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Korean J Pediatr 2018 January;61(1) :30-34.
Published online 2018 January 15.       
Case of mucinous adenocarcinoma of the lung associated with congenital pulmonary airway malformation in a neonate
Juneyoug Koh1, Euiseok Jung1, Se Jin Jang2, Dong Kwan Kim3, Byong Sop Lee1, Ki-Soo Kim1, Ellen Ai-Rhan Kim1
1Department of Pediatrics, Asan Medical Center, University of Ulsan College of Medicine, Seoul, Korea
2Department of Pathology, Asan Medical Center, University of Ulsan College of Medicine, Seoul, Korea
3Department of Thoracic Surgery, Asan Medical Center, University of Ulsan College of Medicine, Seoul, Korea
Corresponding Author: Ellen Ai-Rhan Kim ,Tel: +82-2-3010-3382, Fax: +82-2-3010-6978, Email:
Copyright © 2018 by The Korean Pediatric Society
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.
Keywords: Congenital cystic adenomatoid malformation of lung | Mucinous adenocarcinoma | Newborn Infant
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