CAJ | 학술논문

目的：探讨起源于肾盏及肾乳头上皮细胞的恶性肿瘤肾髓质癌，分析其流行病学、临床、病理、影像、鉴别诊断及分子生物学特征，以提高对该病的认识、诊疗水平和预后。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诊断、治疗和预后进行总结分析。结果：本病好发于伴有镰状红细胞特征(sickle cell trait，SCT)或镰状细胞血红蛋白病(sickle cell disease，SCD)的青少年。与其他肾肿瘤相比影像学上没有明显的特异表现。Cam5.2、CK、Vim和EMA均(+)有助于同肾集合管癌等相鉴别。分子靶向治疗有望成为提高肾髓质癌预后的突破方向。结论：髓质癌是一种罕见的强侵袭性恶性肿瘤，影像学上无特异性，确诊主要靠病理组织学诊断，肾集合管癌等应与其相鉴别。探索该病新的治疗靶点，提高患者预后，但早期诊断和疾病意识增强仍是提高其疗效的关键。
목적：탐토기원우신잔급신유두상피세포적악성종류신수질암，분석기류행병학、림상、병리、영상、감별진단급분자생물학특정，이제고대해병적인식、진료수평화예후。방법：대1례신수질암적진치과정진행회고성분석。결합국내외문헌대본병적진단、치료화예후진행총결분석。결과：본병호발우반유렴상홍세포특정(sickle cell trait，SCT)혹렴상세포혈홍단백병(sickle cell disease，SCD)적청소년。여기타신종류상비영상학상몰유명현적특이표현。Cam5.2、CK、Vim화EMA균(+)유조우동신집합관암등상감별。분자파향치료유망성위제고신수질암예후적돌파방향。결론：수질암시일충한견적강침습성악성종류，영상학상무특이성，학진주요고병리조직학진단，신집합관암등응여기상감별。탐색해병신적치료파점，제고환자예후，단조기진단화질병의식증강잉시제고기료효적관건。
Objective:To discuss the renal medullary carcinoma which originatesd in the calyceal or the epithelial papillary cells, analyse the characteristics of its epidemiology, clinical, pathological, imaging, differential diagnosis and molecular biology. And so as to improve the level of the recognize, diagnosis, treatment and prognosis of the disease. Methods:A retrospective study was done in one case of RMC. Combing with the latest domestic and foreign literature, the diagnosis as well as treatment and prognosis were analyzed. Results:hTe disease associating with the sickle-cell disease or trait as its main features primarily affects the young. hTere is no signiifcant speciifc performance in the imaging of RMC compared with the other renal tumors. Immunohisto chemical studies, such as the tumor cells were positive for Cam5.2, CK, Vim, and EMA, can be helpful in distinguishing renal medullary carcinoma from other poorly differentiated kidney tumors, except for collecting duct carcinoma. hTe molecular and genetic factors implicated with this rare disease is progressively opening doors to promising targeted therapies. Conclusion:Renal medullary carcinoma is a rare aggressive and malignant tumor which has no speciifc feature in imaging and depends mainly on pathological diagnosis, primarily identifies with the renal collecting duct carcinoma. hTe recognize of disease, early diagnosis and treatment are the key to improve its effcacy, exploring a new target for the tumor also being urgent.