During his tenure, he served as director of anatomic pathology and surgical pathologist-in-chief at Barnes-Jewish Hospital and as the first pathologist-in-chief at St. Louis Children’s Hospital. In 1977, Dr. Dehner was the first to identify and characterize the pediatric lung neoplasm known as pleuropulmonary blastoma (PPB). He and colleagues, notably Dr. Ashley Hill, another School of Medicine graduate, detected the germline mutation in DICER1, which is the genetic substrate for PPB and other related neoplasms.
Dr. Dehner published the first edition of his Pediatric Surgical Pathology in 1975; the fourth edition of his Pediatric Pathology appeared in 2016. He has authored more than 35 books and book chapters, published more than 400 peer-reviewed papers and has held editorial board responsibilities for several renowned journals. He has received numerous awards and invitations to speak around the world. Dr. Dehner has received the Distinguished Pathology Award of the United States and Canadian Academy of Pathology (USCAP), the field’s highest honor. He was elected Councilor of USCAP and later served as its president. Dr. Dehner has been honored with the President’s Award from the Pediatric Pathology Society, the Washington University Medical Center Alumni Association Faculty Alumni Award, and the School of Medicine’s Distinguished Clinician Award.