ArticleVirchows Archiv : an international journal of pathology2020
Atypical goblet cell hyperplasia occurs in CPAM 1, 2, and 3, and is a probable precursor lesion for childhood adenocarcinoma.
Article in Virchows Archiv : an international journal of pathology, 2020. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 11 papers.
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11 citing papers in PubMed, 29 citations in OpenAlex.
- An extremely rare case of congenital pulmonary airway malformation (CPAM) and atypical adenomatous hyperplasia (AAH) with EGFR mutation in a five-year-old girl.BMC pediatrics · 2026Article
- Congenital Pulmonary Airway Malformations in Children: Beyond the Pulmonary Cystic Lesion Is There Really an Associated Laryngo-Tracheal Abnormality?Pediatric pulmonology · 2026Review
- Atypical mesenchyme in congenital pulmonary airways malformation: a promising new focus.Respiratory research · 2025Article
- Comprehensive morphological, immunohistochemical, and molecular characterization of congenital pulmonary airway malformation (CPAM).Virchows Archiv : an international journal of pathology · 2025Article
- A unique case of pulmonary minimally invasive mucinous adenocarcinoma arising from atypical goblet cell hyperplasia in the bronchial epithelium of a 9-year-old girl.BMC pediatrics · 2025Article
- Factors affecting inflammatory changes in congenital lung malformations.Pediatric surgery international · 2024Article
- Congenital Pulmonary Airway Malformation Associated With Papillary Adenocarcinoma.Annals of thoracic surgery short reports · 2024Article
- Article
- Selection of potential targets for stratifying congenital pulmonary airway malformation patients with molecular imaging: is MUC1 the one?European respiratory review : an official journal of the European Respiratory Society · 2023Review
- Cancer or Not Cancer: That Is the Question.American journal of respiratory and critical care medicine · 2023Article
- Defining the spatial landscape of KRAS mutated congenital pulmonary airway malformations: a distinct entity with a spectrum of histopathologic features.Modern pathology : an official journal of the United States and Canadian Academy of Pathology, Inc · 2022Article
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Authors and funding
9 authors at 4 institutions in 4 countries.
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Abstract
Congenital pulmonary airway malformation (CPAM) is a developmental disorder. Types 1-2-3 are the more common ones. Atypical goblet cell hyperplasia (AGCH) in CPAM might be a precursor lesion for pulmonary adenocarcinomas. In nine out of 33 CPAM cases, types 1-3 showed foci of goblet cell proliferations. As these cells completely replace normal epithelium, we prefer to name these proliferations AGCH. In 5 cases, adenocarcinomas were seen (AC). All cases were analyzed for proteins possibly being associated with CPAM development: fibroblast growth factor 10 (FGF10) and receptor 2 (FGFR2), forkhead box A1 (FOXA1) and A2 (FOXA2), MUC protein 5AC (MUC5AC), human epidermal growth factor receptor 2 (erbB2, HER2/neu), hepatocyte nuclear factor 4α (HNF4α), SOX2, and Ying Yang protein 1 (YY1). By next generation sequencing, AGCH and adenocarcinomas were evaluated for driver mutations. Expression for FGF10, FGFR2, FOXA1, and FOXA2 was seen in CPAM epithelium and stroma, but not differently in AGCH and AC. SOX2 was positive in CPAM epithelium and AGCH, however weakly in AC. YY1 and MUC5AC showed more intense staining in AGCH and AC than in CPAM epithelium. HER2 was intensely expressed in AC and less intensely in AGCH, but not in CPAM epithelium. KRAS mutation in exon 2 was detected in all AGCH and AC, but was absent in CPAM epithelia. AGCH can arise in CPAM types 1-3. Oncogenic KRAS mutation seems to be the oncogenic driver already in AGCH, proving its role as a precursor lesion for adenocarcinoma. It might upregulate HER2 at the protein level. YY1 seems to be involved in carcinogenesis.
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