Please use this identifier to cite or link to this item: http://hdl.handle.net/10201/52384

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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.identifier.citationHistology and histopathology, Vol. 27, nº 2 (2012)es_ES
dc.identifier.issn1699-5848-
dc.identifier.issn0213-3911-
dc.identifier.urihttp://hdl.handle.net/10201/52384-
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_ES
dc.formatapplication/pdfes_ES
dc.format.extent8es_ES
dc.languageenges_ES
dc.publisherF. Hernández y Juan F. Madrid. Universidad de Murcia: Departamento de Biología Celular e Histologíaes_ES
dc.rightsinfo:eu-repo/semantics/openAccesses_ES
dc.subjectTFE3es_ES
dc.subjectImmunohistochemistryes_ES
dc.subject.otherCDU::6 - Ciencias aplicadas::61 - Medicina::616 - Patología. Medicina clínica. Oncologíaes_ES
dc.titleReview of renal carcinoma associated with Xp11.2 translocations/TFE3 gene fusions with focus on pathobiological aspectes_ES
dc.typeinfo:eu-repo/semantics/articlees_ES
Appears in Collections:Vol.27, nº 2 (2012)

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