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

Koopman, T., Rottier, B. L., Ter Elst, A. & Timens, W., 24-Feb-2020, In : BMC Pulmonary Medicine. 20, 1, 4 p., 52.

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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.

Original languageEnglish
Article number52
Number of pages4
JournalBMC Pulmonary Medicine
Issue number1
Publication statusPublished - 24-Feb-2020

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