Korean J Pediatr.  2018 Jan;61(1):30-34. 10.3345/kjp.2018.61.1.30.

Case of mucinous adenocarcinoma of the lung associated with congenital pulmonary airway malformation in a neonate

• Koh J
• Jung E
• Jang SJ
• Kim DK
• Lee BS
• Kim KS
• Kim EA

• KMID: 2406084
• DOI: http://doi.org/10.3345/kjp.2018.61.1.30

Abstract

Congenital pulmonary airway malformation (CPAM), previously known as congenital cystic adenomatoid malformation, is a rare developmental lung abnormality associated with rhabdomyosarcoma, pleuropulmonary blastoma, and mucinous adenocarcinoma of the lung. We report an unusual case of a 10-day-old male newborn with a left lower lobe pulmonary cyst who underwent lobectomy, which revealed type II CPAM complicated by multifocal mucinous adenocarcinoma. KRAS sequencing revealed a somatic mutation in Codon12 (GGT → GAT), suggesting the development of a mucinous adenocarcinoma in the background of mucinous metaplasia. Mucinous adenocarcinoma is the most common lung tumor associated with CPAM, but it generally occurs in older children and adults. Further, all cases in the literature are of type I CPAM. This case in a neonate indicates that malignant transformation can occur very early in type II CPAM.

Keyword

Congenital cystic adenomatoid malformation of lung; Mucinous adenocarcinoma; Newborn Infant

MeSH Terms

Adenocarcinoma, Mucinous*
Adult
Child
Cystic Adenomatoid Malformation of Lung, Congenital
Humans
Infant, Newborn*
Lung*
Male
Metaplasia
Mucins*
Rhabdomyosarcoma
Mucins