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Ciliated Muconodular Papillary Tumors of the Lung Can Occur in Western Patients and Show Mutations in BRAF and AKT1

Liu, Liping MD, PhD; Aesif, Scott W. MD, PhD; Kipp, Benjamin R. PhD; Voss, Jesse S. CT(ASCP); Daniel, Silver MD; Aubry, Marie Christine MD; Boland, Jennifer M. MD

The American Journal of Surgical Pathology: December 2016 - Volume 40 - Issue 12 - p 1631–1636
doi: 10.1097/PAS.0000000000000707
Original Articles

Ciliated muconodular papillary tumors (CMPTs) are rare peripheral lung lesions, characterized by papillary architecture and ciliated columnar cells admixed with mucinous cells and basal cells. They often have prominent surrounding intra-alveolar mucin, which can lead to diagnostic confusion with mucinous adenocarcinoma. Recognition of the ciliated component is the key to diagnosis of CMPT. The literature contains few reported cases to date, all occurring in East-Asian patients. Although follow-up data are limited, CMPT seems to be an indolent tumor with very good prognosis, leading some to question whether it is a reactive or hamartomatous lesion. However, a very recent molecular study has identified BRAF (40%) and EGFR (30%) alterations in CMPT, supporting a truly neoplastic process. Here for the first time, we report 4 cases of morphologically typical CMPT in western patients, occurring in 1 man (60 y) and 3 women (71 to 83 y). Interestingly, 1 case occurred in background of pronounced small airway disease with necrotizing bronchiolitis and multiple carcinoid tumorlets. We further analyzed 1 tumor using a 50 gene next-generation sequencing oncology panel that identified 2 pathogenic mutations (BRAF V600E and AKT1 E17K). Our study is the first to describe that CMPT can occur in western (non-Asian) patients. Our data confirm BRAF V600E mutation as a probable driver in a subset of these tumors, along with AKT1 mutation, which further supports that CMPT are indolent pulmonary neoplasms.

*Department of Laboratory Medicine and Pathology, Mayo Clinic, Rochester, MN

Department of Pathology, Northwestern Medicine Central DuPage Hospital, Winfield, IL

Conflicts of Interest and Source of Funding: The authors have disclosed that they have no significant relationships with, or financial interest in, any commercial companies pertaining to this article.

Correspondence: Jennifer M. Boland, MD, Division of Anatomic Pathology, Mayo Clinic, 200 First Street SW, Rochester, MN 55905 (e-mail:

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