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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,以提高本病的诊疗水平。方法:对最近收治的1例肾髓质癌患者的诊治过程进行回顾性分析,结合国内外最新的文献对本病的临床、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(Sickle cell disease,SCD)或镰状红细胞特征是其主要特点。肿瘤生长于肾髓质内;瘤细胞呈片块状弥漫分布,网状或巢状排列,间质明显纤维化及许多中性粒细胞浸润形成微脓肿是其特征性的变化。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相鉴别。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 investigate the renal medullary carcinoma originating from the calyceal epithelium and to analyze its clinical, pathological, imaging, differential diagnosis and molecular biological characteristics in order to improve the diagnosis and treatment of this disease. Methods: A retrospective analysis was made on the diagnosis and treatment of 1 case of newly diagnosed renal-medullary carcinoma patients. Combined with the latest literature both at home and abroad, the clinical, pathological, imaging, differential diagnosis and molecular biology of the disease were reviewed. Prognosis summary analysis. Results: The disease occurs in middle-aged and young people. The main features of Sickle cell disease (SCD) or sickle cell are its characteristics. Tumors grew in the medulla; tumor cells were diffuse patches, reticular or nested arrangement, interstitial fibrosis and many neutrophils infiltrating the formation of micro abscess is its characteristic changes. Compared with other renal tumors, no significant radiological manifestations. CK, Vim and EMA are +, contribute to the differential diagnosis of renal tubular cancer. Topoisomerase 2α is expected to become a new target for the treatment of renal medullary carcinoma. Conclusion: Medullary carcinoma of the kidney is a rare invasive malignant tumor, which is nonspecific in imaging studies. The diagnosis mainly depends on the pathological diagnosis, and should mainly be differentiated from renal tubules. Explore the new target of the disease, try to early diagnosis and treatment, is to improve the efficacy of the key.