BRONCHOPULMONARY-FOREGUT MALFORMATIONS - A CONTINUUM OF PARACRINE HAMARTOMAS

The bronchopulmonary-foregut malformations (BPFM) are usually sporadic , solitary cystic hamartomas involving conducting airways, arteries, v enous drainage, and lung parenchyma. Transitional, compound hamartomas exist, and only their morphology is well-known, Between 1984-1994 we encountered and studied 10 unrelated patients and a stillborn infant w ith BPFM (out of 24,000 families). Ten were diagnosed in utero and one at birth as having congenital cystic adenomatoid malformation of the lung (CCAML), Postnatally, two diagnoses (20%) were corrected to bronc hogenic cyst (BC) and diaphragmatic hernia, respectively. Bilateral lu ng involvement was present in 1 patient, and in 2 there was a consider able macroscopic regression of the hamartoma. Histologic studies of th e six resected CCAML confirmed the diagnosis and implied dysregulated paracrine growth with its cellular and extracellular growth factors, p rotooncogenes, oncogenes, cytokines, cell-adhesive molecules, and rece ptors of these regulatory peptides, and their complex interactions as developmental morphogens in time and space. (C) 1997 Wiley-Liss, Inc.