Abstract
BackgroundCiliated muconodular papillary tumors (CMPTs) are newly recognized rare peripheral lung nodules that are histologically characterized by ciliated columnar, goblet, and basal cells. Although recent studies have shown that CMPTs constitute a neoplastic disease, the complete histogenesis of CMPTs is not fully understood and molecular data are limited.MethodsWe reviewed four cases of CMPT and performed immunohistochemical and genomic analyses to establish CMPT profiles.ResultsAll cases were positive for hepatocyte nuclear factor-4α and mucin 5B and negative for programmed death ligand 1 expression, as determined by immunohistochemistry. The genetic analysis revealed three pathogenic mutations (BRAF V600E, AKT1 E17K, and KRAS G12D), with the KRAS mutation reported here for the first time.ConclusionHistological and genetic profiles indicate that CMPTs are likely neoplastic and exhibit features similar to mucinous adenocarcinoma. This suggests that some CMPTs may be a precursor lesion of mucinous adenocarcinoma.
Highlights
Ciliated muconodular papillary tumors (CMPTs) are newly recognized rare peripheral lung nodules that are histologically characterized by ciliated columnar, goblet, and basal cells
CMPTs were first described based on certain pathological features that suggested a malignant potential, similar diseases such as extremely well-differentiated papillary adenocarcinoma with prominent cilia formation have been reported under different names [1,2,3]
Recent reports have revealed the frequent presence of driver gene mutations, and CMPT is recognized as a neoplasia [4,5,6]
Summary
Ciliated muconodular papillary tumors (CMPTs) are newly recognized rare peripheral lung nodules that are histologically characterized by ciliated columnar, goblet, and basal cells. Ciliated muconodular papillary tumors (CMPTs) are a newly recognized small-size papillary tumor of the peripheral lung that contain columnar cells, occasional basal cells, and mucus-producing cells as well as extracellular mucin pools of various sizes. CMPTs were first described based on certain pathological features that suggested a malignant potential, similar diseases such as extremely well-differentiated papillary adenocarcinoma with prominent cilia formation have been reported under different names [1,2,3]. Recent reports have revealed the frequent presence of driver gene mutations, and CMPT is recognized as a neoplasia [4,5,6].
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