Skip to main content
Top
Published in: Diagnostic Pathology 1/2017

Open Access 01-12-2017 | Research

Ciliated muconodular papillary tumors of the lung with KRAS/BRAF/AKT1 mutation

Authors: Emiko Udo, Bungo Furusato, Kazuko Sakai, Leah M Prentice, Tomonori Tanaka, Yuka Kitamura, Tomoshi Tsuchiya, Naoya Yamasaki, Takeshi Nagayasu, Kazuto Nishio, Junya Fukuoka

Published in: Diagnostic Pathology | Issue 1/2017

Login to get access

Abstract

Background

Ciliated muconodular papillary tumors (CMPTs) are newly recognized rare peripheral lung nodules that are histologically characterized by ciliated columnar, goblet, and basal cells. Although recent studies have shown that CMPTs constitute a neoplastic disease, the complete histogenesis of CMPTs is not fully understood and molecular data are limited.

Methods

We reviewed four cases of CMPT and performed immunohistochemical and genomic analyses to establish CMPT profiles.

Results

All cases were positive for hepatocyte nuclear factor-4α and mucin 5B and negative for programmed death ligand 1 expression, as determined by immunohistochemistry. The genetic analysis revealed three pathogenic mutations (BRAF V600E, AKT1 E17K, and KRAS G12D), with the KRAS mutation reported here for the first time.

