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خرید پکیج
تعداد آیتم قابل مشاهده باقیمانده : -19 مورد

Classification and characteristics of congenital pulmonary airway malformations

Classification and characteristics of congenital pulmonary airway malformations
Stocker classification Alternate nomenclature Genetic associations Characteristics
CPAM type 0 Acinar dysplasia TBX4-FGF10 pathway (germline)
  • 1 to 3% of CPAM-type malformations
  • Small cysts (<0.5 cm)
  • Tracheal or bronchial origin
  • Lethal at birth
CPAM type 1   KRAS (somatic)
  • 60 to 70% of CPAMs
  • Single or multilocular thin-walled cysts (2 to 10 cm)
  • Distal bronchi or proximal bronchiolar origin
  • Course depends on cyst size
  • Modest malignant potential; can be difficult to distinguish from type 4
CPAM type 2 Bronchial atresia-related cystic lung disease  
  • 15 to 20% of CPAMs
  • Small mixed solid-cystic lesions (0.5 to 2 cm)
  • 80 to 90% have associated bronchial atresia
  • May have associated extra and intralobar sequestration
  • Often associated with other congenital anomalies*
CPAM type 3   KRAS or BRAF (somatic)
  • 5 to 10% of CPAMs
  • Numerous small cysts (<0.5 cm); solid or mixed solid-cystic
  • May involve an entire lobe or more
  • Acinar origin
  • Often severe respiratory distress at birth
CPAM type 4 PPB type 1 DICER1 (germline)
  • 5 to 10% of CPAM-type malformations
  • Large thin-walled cysts (<7 cm), may be bilateral or multifocal, or single large air-filled cyst
  • Radiographic appearance can be similar to CPAM type 1
  • Strong malignant potential; considered a form of PPB
  • Clues to the diagnosis:
    • Often present with pneumothorax
    • More likely if cysts are bilateral or multifocal
    • May have a family history of DICER1 syndrome (PPB, ovarian or kidney tumors, or a variety of other malignancies)

CPAM: congenital pulmonary airway malformation; PPB: pleuropulmonary blastoma.

* Associated congenital anomalies include esophageal atresia-tracheoesophageal fistula, renal agenesis, intestinal atresias, other pulmonary malformations, and diaphragmatic, cardiac, central nervous system, and bony anomalies.

Reference:
  1. Dehner LP, Schultz KAP, Hill DA. Congenital pulmonary airway malformations with a reconsideration and current perspective on the stocker classification. Pediatr Dev Pathol 2023; 26:241.
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