Bronchioloalveolar carcinoma arising in congenital pulmonary airway
malformation in a neonate
Abstract
Congenital pulmonary airway malformation (CPAM), previously known as
congenital cystic adenomatoid malformation (CCAM), is a rare
developmental lung abnormality with the potential for malignant
transformation. Bronchioloalveolar carcinoma (BAC), pleuropulmonary
blastoma (PPB), rhabdomyomatous dysplasia/rhabdomyosarcoma (RMS) have
been associated with CPAM. We report an unusual case of a 1-day-old male
newborn who underwent lobectomy for a cystic lung lesion, which was
found to be a mucinous BAC with K-ras mutation in a type 1 CPAM. The
case supports the relationship between type 1 CPAM and BAC/KRAS mutant,
and highlights that the malignant transformation can occur in very early
stage of the infancy.