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Review of renal carcinoma associated with Xp11.2 translocations/TFE3 gene fusions with focus on pathobiological aspect

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dc.contributor.authorKuroda, Naoto
dc.contributor.authorMikami, Shuji
dc.contributor.authorPan, Chin-Chen
dc.contributor.authorCohen, Ronald J.
dc.contributor.authorHes, Ondrej
dc.contributor.authorMichal, Michal
dc.contributor.authorNagashima, Yoji
dc.contributor.authorTanaka, Yukichi
dc.contributor.authorInoue, Keiji
dc.contributor.authorShuin, Taro
dc.contributor.authorLee, Gang-Hong
dc.date.accessioned2017-03-08T18:18:43Z
dc.date.available2017-03-08T18:18:43Z
dc.date.issued2012
dc.description.abstractThe concept of Xp11.2 renal cell carcinoma (RCC) was recently established as a tumor affecting 15% of RCC patients <45 years. Many patients present with advanced stage with frequent lymph node metastases. Histologically, Xp11.2 RCC is characterized by mixed papillary nested/alveolar growth pattern and tumor cells with clear and/or eosinophilic, voluminous cytoplasm. Neoplastic cells show intense nuclear immunoreactivity to TFE3, while focal immunostaining for melanocytic markers, including melanosome-associated antigen or Melan A in some cases, are also noted. Alpha smooth muscle actin and TFEB are consistently negative. Ultrastructurally, the ASPL-TFE3 RCC variant contains rhomboid crystals in the cytoplasm, similar to that observed in alveolar soft part sarcoma. The fusion of the TFE3 gene with several different genes, including ASPL(17q25), PRCC(1q21), PSF(1q34), NonO (Xq12) and CLTC (17q23) have been identified to date. The behavior of Xp11.2 RCC in children and young adults is considered as indolent even when diagnosed at advanced stage, including lymph node metastasis. However, Xp11.2 RCC in older patients behaves in a more aggressive fashion. Therapy includes nephrectomy with extended lymphadenectomy. There may be a role for new protease inhibitors in advanced inoperable disease. Further research is required to correlate clinical behavior with the expanding genetic spectrum of this tumor, and to establish standard therapy protocols for primary and metastatic lesionses
dc.formatapplication/pdfes
dc.format.extent8es
dc.identifier.citationHistology and histopathology, Vol. 27, nº 2 (2012)
dc.identifier.issn1699-5848
dc.identifier.issn0213-3911
dc.identifier.urihttp://hdl.handle.net/10201/52384
dc.languageenges
dc.publisherF. Hernández y Juan F. Madrid. Universidad de Murcia: Departamento de Biología Celular e Histologíaes
dc.rightsinfo:eu-repo/semantics/openAccesses
dc.subjectTFE3es
dc.subjectImmunohistochemistryes
dc.subject.otherCDU::6 - Ciencias aplicadas::61 - Medicina::616 - Patología. Medicina clínica. Oncologíaes
dc.titleReview of renal carcinoma associated with Xp11.2 translocations/TFE3 gene fusions with focus on pathobiological aspectes
dc.typeinfo:eu-repo/semantics/articlees
dspace.entity.typePublicationes
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Histology and histopathology Vol.27, nº 2 (2012)