Abstract
BackgroundsThe pulmonary ciliated muconodular papillary tumor (CMPT) is a very rare tumor with only several case reports in published literatures, and its clinicopathological features, standard treatment methods and prognosis has not been well defined.MethodsTwo cases of CMPT diagnosed and treated in our hospital and 39 cases reported in the published literature were analyzed retrospectively.ResultsThe cohort of 41 CMPT patients comprised of 20 males and 21 females, aged 9–84 years. The diameter of the primary tumor was 0.3–4.5 cm. Most of these lesions were subsolid nodules, as observed on computed tomography and easily misdiagnosed as early lung adenocarcinoma. Tumors of 26 patients were stained by immunohistochemistry method, which revealed that CK7, CEA, and TTF-1 were positive and CK20 was negative in most patients. The results of gene alternation demonstrated mutations in EGFR, KRAS, and BRAF and ALK rearrangements in CMPT. All the patients underwent surgical treatment and did not receive postoperative adjuvant therapy. The follow-up duration was 0–120 months, and no case of tumor recurrence was found until the final follow-up.ConclusionsThe incidence of CMPT was low and rate of image misdiagnosis high. Immunohistochemistry is helpful for accurate diagnosis of CMPT. Sub-lobectomy may be proper and adjuvant treatment should be avoided since the disease is now prone to benign lesions. Furthermore, since the biological behavior of this tumor is not yet fully elucidated, additional case data are essential for accurate conclusions.
Highlights
Ishikawa [1], for the first time, reported a rare case of lung tumor in 2002
The first patient had a family history of malignant tumor and exhibited 2 subsolid ground-glass lung nodules and several small pure ground-glass lung nodules by chest computed tomography (CT) scan in a routine examination; the second patient was found to have two nodules in the lung by chest CT scan during regular examination after an operation of thyroid carcinoma 1 year ago
The ciliated muconodular papillary tumor (CMPT) mostly appears as subsolid nodules with a distinct or irregular shape in the CT image and can be diagnosed as lung adenocarcinoma
Summary
Ishikawa [1], for the first time, reported a rare case of lung tumor in 2002. The tumor was 1.5 cm and located in the periphery lung. It exhibited papillary structure and abundant ciliated columnar cells, goblet cells, and basal cells; the alveolar cavity around the primary lesion was filled with mucus lakes. This tumor proliferated slowly and lacked nuclear atypia. The incidence of CMPT is very low; only a few cases have been reported that primarily include Asian population. We retrospectively summarized the clinicopathological data of 41 CMPT cases to provide diagnosis and treatment reference for clinicians
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