Clinical and Experimental Pediatrics

The Korean Pediatric Society (대한소아청소년과학회)
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Case of mucinous adenocarcinoma of the lung associated with congenital pulmonary airway malformation in a neonate

  • Koh, Juneyoug (Department of Pediatrics, Asan Medical Center, University of Ulsan College of Medicine) ;
  • Jung, Euiseok (Department of Pediatrics, Asan Medical Center, University of Ulsan College of Medicine) ;
  • Jang, Se Jin (Department of Pathology, Asan Medical Center, University of Ulsan College of Medicine) ;
  • Kim, Dong Kwan (Department of Thoracic Surgery, Asan Medical Center, University of Ulsan College of Medicine) ;
  • Lee, Byong Sop (Department of Pediatrics, Asan Medical Center, University of Ulsan College of Medicine) ;
  • Kim, Ki-Soo (Department of Pediatrics, Asan Medical Center, University of Ulsan College of Medicine) ;
  • Kim, Ellen Ai-Rhan (Department of Pediatrics, Asan Medical Center, University of Ulsan College of Medicine)
  • Received : 2017.08.10
  • Accepted : 2017.10.12
  • Published : 2018.01.15
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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 ($GGT{\rightarrow}GAT$), 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.

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References

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