Ciliated muconodular papillary tumor of the lung: a case report and literature review

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CASE REPORT

Ciliated muconodular papillary tumor of the lung: a case report and literature review Miyuki Abe1 · Atsushi Osoegawa1 · Michiyo Miyawaki1 · Daiki Noda1 · Takashi Karashima1 · Yohei Takumi1 · Takafumi Hashimoto1 · Tatsuro Okamoto1 · Tsutom Daa2 · Kenji Sugio1  Received: 9 July 2019 / Accepted: 10 November 2019 © The Japanese Association for Thoracic Surgery 2019

Abstract Ciliated muconodular papillary tumor (CMPT) of the lung is characterized as a peripheral low-grade malignant tumor with ciliated columnar cells and goblet cells with basaloid cell proliferation. Herein, we report on a case of CMPT with a radiologically abnormal shadow which was reminiscent of adenocarcinoma. The patient underwent right S6 + S8a segmentectomy because an intraoperative biopsy suggested CMPT, the malignancy of which was difficult to distinguish; however, the tumor was small and located in the peripheral lung. Many details of this tumor remain unclear, as CMPT is a rare tumor with few reports. CMPT has therefore not yet been classified by the WHO. In this report, we will consider the characteristics of CMPT and treatment based on our case and previous case reports. Keywords  Ciliated muconodular papillary tumor · Low-grade malignancy · Lung tumor · CMPT · Segmentectomy

Introduction

Case

Lung tumors with ciliated cells are usually benign, typically occurring in the central airway. Ciliated muconodular papillary tumor (CMPT) is a low-grade malignant tumor and usually appears in the peripheral lung. CMPT is characterized with ciliated columnar cells and goblet cells and it was first reported by Ishikawa et al. in 2002 [1]. Because CMPT is a rare tumor and there are few reports, the radiological findings and clinical behavior are not yet well described. So, CMPT has not yet been classified by the WHO. We report a case of CMPT and consider its characteristics and treatment based on our case and previous case reports.

A 76-year-old man who had a smoking history of 33 packyears was referred to our hospital with abnormal shadow on a chest X-ray during medical checkup. Chest computed tomography (CT) scanning showed an irregular nodule 14 mm in diameter in the peripheral right lower lobe which was reminiscent of adenocarcinoma (Fig. 1a). No fluorodeoxyglucose (FDG) uptake was seen on FDG positron-emission tomography (PET) CT (Fig. 1b). However, the possibility of well-differentiated adenocarcinoma could not be excluded, so he underwent surgical resection. Partial resection was performed and submitted to intraoperative pathological examination, which showed a suspicion of CMPT. We performed right S6 + S8a segmentectomy plus lymph node dissection, because the possibility of malignancy could not be denied. Histological examination revealed a papillary tumor with central fibrosis, proliferating along the alveolar walls, surrounded by mucous lakes, and consisting of ciliated columnar cells and goblet cells with basaloid cell proliferation (Fig. 2). The ultimate histopathological diagnosis was CMPT. No alterations of EGFR, ALK and BRA