Arnoux V, Long J-A, Fiard G, Pasquier D, Bensaadi L, Terrier N, Rambeaud J-J, Descotes J-L
Service d'urologie et transplantation rénale, CHU de Grenoble, Grenoble cedex, France.
Prog Urol. 2012 Nov;22(15):932-7. doi: 10.1016/j.purol.2012.06.009. Epub 2012 Jul 26.
To describe demographic, therapeutic and follow-up data of four cases of renal cell carcinoma with Xp11.2 translocation in adults older than 50 years of age.
Between January 2008 and December 2011, 170 patients underwent surgery for renal cell carcinoma in our center. Systematic histopathologic analysis of specimen removed was performed. Complementary immunohistochemical analysis was performed only in cases with uncertain diagnosis or in patients younger than 40 years of age.
Among these 170 patients with a median age of 59years old (21-89), immunohistochemistry helped find a TFE3 translocation in four cases (2.4%). There were three women and one man of 53, 71, 75 and 86years old respectively. One patient was metastatic at diagnosis. Radical nephrectomy was first performed in all cases. TNM staging was T3aN2R0, T3bN0R0, T2N2R0 and T3aN2R2, with a Furhman grade of 4. Two patients progressed with metastasis 5 and 7months after surgery, and two with lymphatic invasion 2 and 9months after nephrectomy. One patient died during follow-up.
Xp11.2 translocation renal cell carcinoma was uncommon after 50years of age in our series, but probably under estimated. It seemed to be associated with a poor prognosis. Larger studies must be performed to optimize its specific treatment.
描述4例年龄大于50岁的成人Xp11.2易位肾细胞癌的人口统计学、治疗及随访数据。
2008年1月至2011年12月期间，170例患者在我们中心接受了肾细胞癌手术。对切除的标本进行了系统的组织病理学分析。仅在诊断不明确的病例或年龄小于40岁的患者中进行了补充免疫组化分析。
在这170例中位年龄为59岁（21 - 89岁）的患者中，免疫组化帮助发现4例（2.4%）存在TFE3易位。有3名女性和1名男性，年龄分别为53、71、75和86岁。1例患者在诊断时已有转移。所有病例均首先进行了根治性肾切除术。TNM分期分别为T3aN2R0、T3bN0R0、T2N2R0和T3aN2R2，Fuhrman分级为4级。2例患者在术后5个月和7个月出现转移进展，2例在肾切除术后2个月和9个月出现淋巴侵犯。1例患者在随访期间死亡。
在我们的系列研究中，Xp11.2易位肾细胞癌在50岁以后并不常见，但可能被低估了。它似乎与预后不良有关。必须进行更大规模的研究以优化其特异性治疗。
