Clinicopathological features and prognosis of ciliated muconodular papillary tumor
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 a...
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| Format: | Article |
| Language: | English |
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BMC
2019-07-01
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| Series: | Journal of Cardiothoracic Surgery |
| Subjects: | |
| Online Access: | http://link.springer.com/article/10.1186/s13019-019-0962-3 |
| _version_ | 1828848572064333824 |
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| author | Kang Shao Yalong Wang Qi Xue Juwei Mu Yushun Gao Yonggang Wang Bingzhi Wang Lina Zhou Shugeng Gao |
| author_facet | Kang Shao Yalong Wang Qi Xue Juwei Mu Yushun Gao Yonggang Wang Bingzhi Wang Lina Zhou Shugeng Gao |
| author_sort | Kang Shao |
| collection | DOAJ |
| description | 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. |
| first_indexed | 2024-12-12T22:33:20Z |
| format | Article |
| id | doaj.art-c23d9c4b2fad46f6a7885a4bb652fbe2 |
| institution | Directory Open Access Journal |
| issn | 1749-8090 |
| language | English |
| last_indexed | 2024-12-12T22:33:20Z |
| publishDate | 2019-07-01 |
| publisher | BMC |
| record_format | Article |
| series | Journal of Cardiothoracic Surgery |
| spelling | doaj.art-c23d9c4b2fad46f6a7885a4bb652fbe22022-12-22T00:09:33ZengBMCJournal of Cardiothoracic Surgery1749-80902019-07-011411710.1186/s13019-019-0962-3Clinicopathological features and prognosis of ciliated muconodular papillary tumorKang Shao0Yalong Wang1Qi Xue2Juwei Mu3Yushun Gao4Yonggang Wang5Bingzhi Wang6Lina Zhou7Shugeng Gao8Department of Thoracic Surgery, National Cancer Center/ National Clinical Research Center for Cancer/ Cancer Hospital, Chinese Academy of Medical Sciences and Peking Union Medical CollegeDepartment of Thoracic Surgery, National Cancer Center/ National Clinical Research Center for Cancer/ Cancer Hospital, Chinese Academy of Medical Sciences and Peking Union Medical CollegeDepartment of Thoracic Surgery, National Cancer Center/ National Clinical Research Center for Cancer/ Cancer Hospital, Chinese Academy of Medical Sciences and Peking Union Medical CollegeDepartment of Thoracic Surgery, National Cancer Center/ National Clinical Research Center for Cancer/ Cancer Hospital, Chinese Academy of Medical Sciences and Peking Union Medical CollegeDepartment of Thoracic Surgery, National Cancer Center/ National Clinical Research Center for Cancer/ Cancer Hospital, Chinese Academy of Medical Sciences and Peking Union Medical CollegeDepartment of Thoracic Surgery, National Cancer Center/ National Clinical Research Center for Cancer/ Cancer Hospital, Chinese Academy of Medical Sciences and Peking Union Medical CollegeDepartment of Pathology, National Cancer Center/ National Clinical Research Center for Cancer/ Cancer Hospital, Chinese Academy of Medical Sciences and Peking Union Medical CollegeDepartment of Diagnostic Radiology, National Cancer Center/ National Clinical Research Center for Cancer/ Cancer Hospital, Chinese Academy of Medical Sciences and Peking Union Medical CollegeDepartment of Thoracic Surgery, National Cancer Center/ National Clinical Research Center for Cancer/ Cancer Hospital, Chinese Academy of Medical Sciences and Peking Union Medical CollegeAbstract 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.http://link.springer.com/article/10.1186/s13019-019-0962-3Ciliated muconodular papillary tumorLungPrognosis |
| spellingShingle | Kang Shao Yalong Wang Qi Xue Juwei Mu Yushun Gao Yonggang Wang Bingzhi Wang Lina Zhou Shugeng Gao Clinicopathological features and prognosis of ciliated muconodular papillary tumor Journal of Cardiothoracic Surgery Ciliated muconodular papillary tumor Lung Prognosis |
| 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 | Ciliated muconodular papillary tumor Lung Prognosis |
| url | http://link.springer.com/article/10.1186/s13019-019-0962-3 |
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