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

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• 作者：Ryan J. Summers ; Bahig M. Shehata ; John C. Bleacher ; Christina Stockwell ; Louis Rapkin
• 关键词：Mucinous Adenocarcinoma ; Congenital pulmonary airway malformation (CPAM) ; KRAS
• 刊名：Journal of Pediatric Surgery
• 出版年：2010
• 出版时间：November 2010
• 年：2010
• 卷：45
• 期：11
• 页码：2256-2259
• 全文大小：329 K

文摘

Congenital 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.