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Clinicopathological features of Xp11.2 translocation renal cell carcinoma
PURPOSE: Xp11.2 translocation renal cell carcinoma (RCC) is characterized by various translocations of the TFE3 transcription factor gene. These rare cancers occur predominantly in children and young adults. Here, we review the clinicopathological features of Xp11.2 translocation RCC. MATERIALS AND...
Autores principales: | , , , , , , , , , , |
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Formato: | Online Artículo Texto |
Lenguaje: | English |
Publicado: |
The Korean Urological Association
2015
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Materias: | |
Acceso en línea: | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4355432/ https://www.ncbi.nlm.nih.gov/pubmed/25763125 http://dx.doi.org/10.4111/kju.2015.56.3.212 |
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author | Lim, Bumjin You, Dalsan Jeong, In Gab Kwon, Taekmin Hong, Sungwoo Song, Cheryn Cho, Yong Mee Hong, Bumsik Hong, Jun Hyuk Ahn, Hanjong Kim, Choung Soo |
author_facet | Lim, Bumjin You, Dalsan Jeong, In Gab Kwon, Taekmin Hong, Sungwoo Song, Cheryn Cho, Yong Mee Hong, Bumsik Hong, Jun Hyuk Ahn, Hanjong Kim, Choung Soo |
author_sort | Lim, Bumjin |
collection | PubMed |
description | PURPOSE: Xp11.2 translocation renal cell carcinoma (RCC) is characterized by various translocations of the TFE3 transcription factor gene. These rare cancers occur predominantly in children and young adults. Here, we review the clinicopathological features of Xp11.2 translocation RCC. MATERIALS AND METHODS: We identified 21 patients with Xp11.2 translocation RCC. We retrospectively analyzed patient characteristics, clinical manifestations, and specific pathological features to assess definitive diagnosis, surgical and systemic treatments, and clinical outcomes. RESULTS: The mean age at diagnosis was 43.4±20.0 years (range, 8-80 years; 8 males and 13 females). Eleven patients were incidentally diagnosed, nine patients presented with local symptoms, and one patient presented with systemic symptoms. The mean tumor size was 6.2±3.8 cm (range, 1.9-14 cm). At the time of diagnosis, 11, 1, and 5 patients showed stage I, II, and III, respectively. Four patients showed distant metastasis. At analysis, 15 patients were disease-free after a median follow-up period of 30.0 months. Four patients received target therapy but not effectively. CONCLUSIONS: Xp11 translocation RCC tends to develop in young patients with lymph node metastasis. Targeted therapy did not effectively treat our patients. Surgery is the only effective therapy for Xp11 translocation RCC, and further studies are needed to assess systemic therapy and long-term prognosis. |
format | Online Article Text |
id | pubmed-4355432 |
institution | National Center for Biotechnology Information |
language | English |
publishDate | 2015 |
publisher | The Korean Urological Association |
record_format | MEDLINE/PubMed |
spelling | pubmed-43554322015-03-11 Clinicopathological features of Xp11.2 translocation renal cell carcinoma Lim, Bumjin You, Dalsan Jeong, In Gab Kwon, Taekmin Hong, Sungwoo Song, Cheryn Cho, Yong Mee Hong, Bumsik Hong, Jun Hyuk Ahn, Hanjong Kim, Choung Soo Korean J Urol Original Article PURPOSE: Xp11.2 translocation renal cell carcinoma (RCC) is characterized by various translocations of the TFE3 transcription factor gene. These rare cancers occur predominantly in children and young adults. Here, we review the clinicopathological features of Xp11.2 translocation RCC. MATERIALS AND METHODS: We identified 21 patients with Xp11.2 translocation RCC. We retrospectively analyzed patient characteristics, clinical manifestations, and specific pathological features to assess definitive diagnosis, surgical and systemic treatments, and clinical outcomes. RESULTS: The mean age at diagnosis was 43.4±20.0 years (range, 8-80 years; 8 males and 13 females). Eleven patients were incidentally diagnosed, nine patients presented with local symptoms, and one patient presented with systemic symptoms. The mean tumor size was 6.2±3.8 cm (range, 1.9-14 cm). At the time of diagnosis, 11, 1, and 5 patients showed stage I, II, and III, respectively. Four patients showed distant metastasis. At analysis, 15 patients were disease-free after a median follow-up period of 30.0 months. Four patients received target therapy but not effectively. CONCLUSIONS: Xp11 translocation RCC tends to develop in young patients with lymph node metastasis. Targeted therapy did not effectively treat our patients. Surgery is the only effective therapy for Xp11 translocation RCC, and further studies are needed to assess systemic therapy and long-term prognosis. The Korean Urological Association 2015-03 2015-02-26 /pmc/articles/PMC4355432/ /pubmed/25763125 http://dx.doi.org/10.4111/kju.2015.56.3.212 Text en © The Korean Urological Association, 2015 http://creativecommons.org/licenses/by-nc/3.0/ This is an Open Access article distributed under the terms of the Creative Commons Attribution Non-Commercial License (http://creativecommons.org/licenses/by-nc/3.0/) which permits unrestricted non-commercial use, distribution, and reproduction in any medium, provided the original work is properly cited. |
spellingShingle | Original Article Lim, Bumjin You, Dalsan Jeong, In Gab Kwon, Taekmin Hong, Sungwoo Song, Cheryn Cho, Yong Mee Hong, Bumsik Hong, Jun Hyuk Ahn, Hanjong Kim, Choung Soo Clinicopathological features of Xp11.2 translocation renal cell carcinoma |
title | Clinicopathological features of Xp11.2 translocation renal cell carcinoma |
title_full | Clinicopathological features of Xp11.2 translocation renal cell carcinoma |
title_fullStr | Clinicopathological features of Xp11.2 translocation renal cell carcinoma |
title_full_unstemmed | Clinicopathological features of Xp11.2 translocation renal cell carcinoma |
title_short | Clinicopathological features of Xp11.2 translocation renal cell carcinoma |
title_sort | clinicopathological features of xp11.2 translocation renal cell carcinoma |
topic | Original Article |
url | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4355432/ https://www.ncbi.nlm.nih.gov/pubmed/25763125 http://dx.doi.org/10.4111/kju.2015.56.3.212 |
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