title: Multifocal synchronous mucinous adenocarcinomas arising in congenital pulmonary airway malformation: a case report with molecular study.
abstract: OBJECTIVE
  Congenital pulmonary airway malformation (CPAM) is a rare developmental anomaly of the lung. Here, we report a case of mucinous adenocarcinoma arising in CPAM. A 23-month-old boy underwent a thoracoscopic lobectomy of the left upper lobe of the lung based on a presumptive diagnosis of asymptomatic CPAM, found in antenatal sonogram.
  
  
  RESULTS
  Histologically, the lesion was consistent with CPAM, Stocker type I. In addition, multiple foci ranging from mucinous epithelial hyperplasia to mucinous adenocarcinoma were detected. All lesions shared the same immunoprofile with the expression of cytokeratin (CK) 20, MUC5AC, and human epidermal growth factor receptor2 (HER2), but were negative for CK7, transcription factor 1 (TTF-1), MUC1, CDX2, BRAF (VE1) and anaplastic lymphoma kinase (ALK). K-RAS point mutation (G12V) was also detected in all micro-dissected mucinous lesions but EGFR mutation was not found. All lesions were consistent with mucinous adenocarcinoma. The patient's clinical course has been uneventful during the 12-months follow-up period.
  
  
  CONCLUSIONS
  This interesting case demonstrated that multiple foci in CPAM can synchronously transform into malignancies.
authors: Department of Pathology, Seoul National University Hospital, Seoul, South Korea.
authors: Moon-Young
authors: MY
authors: Kim
authors: Chang Hyun
authors: CH
authors: Kang
authors: Sung-Hye
authors: SH
authors: Park
mesh_terms: D002288:Adenocarcinoma, Mucinous / Q000150:complications* / Q000151:congenital / Q000473:pathology; D002675:Child, Preschool; D015615:Cystic Adenomatoid Malformation of Lung, Congenital / Q000150:complications*; D006801:Humans; D008175:Lung Neoplasms / Q000150:complications* / Q000473:pathology; D008297:Male; D009378:Neoplasms, Multiple Primary / Q000150:complications* / Q000151:congenital / Q000473:pathology
keywords: K-RAS mutation; congenital pulmonary airway malformation; lung cancer; mucinous adenocarcinoma