肾Bellini集合管癌的诊断和治疗

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目的:探讨肾Bellini集合管癌(collecting duct carcinoma,CDC)的临床特点、诊疗及预后。方法:回顾性分析3例CDC患者的临床资料,并检索PubMed等相关文献进行复习。结果:3例CDC患者男2例,女1例,年龄37~78岁。因乏力、体重下降就诊1例,体检发现2例。1例患者行两次肿瘤穿刺病理诊断为肾脏透明细胞癌。1例行腹腔镜肾癌根治术,1例开放根治性肾切除术加肾门淋巴结切除,1例因肾门部淋巴结包裹腹主动脉并伴有广泛腹膜后淋巴结转移行姑息性肾切除术。3例均为CDC。1例术前诊断为肾透明细胞癌者,术后病理为CDC并灶状肾透明细胞癌;1例为CDC并部分乳头状肾细胞癌。3例患者中2例目前为术后1个月,仍存活。1例随访21个月,无肿瘤复发和转移。结论:CDC少见,就诊时通常为高分期、高分级,疾病恶性程度高,进展快,预后差。根治性肾切除是主要治疗方法,术后可辅以化疗和靶向治疗。 Objective: To investigate the clinical features, diagnosis, treatment and prognosis of renal ductini collecting duct carcinoma (CDC). Methods: The clinical data of 3 patients with CDC were retrospectively analyzed. Relevant literature such as PubMed was searched for review. Results: There were 2 males and 1 females in 3 CDC patients, ranging in age from 37 to 78 years. Due to fatigue, weight loss treatment in 1 case, physical examination found 2 cases. One patient underwent tumor puncture pathological diagnosis of clear cell renal cell carcinoma. One case underwent laparoscopic radical nephrectomy, one case underwent radical nephrectomy plus nephronectomy, and one case underwent nephrotic parietal nephroureterectomy with extensive peritoneal lymph node metastases. 3 cases were all CDC. One case was diagnosed as renal clear cell carcinoma preoperatively, the pathology was CDC with focal clear cell renal cell carcinoma, and one case was CDC with some papillary renal cell carcinoma. Two of the three patients are currently alive 1 month after surgery. One case was followed up for 21 months without tumor recurrence and metastasis. CONCLUSIONS: CDC is rare and often presents with high grade, high grade, high degree of malignancy, rapid progression and poor prognosis. Radical nephrectomy is the main treatment, postoperative adjuvant chemotherapy and targeted therapy.
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