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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| Main Authors: | , , |
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| Format: | Article (Journal) |
| Language: | English |
| Published: |
2017
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| In: |
Journal of thoracic disease
Year: 2017, Volume: 9, Issue: 9, Pages: E808-E814 |
| ISSN: | 2077-6624 |
| DOI: | 10.21037/jtd.2017.07.95 |
| Online Access: | Verlag, kostenfrei, Volltext: http://dx.doi.org/10.21037/jtd.2017.07.95 Verlag, kostenfrei, Volltext: http://jtd.amegroups.com/article/view/15337 
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| Author Notes: | Wieslawa Grajkowska, Ewa Matyja, Jacek Kunicki, Sylwia Szymanska, Alexander Marx, Cleo-Aron Weis, Renata Langfort, Malgorzata Szolkowska |
| Summary: | 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. |
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| Item Description: | Gesehen am 23.07.2018 |
| Physical Description: | Online Resource |
| ISSN: | 2077-6624 |
| DOI: | 10.21037/jtd.2017.07.95 |