Bronchopulmonary‐foregut malformations:A continuum of paracrine hamartomas?

Abstract

The bronchopulmonary-foregut malformations (BPFM) are usually sporadic, solitary cystic hamartomas involving conducting airways, arteries, venous 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 with 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 bronchogenic cyst (BC) and diaphragmatic hernia, respectively. Bilateral lung involvement was present in 1 patient, and in 2 there was a considerable macroscopic regression of the hamartoma. Histologic studies of the six resected CCAML confirmed the diagnosis and implied dysregulated paracrine growth with its cellular and extracellular growth factors, protooncogenes, oncogenes, cytokines, cell-adhesive molecules, and receptors of these regulatory peptides, and their complex interactions as developmental morphogens in time and space.

Congenital pulmonary malformations associated with esophageal duplication and teratoma: prenatal to postnatal management

“…Because of the unusual imaging appearances and the lack of a definitive diagnosis, elective surgical excision was considered appropriate at an older age. It is thought that BPFMs form a spectrum of malformations that include all congenital cystic changes such as CCAM and which probably share a common embryologic pathogenesis [7]. In our case, the association of esophageal duplication cyst with a nonpatent stalklike attachment to the esophagus, and EPS with CCAM, is consistent with the concept of a shared etiology for BPFMs.…”

“…In our case, the association of esophageal duplication cyst with a nonpatent stalklike attachment to the esophagus, and EPS with CCAM, is consistent with the concept of a shared etiology for BPFMs. Development of the alimentary and respiratory tract is regulated by a complex interaction of factors in time and space, and the development of the BPFMs appears to be related to a dysregulation of these developmental morphogens that include extracellular growth factors, proto-oncogenes, oncogenes, cytokines, cell adhesion molecules, and regulatory peptides [7,8]. In the current case, it could be hypothesized that the BPFM and teratoma shared a common etiology for dysregulated morphogens.…”

Fetal lung lesions: a spectrum of disease. New classification based on pathogenesis, two‐dimensional and color Doppler ultrasound

“…More than 60 years following the first report by Chin and Tang13, the pathogenesis of CCAM and BPS is still unclear. Although all lung lesions appear as distinct types, their embryogenesis may overlap significantly26. Various etiologies have been suggested to describe the pathogenesis of CCAM, among them overgrowth13, hyperplasia27 and hamartoma28.…”

Broncopulmonary foregut malformations presenting as mass lesions in children: spectrum of imaging findings

“…Several malformations that present as mass lesions have been described, some of which include bronchogenic cyst (BC), pulmonary sequestration (PS), congenital cystic adenomatoid malformation (CCAM) [currently named as congenital pulmonary airway malformation (CPAM)], congenital lobar emphysema (CLA) [currently named as congenital lobar overinflation (CLO)], bronchial atresia, and congenital pulmonary cyst (CPC) (1)(2)(3)(4). Moreover, combinations of those malformations (hybrid malformations) have also been described (2,(5)(6)(7). These malformations can manifest in various ways from respiratory distress at birth to incidental findings on chest radiograph.…”