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.

Pediatric pulmonology 2021 year in review: Rare and diffuse lung disease

“…One of the arguments in favor of resection of all CLM has been the discovery of premalignant or malignant cells within several different lesion types 75 . Viganó et al 79 detected multiple foci of bronchoalveolar carcinoma cells in a symptomatic antenatally‐diagnosed CPAM removed from a one‐day old neonate. Within tissue from the lesion, the authors identified an activating variant in the KRAS oncogene, previously reported in other similar cases 80 .…”