KMID : 0358320150560030212
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Korean Journal of Urology 2015 Volume.56 No. 3 p.212 ~ p.217
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Clinicopathological features of Xp11.2 translocation renal cell carcinoma
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Lim Bum-Jin
You Dal-San Jeong In-Gab Kwon Taek-Min Hong Sung-Woo Song Che-Ryn Cho Yong-Mee Hong Bum-Sik Hong Jun-Hyuk Ahn Han-Jong Kim Choung-Soo
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Abstract
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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¡¾20.0 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¡¾3.8 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.
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KEYWORD
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Angiogenesis inducing agents, Genetic translocation, Renal cell carcinoma
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