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Clinicopathological features and prognosis of ciliated muconodular papillary tumor

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 trea...

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Autores principales: Shao, Kang, Wang, Yalong, Xue, Qi, Mu, Juwei, Gao, Yushun, Wang, Yonggang, Wang, Bingzhi, Zhou, Lina, Gao, Shugeng
Formato: Online Artículo Texto
Lenguaje:English
Publicado: BioMed Central 2019
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6651997/
https://www.ncbi.nlm.nih.gov/pubmed/31340823
http://dx.doi.org/10.1186/s13019-019-0962-3
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author Shao, Kang
Wang, Yalong
Xue, Qi
Mu, Juwei
Gao, Yushun
Wang, Yonggang
Wang, Bingzhi
Zhou, Lina
Gao, Shugeng
author_facet Shao, Kang
Wang, Yalong
Xue, Qi
Mu, Juwei
Gao, Yushun
Wang, Yonggang
Wang, Bingzhi
Zhou, Lina
Gao, Shugeng
author_sort Shao, Kang
collection PubMed
description 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.
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spelling pubmed-66519972019-07-31 Clinicopathological features and prognosis of ciliated muconodular papillary tumor Shao, Kang Wang, Yalong Xue, Qi Mu, Juwei Gao, Yushun Wang, Yonggang Wang, Bingzhi Zhou, Lina Gao, Shugeng J Cardiothorac Surg Research Article 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. BioMed Central 2019-07-24 /pmc/articles/PMC6651997/ /pubmed/31340823 http://dx.doi.org/10.1186/s13019-019-0962-3 Text en © The Author(s). 2019 Open Access This article is distributed under the terms of the Creative Commons Attribution 4.0 International License (http://creativecommons.org/licenses/by/4.0/), which permits unrestricted use, distribution, and reproduction in any medium, provided you give appropriate credit to the original author(s) and the source, provide a link to the Creative Commons license, and indicate if changes were made. The Creative Commons Public Domain Dedication waiver (http://creativecommons.org/publicdomain/zero/1.0/) applies to the data made available in this article, unless otherwise stated.
spellingShingle Research Article
Shao, Kang
Wang, Yalong
Xue, Qi
Mu, Juwei
Gao, Yushun
Wang, Yonggang
Wang, Bingzhi
Zhou, Lina
Gao, Shugeng
Clinicopathological features and prognosis of ciliated muconodular papillary tumor
title Clinicopathological features and prognosis of ciliated muconodular papillary tumor
title_full Clinicopathological features and prognosis of ciliated muconodular papillary tumor
title_fullStr Clinicopathological features and prognosis of ciliated muconodular papillary tumor
title_full_unstemmed Clinicopathological features and prognosis of ciliated muconodular papillary tumor
title_short Clinicopathological features and prognosis of ciliated muconodular papillary tumor
title_sort clinicopathological features and prognosis of ciliated muconodular papillary tumor
topic Research Article
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6651997/
https://www.ncbi.nlm.nih.gov/pubmed/31340823
http://dx.doi.org/10.1186/s13019-019-0962-3
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