Ciliated muconodular papillary tumor/bronchiolar adenoma of the lung

Abstract

Ciliated muconodular papillary tumor (CMPT) is an uncommon tumor of the lung, usually detected incidentally in the peripheral lung fields on computed tomography. It is most commonly diagnosed in patients between the sixth and last decades of life. The canonical microscopic feature is a tripartite mixture of ciliated columnar, mucous, and basal cells with papillary architecture. However, studies have shown wide morphological variations in this tumor, such as a lack of ciliated columnar or mucous cells and flat instead of papillary architecture. Hence, a new umbrella term, bronchiolar adenoma (BA), has been proposed to encompass all tumors comprising a uniform layer of basal cells underneath the luminal epithelium displaying bronchiolar differentiation. Recent molecular studies on CMPT/BA discovered a high frequency of driver gene alterations, including mutations in the BRAF, EGFR, AKT1, KRAS, and HRAS genes, as well as ALK gene rearrangements. The tumor follows an indolent clinical course, as no recurrences or metastases have been reported in patients who underwent surgical resection. In this article we review the clinical, radiological, and pathological features, and molecular characteristics of CMPTs/BAs.