Congenital Pulmonary Airway Malformation
Also written as: CPAM — Congenital Pulmonary Airway Malformation
A spectrum of cystic and adenomatoid lesions of the lung arising from abnormal branching morphogenesis of the tracheobronchial tree, formerly termed cystic adenomatoid malformation.
Full Definition
Congenital pulmonary airway malformation (CPAM) encompasses five subtypes (Types 0–4, per Stocker's classification) distinguished by the level of airway involved and the size and nature of the cystic components. Formerly called congenital cystic adenomatoid malformation (CCAM), the revised term CPAM is now preferred in contemporary literature because it reflects the full spectrum including non-adenomatoid lesions. Pathological examination of resected specimens must include cyst-size measurement, epithelial lining characterisation, and mucogenic cell identification, as these features determine subtype and inform malignant potential (particularly for pleuropulmonary blastoma, which can mimic Type 1 or Type 4 CPAM). Editors should use CPAM as the preferred term; CCAM should be flagged as outdated.
Usage
Usage note: CCAM (congenital cystic adenomatoid malformation) is the legacy term; editors should update CCAM to CPAM unless the document is historical or quotation-based.
In Context
- "The resected left lower lobe contained a Type 1 congenital pulmonary airway malformation with large cysts lined by pseudostratified ciliated epithelium." — Pediatric surgical pathology report
- "The author used 'CCAM' throughout the manuscript; the editor updated all instances to 'CPAM' in line with current terminology." — Manuscript copyedit note