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Lymphangioleiomyomatosis : a case report and review of diagnosis and treatment

  • Lymphangioleiomyomatosis (LAM) is a rare disease that generally affects young women and involves the abnormal proliferation of smooth muscle-like cells (LAM cells) in the lungs (pulmonary LAM) and extrapulmonary sites (extrapulmonary LAM). This disease is rare in males. It is hard to distinguish between lung cancer and pulmonary LAM, especially during early stages. Herein, we present a case of a 66-year-old man with a small nodule in the right upper lobe that was first diagnosed as a lung malignancy using a chest CT scan. After a wedge dissection, a pathologist performed a histologic and immunohistochemical examination, and a diagnosis of pulmonary LAM was made. We further performed a 518-gene panel analysis using next-generation sequencing, and only three genes, BARD1, BLM, and BRCA2, were found to have mutations. We also provide a summary of the diagnosis and treatment of this disease.
Metadaten
Verfasserangaben:Yi Liu, Zhibin Guo, Chenlong Zhao, Xin Li, Hongyu Liu, Jun Chen
URN:urn:nbn:de:hebis:30:3-484371
DOI:https://doi.org/10.2147/OTT.S161360
ISSN:1178-6930
Pubmed-Id:https://pubmed.ncbi.nlm.nih.gov/30214240
Titel des übergeordneten Werkes (Englisch):OncoTargets and therapy
Verlag:Dove Medical Press
Verlagsort:Albany, Auckland
Sonstige beteiligte Person(en):Geoffrey Pietersz
Dokumentart:Wissenschaftlicher Artikel
Sprache:Englisch
Jahr der Fertigstellung:2018
Datum der Erstveröffentlichung:31.08.2018
Veröffentlichende Institution:Universitätsbibliothek Johann Christian Senckenberg
Datum der Freischaltung:07.03.2019
Freies Schlagwort / Tag:diagnosis and treatment; lung cancer; lymphangioleiomyomatosis
Jahrgang:11
Seitenzahl:9
Erste Seite:5339
Letzte Seite:5347
Bemerkung:
This work is published and licensed by Dove Medical Press Limited. The full terms of this license are available at https://www.dovepress.com/terms.php and incorporate the Creative Commons Attribution - Non Commercial (unported, v3.0) License. By accessing the work you hereby accept the Terms. Non-commercial uses of the work are permitted without any further permission from Dove Medical Press Limited, provided the work is properly attributed. For permission for commercial use of this work, please see paragraphs 4.2 and 5 of our Terms.
HeBIS-PPN:448056844
Institute:keine Angabe Fachbereich / Extern
DDC-Klassifikation:6 Technik, Medizin, angewandte Wissenschaften / 61 Medizin und Gesundheit / 610 Medizin und Gesundheit
Lizenz (Deutsch):License LogoCreative Commons - Namensnennung-Nicht kommerziell-Keine Bearbeitung 3.0

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