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Characterization of pulmonary cysts in Birt–Hogg–Dubé syndrome: histopathological and morphometric analysis of 229 pulmonary cysts from 50 unrelated patients

AIMS: To characterize the pathological features of pulmonary cysts, and to elucidate the possible mechanism of cyst formation in the lungs of patients with Birt–Hogg–Dubé syndrome (BHDS), a tumour suppressor gene syndrome, using histological and morphometric analyses. METHODS AND RESULTS: We evaluat...

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Autores principales: Kumasaka, Toshio, Hayashi, Takuo, Mitani, Keiko, Kataoka, Hideyuki, Kikkawa, Mika, Tobino, Kazunori, Kobayashi, Etsuko, Gunji, Yoko, Kunogi, Makiko, Kurihara, Masatoshi, Seyama, Kuniaki
Formato: Online Artículo Texto
Lenguaje:English
Publicado: BlackWell Publishing Ltd 2014
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4237186/
https://www.ncbi.nlm.nih.gov/pubmed/24393238
http://dx.doi.org/10.1111/his.12368
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author Kumasaka, Toshio
Hayashi, Takuo
Mitani, Keiko
Kataoka, Hideyuki
Kikkawa, Mika
Tobino, Kazunori
Kobayashi, Etsuko
Gunji, Yoko
Kunogi, Makiko
Kurihara, Masatoshi
Seyama, Kuniaki
author_facet Kumasaka, Toshio
Hayashi, Takuo
Mitani, Keiko
Kataoka, Hideyuki
Kikkawa, Mika
Tobino, Kazunori
Kobayashi, Etsuko
Gunji, Yoko
Kunogi, Makiko
Kurihara, Masatoshi
Seyama, Kuniaki
author_sort Kumasaka, Toshio
collection PubMed
description AIMS: To characterize the pathological features of pulmonary cysts, and to elucidate the possible mechanism of cyst formation in the lungs of patients with Birt–Hogg–Dubé syndrome (BHDS), a tumour suppressor gene syndrome, using histological and morphometric analyses. METHODS AND RESULTS: We evaluated 229 lung cysts from 50 patients with BHDS and 117 from 34 patients with primary spontaneous pneumothorax (PSP) for their number, size, location and absence or presence of inflammation. The BHDS cysts abutted on interlobular septa (88.2%) and had intracystic septa (13.6%) or protruding venules (39.5%) without cell proliferation or inflammation. The frequencies of these histological characteristics differed significantly from those seen in the lungs of patients with PSP (P < 0.05). Although the intrapulmonary BHDS cysts were smaller than the subpleural BHDS cysts (P < 0.001), there was no difference in size between them when there was no inflammation. The number of cysts diminished logarithmically and the proportion of cysts with inflammation increased as their individual sizes became greater (P < 0.05). CONCLUSIONS: These results imply that the BHDS cysts are likely to develop in the periacinar region, an anatomically weak site in a primary lobule, where alveoli attach to connective tissue septa. We hypothesize that the BHDS cysts possibly expand in size as the alveolar walls disappear at the alveolar-septal junction, and grow even larger when several cysts fuse.
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spelling pubmed-42371862014-12-15 Characterization of pulmonary cysts in Birt–Hogg–Dubé syndrome: histopathological and morphometric analysis of 229 pulmonary cysts from 50 unrelated patients Kumasaka, Toshio Hayashi, Takuo Mitani, Keiko Kataoka, Hideyuki Kikkawa, Mika Tobino, Kazunori Kobayashi, Etsuko Gunji, Yoko Kunogi, Makiko Kurihara, Masatoshi Seyama, Kuniaki Histopathology Original Articles AIMS: To characterize the pathological features of pulmonary cysts, and to elucidate the possible mechanism of cyst formation in the lungs of patients with Birt–Hogg–Dubé syndrome (BHDS), a tumour suppressor gene syndrome, using histological and morphometric analyses. METHODS AND RESULTS: We evaluated 229 lung cysts from 50 patients with BHDS and 117 from 34 patients with primary spontaneous pneumothorax (PSP) for their number, size, location and absence or presence of inflammation. The BHDS cysts abutted on interlobular septa (88.2%) and had intracystic septa (13.6%) or protruding venules (39.5%) without cell proliferation or inflammation. The frequencies of these histological characteristics differed significantly from those seen in the lungs of patients with PSP (P < 0.05). Although the intrapulmonary BHDS cysts were smaller than the subpleural BHDS cysts (P < 0.001), there was no difference in size between them when there was no inflammation. The number of cysts diminished logarithmically and the proportion of cysts with inflammation increased as their individual sizes became greater (P < 0.05). CONCLUSIONS: These results imply that the BHDS cysts are likely to develop in the periacinar region, an anatomically weak site in a primary lobule, where alveoli attach to connective tissue septa. We hypothesize that the BHDS cysts possibly expand in size as the alveolar walls disappear at the alveolar-septal junction, and grow even larger when several cysts fuse. BlackWell Publishing Ltd 2014-07 2014-03-04 /pmc/articles/PMC4237186/ /pubmed/24393238 http://dx.doi.org/10.1111/his.12368 Text en © 2014 The Authors. Histopathology Published by John Wiley & Sons Ltd. http://creativecommons.org/licenses/by-nc-nd/3.0/ This is an open access article under the terms of the Creative Commons Attribution-NonCommercial-NoDerivs License, which permits use and distribution in any medium, provided the original work is properly cited, the use is non-commercial and no modifications or adaptations are made.
spellingShingle Original Articles
Kumasaka, Toshio
Hayashi, Takuo
Mitani, Keiko
Kataoka, Hideyuki
Kikkawa, Mika
Tobino, Kazunori
Kobayashi, Etsuko
Gunji, Yoko
Kunogi, Makiko
Kurihara, Masatoshi
Seyama, Kuniaki
Characterization of pulmonary cysts in Birt–Hogg–Dubé syndrome: histopathological and morphometric analysis of 229 pulmonary cysts from 50 unrelated patients
title Characterization of pulmonary cysts in Birt–Hogg–Dubé syndrome: histopathological and morphometric analysis of 229 pulmonary cysts from 50 unrelated patients
title_full Characterization of pulmonary cysts in Birt–Hogg–Dubé syndrome: histopathological and morphometric analysis of 229 pulmonary cysts from 50 unrelated patients
title_fullStr Characterization of pulmonary cysts in Birt–Hogg–Dubé syndrome: histopathological and morphometric analysis of 229 pulmonary cysts from 50 unrelated patients
title_full_unstemmed Characterization of pulmonary cysts in Birt–Hogg–Dubé syndrome: histopathological and morphometric analysis of 229 pulmonary cysts from 50 unrelated patients
title_short Characterization of pulmonary cysts in Birt–Hogg–Dubé syndrome: histopathological and morphometric analysis of 229 pulmonary cysts from 50 unrelated patients
title_sort characterization of pulmonary cysts in birt–hogg–dubé syndrome: histopathological and morphometric analysis of 229 pulmonary cysts from 50 unrelated patients
topic Original Articles
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4237186/
https://www.ncbi.nlm.nih.gov/pubmed/24393238
http://dx.doi.org/10.1111/his.12368
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