Clinicopathological features and prognosis of ciliated muconodular papillary tumor
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(2019) 14:143
RESEARCH ARTICLE
Open Access
Clinicopathological features and prognosis of ciliated muconodular papillary tumor Kang Shao1†, Yalong Wang1†, Qi Xue1, Juwei Mu1, Yushun Gao1, Yonggang Wang1, Bingzhi Wang2, Lina Zhou3 and Shugeng Gao1*
Abstract Backgrounds: The 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. Methods: Two cases of CMPT diagnosed and treated in our hospital and 39 cases reported in the published literature were analyzed retrospectively. Results: The 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. Conclusions: The 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. Keywords: Ciliated muconodular papillary tumor, Lung, Prognosis
Background 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. Microscopically, 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. According to the morphological characteristics and clinical manifestations, it was termed as the ciliated muconodular * Correspondence: [email protected] † Kang Shao and Yalong Wang contributed equally to this work. 1 Department of Thoracic Surgery, National Cancer Center/ National Clinical Research Center for Cancer/ Cancer Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, Beijing 100021, People’s Republic of China Full list of author information is available at the end of the article
papillary tumor [2]. The incidence of CMPT is very low; only a few cases have been reported that primarily include Asian population. The nomenclature of CMPT has not yet been classified into World Health Organization lung tumor classification. Theref
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