Conclusion

Histological and genetic profiles indicate that CMPTs are likely neoplastic and exhibit features similar to mucinous adenocarcinoma. This suggests that some CMPTs may be a precursor lesion of mucinous adenocarcinoma.
Appendix
Available only for authorised users
Literature
1.
go back to reference Ishikawa Y. Ciliated muconodular papillary tumor of the peripheral lung: benign or malignant. Patholo Clin Med (Bryouri-to-Rinsho). 2002;20:964–5. Ishikawa Y. Ciliated muconodular papillary tumor of the peripheral lung: benign or malignant. Patholo Clin Med (Bryouri-to-Rinsho). 2002;20:964–5.
2.
go back to reference Nakamura S, Koshikawa T, Sato T, Hayashi K, Suchi T. Extremely well differentiated papillary adenocarcinoma of the lung with prominent cilia formation. Acta Pathol Jpn. 1992;42:745–50.PubMed Nakamura S, Koshikawa T, Sato T, Hayashi K, Suchi T. Extremely well differentiated papillary adenocarcinoma of the lung with prominent cilia formation. Acta Pathol Jpn. 1992;42:745–50.PubMed
3.
go back to reference Harada T, Akiyama Y, Ogasawara H, Kishi F, Hattori A, Okamoto K, Hiramatsu M, Ishikawa Y. Ciliated muconodular papillary tumor of the peripheral lung: a newly defined rare tumor. Respir Med. 2008;1:3. Harada T, Akiyama Y, Ogasawara H, Kishi F, Hattori A, Okamoto K, Hiramatsu M, Ishikawa Y. Ciliated muconodular papillary tumor of the peripheral lung: a newly defined rare tumor. Respir Med. 2008;1:3.
4.
go back to reference Kamata T, Sunami K, Yoshida A, Shiraishi K, Furuta K, Shimada Y, Katai H, Watanabe S, Asamura H, Kohno T, Tsuta K. Frequent BRAF or EGFR mutations in ciliated muconodular papillary tumors of the lung. J Thorac Oncol. 2016;11:261–5.CrossRefPubMed Kamata T, Sunami K, Yoshida A, Shiraishi K, Furuta K, Shimada Y, Katai H, Watanabe S, Asamura H, Kohno T, Tsuta K. Frequent BRAF or EGFR mutations in ciliated muconodular papillary tumors of the lung. J Thorac Oncol. 2016;11:261–5.CrossRefPubMed
5.
go back to reference Liu L, Aesif SW, Kipp BR, Voss JS, Daniel S, Aubry MC, Boland JM. Ciliated muconodular papillary tumors of the lung can occur in Western patients and show mutations in BRAF and AKT1. Am J Surg Pathol. 2016;40:1631–6.CrossRefPubMed Liu L, Aesif SW, Kipp BR, Voss JS, Daniel S, Aubry MC, Boland JM. Ciliated muconodular papillary tumors of the lung can occur in Western patients and show mutations in BRAF and AKT1. Am J Surg Pathol. 2016;40:1631–6.CrossRefPubMed
6.
go back to reference Jin Y, Shen X, Shen L, Sun Y, Chen H, Li Y. Ciliated muconodular papillary tumor of the lung harboring ALK gene rearrangement: Case report and review of the literature. Pathol Int. 2017;67:171–5.CrossRefPubMed Jin Y, Shen X, Shen L, Sun Y, Chen H, Li Y. Ciliated muconodular papillary tumor of the lung harboring ALK gene rearrangement: Case report and review of the literature. Pathol Int. 2017;67:171–5.CrossRefPubMed
7.
go back to reference Kogita A, Yoshioka Y, Sakai K, Togashi Y, Sogabe S, Nakai T, Okuno K, Nishio K. Inter- and intra-tumor profiling of multi-regional colon cancer and metastasis. Biochem Biophys Res Commun. 2015;458:52–6.CrossRefPubMed Kogita A, Yoshioka Y, Sakai K, Togashi Y, Sogabe S, Nakai T, Okuno K, Nishio K. Inter- and intra-tumor profiling of multi-regional colon cancer and metastasis. Biochem Biophys Res Commun. 2015;458:52–6.CrossRefPubMed
8.
go back to reference Sato S, Koike T, Homma K, Yokoyama A. Ciliated muconodular papillary tumour of the lung: a newly defined low-grade malignant tumour. Interact Cardiovasc Thorac Surg. 2010;11:685–7.CrossRefPubMed Sato S, Koike T, Homma K, Yokoyama A. Ciliated muconodular papillary tumour of the lung: a newly defined low-grade malignant tumour. Interact Cardiovasc Thorac Surg. 2010;11:685–7.CrossRefPubMed
9.
go back to reference Hata Y, Yuasa R, Sato F, Otsuka H, Goto H, Isobe K, Mitsuda A, Wakayama M, Shibuya K, Takagi K, Watanabe Y. Ciliated muconodular papillary tumor of the lung: a newly defined low-grade malignant tumor with CT findings reminiscent of adenocarcinoma. Jpn J Clin Oncol. 2013;43:205–7.CrossRefPubMed Hata Y, Yuasa R, Sato F, Otsuka H, Goto H, Isobe K, Mitsuda A, Wakayama M, Shibuya K, Takagi K, Watanabe Y. Ciliated muconodular papillary tumor of the lung: a newly defined low-grade malignant tumor with CT findings reminiscent of adenocarcinoma. Jpn J Clin Oncol. 2013;43:205–7.CrossRefPubMed
