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. 2018 Jan;61(1):30-34.
doi: 10.3345/kjp.2018.61.1.30. Epub 2018 Jan 22.

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

Juneyoug Koh  1 , Euiseok Jung  1 , Se Jin Jang  2 , Dong Kwan Kim  3 , Byong Sop Lee  1 , Ki-Soo Kim  1 , Ellen Ai-Rhan Kim  1
Affiliations

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

Juneyoug Koh et al. Korean J Pediatr. 2018 Jan.

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.

Keywords: Congenital cystic adenomatoid malformation of lung; Mucinous adenocarcinoma; Newborn Infant.

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Conflict of interest statement

Conflict of interest: We have no relevant financial relationships to disclose or conflicts of interest to report.

Figures

Fig. 1
Fig. 1. (A) Several large cysts are aggregated on the left lower lobe, with the largest cyst measuring 6 cm in diameter. The left upper lobe has normal features, but is displaced towards the upper section with a decrease in volume due to the mass effect. (B) The mediastinal structures indicate signs of right-sided midline shifting caused by the malformation of the left lower lobe, whereas the right lung has diminished in volume, especially the right upper lobe.
Fig. 2
Fig. 2. (A) Inspection of the resected lobe revealed polycystic lesions, ranging in diameter from 8 to 20 mm, with the main cyst measuring 35×20 mm. (B) Analysis of pathology revealed type II congenital pulmonary airway malformation with extensive mucinous metaplasia. (H&E, ×40)
Fig. 3
Fig. 3. Presence of a mutation in codon 12 of KRAS: G12D (GGT→GAT).
See this image and copyright information in PMC

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