Uncommon radiologic computed tomography appearances of the chest in patients with lymphangioleiomyomatosis

Abstract Lymphangioleiomyomatosis (LAM) is a rare destructive lung disease characterized by multiple thin-walled pulmonary cysts. The currently proposed diagnostic algorithm emphasizes the characteristic cystic appearance on high-resolution computed tomography (HRCT) so uncommon HRCT appearances pre...

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Autores principales:	Yasuhito Sekimoto, Kazuhiro Suzuki, Makiko Okura, Takuo Hayashi, Hiroki Ebana, Toshio Kumasaka, Keiko Mitani, Koichi Nishino, Shouichi Okamoto, Etsuko Kobayashi, Kazuhisa Takahashi, Kuniaki Seyama
Formato:	article
Lenguaje:	EN
Publicado:	Nature Portfolio 2021
Materias:	Medicine R Science Q
Acceso en línea:	https://doaj.org/article/061298c7bdce432fb4472fcc89a17959
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spelling	oai:doaj.org-article:061298c7bdce432fb4472fcc89a179592021-12-02T14:23:14ZUncommon radiologic computed tomography appearances of the chest in patients with lymphangioleiomyomatosis10.1038/s41598-021-85999-52045-2322https://doaj.org/article/061298c7bdce432fb4472fcc89a179592021-03-01T00:00:00Zhttps://doi.org/10.1038/s41598-021-85999-5https://doaj.org/toc/2045-2322Abstract Lymphangioleiomyomatosis (LAM) is a rare destructive lung disease characterized by multiple thin-walled pulmonary cysts. The currently proposed diagnostic algorithm emphasizes the characteristic cystic appearance on high-resolution computed tomography (HRCT) so uncommon HRCT appearances present challenges to establishing the proper LAM diagnosis. The objective of this study is to accrue uncommon chest HRCT appearances, determine frequencies in both tuberous sclerosis complex (TSC)-associated LAM (TSC-LAM) and sporadic LAM (S-LAM) patients. 311 females referred to our hospital, including 272 S-LAM patients (mean age 39.2 years) and 39 TSC-LAM patients (mean age 38.3 years), were retrospectively evaluated. We found 2 types of radiologic findings likely to make HRCT cyst appearance atypical: characteristics of the cyst itself and uncommon findings in addition to cysts. We found that approximately 80% of LAM patients, whether TSC-associated or sporadic, showed typical HRCT appearance with mild to severe cystic destruction. The remaining 20% displayed unusual profiles in cyst appearance as well as additional findings aside from cyst: the former includes large cyst, thickened walls, and irregularly shaped whereas the latter includes ground glass attenuation and diffuse noncalcified nodules. It is important to be aware of various radiologic findings that make HRCT cystic appearance atypical of LAM.Yasuhito SekimotoKazuhiro SuzukiMakiko OkuraTakuo HayashiHiroki EbanaToshio KumasakaKeiko MitaniKoichi NishinoShouichi OkamotoEtsuko KobayashiKazuhisa TakahashiKuniaki SeyamaNature PortfolioarticleMedicineRScienceQENScientific Reports, Vol 11, Iss 1, Pp 1-12 (2021)
institution	DOAJ
collection	DOAJ
language	EN
topic	Medicine R Science Q
spellingShingle	Medicine R Science Q Yasuhito Sekimoto Kazuhiro Suzuki Makiko Okura Takuo Hayashi Hiroki Ebana Toshio Kumasaka Keiko Mitani Koichi Nishino Shouichi Okamoto Etsuko Kobayashi Kazuhisa Takahashi Kuniaki Seyama Uncommon radiologic computed tomography appearances of the chest in patients with lymphangioleiomyomatosis
description	Abstract Lymphangioleiomyomatosis (LAM) is a rare destructive lung disease characterized by multiple thin-walled pulmonary cysts. The currently proposed diagnostic algorithm emphasizes the characteristic cystic appearance on high-resolution computed tomography (HRCT) so uncommon HRCT appearances present challenges to establishing the proper LAM diagnosis. The objective of this study is to accrue uncommon chest HRCT appearances, determine frequencies in both tuberous sclerosis complex (TSC)-associated LAM (TSC-LAM) and sporadic LAM (S-LAM) patients. 311 females referred to our hospital, including 272 S-LAM patients (mean age 39.2 years) and 39 TSC-LAM patients (mean age 38.3 years), were retrospectively evaluated. We found 2 types of radiologic findings likely to make HRCT cyst appearance atypical: characteristics of the cyst itself and uncommon findings in addition to cysts. We found that approximately 80% of LAM patients, whether TSC-associated or sporadic, showed typical HRCT appearance with mild to severe cystic destruction. The remaining 20% displayed unusual profiles in cyst appearance as well as additional findings aside from cyst: the former includes large cyst, thickened walls, and irregularly shaped whereas the latter includes ground glass attenuation and diffuse noncalcified nodules. It is important to be aware of various radiologic findings that make HRCT cystic appearance atypical of LAM.
format	article
author	Yasuhito Sekimoto Kazuhiro Suzuki Makiko Okura Takuo Hayashi Hiroki Ebana Toshio Kumasaka Keiko Mitani Koichi Nishino Shouichi Okamoto Etsuko Kobayashi Kazuhisa Takahashi Kuniaki Seyama
author_facet	Yasuhito Sekimoto Kazuhiro Suzuki Makiko Okura Takuo Hayashi Hiroki Ebana Toshio Kumasaka Keiko Mitani Koichi Nishino Shouichi Okamoto Etsuko Kobayashi Kazuhisa Takahashi Kuniaki Seyama
author_sort	Yasuhito Sekimoto
title	Uncommon radiologic computed tomography appearances of the chest in patients with lymphangioleiomyomatosis
title_short	Uncommon radiologic computed tomography appearances of the chest in patients with lymphangioleiomyomatosis
title_full	Uncommon radiologic computed tomography appearances of the chest in patients with lymphangioleiomyomatosis
title_fullStr	Uncommon radiologic computed tomography appearances of the chest in patients with lymphangioleiomyomatosis
title_full_unstemmed	Uncommon radiologic computed tomography appearances of the chest in patients with lymphangioleiomyomatosis
title_sort	uncommon radiologic computed tomography appearances of the chest in patients with lymphangioleiomyomatosis
publisher	Nature Portfolio
publishDate	2021
url	https://doaj.org/article/061298c7bdce432fb4472fcc89a17959
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Uncommon radiologic computed tomography appearances of the chest in patients with lymphangioleiomyomatosis

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