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Mucinous adenocarcinoma arising in congenital pulmonary airway malformation: clinicopathological analysis of 37 cases

Lookup NU author(s): Dr Fiona Black



This work is licensed under a Creative Commons Attribution 4.0 International License (CC BY 4.0).


© 2020 The Authors. Histopathology published by John Wiley & Sons Ltd. Aims: Mucinous adenocarcinoma arising in congenital pulmonary airway malformation (CPAM) is a rare complication, with little being known about its natural course. The aims of this article are to describe a series of mucinous adenocarcinomas arising from CPAMs, and present their clinicopathological features, genetics, and clinical outcome. Methods and results: Thirty-seven cases were collected within a 34-year period, and the subtype of adenocarcinoma and CPAM, tumour location, stage, growth patterns, molecular data and follow-up were recorded. The cohort comprised CPAM type 1 (n = 33) and CPAM type 2 (n = 4). Morphologically, 34 cases were mucinous adenocarcinomas (21 in situ; 13 invasive), and three were mixed mucinous and non-mucinous adenocarcinoma. Seventeen cases showed purely extracystic (intra-alveolar) adenocarcinoma, 15 were mixed intracystic and extracystic, and five showed purely intracystic proliferation. Genetically, nine of 10 cases tested positive for KRAS mutations, four with exon 2 G12V mutation and five with exon 2 G12D mutation. Residual disease on completion lobectomy was observed in two cases, and three cases recurred 7, 15 and 32 years after the original diagnosis. Two patients died of metastatic invasive mucinous adenocarcinoma. Conclusions: Most adenocarcinoma that arise in type 1 CPAMs, are purely mucinous, and are early-stage disease. Intracystic proliferation is associated with lepidic growth, an absence of invasion, and indolent behaviour, whereas extracystic proliferation may be associated with more aggressive behaviour and advanced stage. Most cases are cured by lobectomy, and recurrence/residual disease seems to be associated with limited surgery. Long-term follow-up is needed, as recurrence can occur decades later.

Publication metadata

Author(s): Chang W-C, Zhang YZ, Wolf JL, Hermelijn SM, Schnater JM, von der Thusen JH, Rice A, Lantuejoul S, Mastroianni B, Farver C, Black F, Popat S, Nicholson AG

Publication type: Article

Publication status: Published

Journal: Histopathology

Year: 2021

Volume: 78

Issue: 3

Pages: 434-444

Print publication date: 01/02/2021

Online publication date: 23/10/2020

Acceptance date: 16/08/2020

Date deposited: 05/11/2020

ISSN (print): 0309-0167

ISSN (electronic): 1365-2559

Publisher: John Wiley & Sons Ltd


DOI: 10.1111/his.14239

PubMed id: 32810914


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