Congenital Cystic Adenomatoid Malformation of the Lung

Fisher, John

doi:10.1001/archpedi.1982.03970480037009

Cited by 12 publications

(1 citation statement)

References 19 publications

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“…To date, this has not been described in literature. Fisher et al described a CPAM type 1 case with papillary architecture [6]. However, they found large papillary projections in an otherwise conventional CPAM and not the complex papillary morphology as seen in the current case.…”

Section: Discussioncontrasting

confidence: 46%

A case report of an unusual non-mucinous papillary variant of CPAM type 1 with KRAS mutations

et al. 2020

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Background: congenital pulmonary airway malformation (CPAM) is the most frequent congenital lung disorder. CPAM type 1 is the most common subtype, typically having a cystic radiological and histological appearance. Mucinous clusters in CPAM type 1 have been identified as premalignant precursors for mucinous adenocarcinoma. These mucinous adenocarcinomas and the mucinous clusters in CPAM commonly harbor a specific KRAS mutation. Case presentation: we present a case of a 6-weeks-old girl with CPAM type 1 where evaluation after lobectomy revealed a highly unusual complex non-mucinous papillary architecture in all cystic parts, in which both mucinous clusters and non-mucinous papillary areas harbored the known KRAS mutation. Conclusions: we found that a KRAS mutation thought to be premalignant in mucinous clusters only, was also present in the other cyst lining epithelial cells of this unusual non-mucinous papillary variant of CPAM type 1, warranting clinical follow-up because of uncertain malignant potential.

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Section: Discussioncontrasting

confidence: 46%