AB thymoma with atypical type A component with delayed multiple lung and brain metastases

An atypical type A thymoma is a newly added entity to the last World Health Organization (WHO) histological classification [2015] of uncertain prognosis. The conventional type A and AB thymomas are usually locally aggressive neoplasms that rarely metastasize with distant metastases to the central ne...

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Hauptverfasser: Grajkowska, Wiesława (VerfasserIn) , Marx, Alexander (VerfasserIn) , Weis, Cleo-Aron Thias (VerfasserIn)
Dokumenttyp: Article (Journal)
Sprache:Englisch
Veröffentlicht: 2017
In: Journal of thoracic disease
Year: 2017, Jahrgang: 9, Heft: 9, Pages: E808-E814
ISSN:2077-6624
DOI:10.21037/jtd.2017.07.95
Online-Zugang:Verlag, kostenfrei, Volltext: http://dx.doi.org/10.21037/jtd.2017.07.95
Verlag, kostenfrei, Volltext: http://jtd.amegroups.com/article/view/15337
Volltext
Verfasserangaben:Wieslawa Grajkowska, Ewa Matyja, Jacek Kunicki, Sylwia Szymanska, Alexander Marx, Cleo-Aron Weis, Renata Langfort, Malgorzata Szolkowska
Beschreibung
Zusammenfassung:An atypical type A thymoma is a newly added entity to the last World Health Organization (WHO) histological classification [2015] of uncertain prognosis. The conventional type A and AB thymomas are usually locally aggressive neoplasms that rarely metastasize with distant metastases to the central nervous system (CNS) occurring extremely exceptionally. We present a history of a woman with a mediastinal tumor originally considered to be a Masaoka-Koga stage II “mixed thymoma with well-differentiated thymic carcinoma component” according to the historic Müller-Hermelink nomenclature. By applying the criteria of the new WHO classification the tumor should be reclassified as an AB thymoma with an atypical A component. The patient developed metastases to the lung and brain 10 and 15 years after the original diagnosis, respectively. All metastases morphologically corresponded to an atypical A component of primary thymoma. Molecular study revealed GTF2I mutations in the primary and one of the metastatic tumors. To our knowledge, this is the first description of a GTF2I mutation in AB thymoma with atypical A component and its metastases. The presented case highlights the necessity of an accurate microscopic search for atypical areas in A or AB thymomas because of their potentially negative impact on prognosis.
Beschreibung:Gesehen am 23.07.2018
Beschreibung:Online Resource
ISSN:2077-6624
DOI:10.21037/jtd.2017.07.95