Clinicopathologic Features of Translocation Renal Cell Carcinoma

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• 作者：Riyad T. Ellati¹ ; Ibrahim Abukhiran¹ ; Kholoud Alqasem¹ ; Judy Jasser¹ ; Juakub Khzouz² ; Tamer Bisharat¹ ; Ibrahim Al-saidi³ ; Ali Al-Daghmin¹ ; ^{AA.11119@khcc.jo}
• 关键词：RCC ; TFE3 ; Translocation RCC ; Xp11.2 translocation
• 刊名：Clinical Genitourinary Cancer
• 出版年：2017
• 出版时间：February 2017
• 年：2017
• 卷：15
• 期：1
• 页码：112-116
• 全文大小：733 K
• 卷排序：15

文摘

Translocation renal cell carcinomas (TFE3 RCC) are associated with variable genetic rearrangements of the TFE3 gene on chromosome Xp11.2. Translocation tumors represent 1% to 5% of all cases of RCC, with the greatest frequency among children and young adults. We sought to characterize the clinicopathologic features of translocation RCC at a Middle Eastern institution.Materials and MethodsThe clinical and pathologic data from a single institution were retrospectively reviewed. A total of 14 patients with translocation RCC had been diagnosed from 2005 to 2014. The outcome measures included patient characteristics, clinical manifestations, pathologic features, treatment outcomes, cancer-specific survival, and progression-free survival.ResultsThe mean age at diagnosis was 35 years. Of the 14 patients, 5 were female. Translocation RCC was an incidental diagnosis for all but 2 of the 14 patients. The mean tumor size was 9 cm; 1 patient had bilateral tumors, and 3 presented with positive lymph nodes. Three patients underwent partial nephrectomy. Three patients had developed metastasis at 4 months, 5 months, and 3 years after diagnosis. One patent had died 4 months after surgery and one had died 21 months after surgery (both of metastases). The disease-free survival rate was 71% at a mean follow-up of 31 months.ConclusionTranslocation RCC is a rare and potentially aggressive subtype of kidney cancer. An overall survival of > 3 years has been noted, unless metastasis is present at diagnosis.