10.
go back to reference Taguchi R, Higuchi K, Sudo M, Kenji M, Miyamoto T, Mishima O, Kitano M, Azuhata K, Ito N. A case of anaplastic lymphoma kinase (ALK)-positive ciliated muconodular papillary tumor (CMPT) of the lung: Case report and review of the literature. Pathol Int. 2017;67:99–104.CrossRefPubMed Taguchi R, Higuchi K, Sudo M, Kenji M, Miyamoto T, Mishima O, Kitano M, Azuhata K, Ito N. A case of anaplastic lymphoma kinase (ALK)-positive ciliated muconodular papillary tumor (CMPT) of the lung: Case report and review of the literature. Pathol Int. 2017;67:99–104.CrossRefPubMed
11.
go back to reference Kamata T, Yoshida A, Kosuge T, Watanabe S, Asamura H, Tsuta K. Ciliated muconodular papillary tumors of the lung: a clinicopathologic analysis of 10 cases. Am J Surg Pathol. 2015;39:753–60.CrossRefPubMed Kamata T, Yoshida A, Kosuge T, Watanabe S, Asamura H, Tsuta K. Ciliated muconodular papillary tumors of the lung: a clinicopathologic analysis of 10 cases. Am J Surg Pathol. 2015;39:753–60.CrossRefPubMed
12.
go back to reference Kon T, Baba Y, Fukai I, Watanabe G, Uchiyama T, Murata T. Ciliated muconodular papillary tumor of the lung: a Report of five cases. Pathol Int. 2016;66:633–9.CrossRefPubMed Kon T, Baba Y, Fukai I, Watanabe G, Uchiyama T, Murata T. Ciliated muconodular papillary tumor of the lung: a Report of five cases. Pathol Int. 2016;66:633–9.CrossRefPubMed
13.
go back to reference Chu HH, Park SY, Cha EJ. Ciliated muconodular papillary tumor of the lung: The risk of false-positive diagnosis in frozen section. Hum Pathol Case Reports. 2017;7:8–10.CrossRef Chu HH, Park SY, Cha EJ. Ciliated muconodular papillary tumor of the lung: The risk of false-positive diagnosis in frozen section. Hum Pathol Case Reports. 2017;7:8–10.CrossRef
14.
go back to reference Chuang HW, Liao JB, Chang HC, Wang JS, Lin SL, Hsieh PP. Ciliated muconodular papillary tumor of the lung: a newly defined peripheral pulmonary tumor with conspicuous mucin pool mimicking colloid adenocarcinoma: a case report and review of literature. Pathol Int. 2014;64:352–7.CrossRefPubMed Chuang HW, Liao JB, Chang HC, Wang JS, Lin SL, Hsieh PP. Ciliated muconodular papillary tumor of the lung: a newly defined peripheral pulmonary tumor with conspicuous mucin pool mimicking colloid adenocarcinoma: a case report and review of literature. Pathol Int. 2014;64:352–7.CrossRefPubMed
15.
go back to reference Lau KW, Aubry MC, Tan GS, Lim CH, Takano AM. Ciliated muconodular papillary tumor: a solitary peripheral lung nodule in a teenage girl. Hum Pathol. 2016;49:22–6.CrossRefPubMed Lau KW, Aubry MC, Tan GS, Lim CH, Takano AM. Ciliated muconodular papillary tumor: a solitary peripheral lung nodule in a teenage girl. Hum Pathol. 2016;49:22–6.CrossRefPubMed
16.
go back to reference Ishikawa M, Sumitomo S, Imamura N, Nishida T, Mineura K, Ono K. Ciliated muconodular papillary tumor of the lung: report of five cases. J Surg Case Rep. 2016;2016:rjw144.CrossRefPubMedPubMedCentral Ishikawa M, Sumitomo S, Imamura N, Nishida T, Mineura K, Ono K. Ciliated muconodular papillary tumor of the lung: report of five cases. J Surg Case Rep. 2016;2016:rjw144.CrossRefPubMedPubMedCentral
17.
go back to reference Kaufmann O, Dietel M. Thyroid transcription factor-1 is the superior immunohistochemical marker for pulmonary adenocarcinomas and large cell carcinomas compared to surfactant proteins A and B. Histopathology. 2000;36:8–16.CrossRefPubMed Kaufmann O, Dietel M. Thyroid transcription factor-1 is the superior immunohistochemical marker for pulmonary adenocarcinomas and large cell carcinomas compared to surfactant proteins A and B. Histopathology. 2000;36:8–16.CrossRefPubMed
Metadata
Title
Ciliated muconodular papillary tumors of the lung with KRAS/BRAF/AKT1 mutation
Authors
Emiko Udo
Bungo Furusato
Kazuko Sakai
Leah M Prentice
Tomonori Tanaka
Yuka Kitamura
Tomoshi Tsuchiya
Naoya Yamasaki
Takeshi Nagayasu
Kazuto Nishio
Junya Fukuoka
Publication date
01-12-2017
Publisher
BioMed Central
Published in
Diagnostic Pathology / Issue 1/2017
Electronic ISSN: 1746-1596
DOI
https://doi.org/10.1186/s13000-017-0651-2

Other articles of this Issue 1/2017

Diagnostic Pathology 1/2017 Go to the issue