• J. Pediatr. Surg. · Nov 2010

    Case Reports

    Mucinous adenocarcinoma of the lung in association with congenital pulmonary airway malformation.

    • Ryan J Summers, Bahig M Shehata, John C Bleacher, Christina Stockwell, and Louis Rapkin.
    • Emory University School of Medicine, Atlanta, GA 30322, USA.
    • J. Pediatr. Surg. 2010 Nov 1; 45 (11): 2256-9.

    AbstractCongenital pulmonary airway malformation (CPAM) is a rare developmental abnormality of the lung that has been associated with the presence of rhabdomyosarcoma, pleuropulmonary blastoma, and most commonly bronchioalveolar carcinoma (BAC) of the lung. Here, we report the case of an 8-year-old patient who developed KRAS mutation positive stage IV mucinous adenocarcinoma of the lung in association with CPAM. This case reflects the previously recognized progression of CPAM to malignancy and suggests that BAC arising in CPAM may take a more aggressive course than previously recognized.Copyright © 2010 Elsevier Inc. All rights reserved.

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