肾髓质癌1例报告及文献复习

Renal Medullary Carcinoma:a Case Report and Review of the Literature

目的:探讨起源于肾盏上皮的肾髓质癌,分析其临床,病理形态学,影像学,鉴别诊断及分子生物学特征,以提高该病的诊疗水平。方法:对1例肾髓质癌的诊治过程进行回顾性分析,结合国内外文献对本病的临床,病理学,影像学,鉴别诊断及分子生物学等特征以及治疗和预后进行总结分析。结果:本病好发于中青年,伴有镰状细胞病或镰状红细胞特征是其主要特点。与其他肾肿瘤相比影像学上没有明显的特异表现。CK、Vim和EMA均(+)有助于同肾集合管癌相鉴别。拓扑异构酶2α有望成为肾髓质癌治疗的新靶点。结论:肾髓质癌是一种少见的侵袭性恶性肿瘤,影像学上无特异性,确诊主要靠病理诊断,主要应与肾集合管癌鉴别。探索该病的新靶点,尽量早期诊断治疗是提高其疗效的关键。

Objective：To discuss the renal medullary carcinoma（RMC） which originated in the calyceal, ana lyse the characteristics of its clinical, pathological, imaging,differential diagnosis and molecular biology, and so as to improve the level of the diagnosis and treatment of the disease. Methods： A retrospective study was done in one case of RMC. Combing with the latest domestic and foreign literature , the clinical, pathology, imaging, differen- tial diagnosis ,molecular biology and other characteristics as well as treatment and prognosis were analyzed. Re- suits：The disease occurs most in the young, associating with the sickle-cell disease or trait as its main features. There is no significant specific performance in the imaging of RMC compared with the other renal tumors. Immu nohistochernically, the tumor cells were positive for CK, Vim, and EMA which helps to distinguish with the renal collecting duct carcinoma. Topoisomerase 2α is expected to become a new target for its therapy. Conclusions： Renal medullary carcinoma is a rare aggressive and malignant tumor which has no specific feature in imaging and depends mainly on pathological diagnosis , primarily identifies with the renal collecting duct carcinoma. Early diagnosis and treatment is the key to improve its efficacy , exploring a new target for the tumor also being crucial.