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囊性部分分化型肾母细胞瘤的部分肾切除术

Partial nephrectomy in a cystic partially differentiated nephroblastoma.

作者信息

Streif W, Gassner I, Janetschek G, Kreczy A, Judmaier W, Fink F M

机构信息

Department of Pediatrics, University of Innsbruck, Austria.

出版信息

Med Pediatr Oncol. 1997 Jun;28(6):416-9. doi: 10.1002/(sici)1096-911x(199706)28:6<416::aid-mpo4>3.0.co;2-j.

Abstract

Cystic partially differentiated nephroblastoma (CPDN) is a rare neoplastic disorder consisting of a well-demarcated cystic lesion of the kidney where blastemal or other embryonic cells are present in the septa of the cysts. Magnetic resonance imaging can detect the cystic character of the lesion and will produce imaging features that are highly suggestive of either CPDN or cystic nephroma (CN) (synonym: multilocular cyst of the kidney), a benign entity. Although malignant potential exists in CPDN, all cases reported to date have had a favorable prognosis after surgery alone. Partial nephrectomy is considered safe, and the treatment of choice in the newborn period. We report a case of CPDN in a newborn that was successfully treated with partial nephrectomy. More than five years after nephron sparing surgery, the involved kidney shows normal anatomical structure except for a diminished upper pole, no evidence of tumor recurrence and good renal function.

摘要

囊性部分分化型肾母细胞瘤(CPDN)是一种罕见的肿瘤性疾病,由肾脏界限清晰的囊性病变组成,囊肿间隔中存在胚基或其他胚胎细胞。磁共振成像可检测病变的囊性特征,并产生高度提示CPDN或囊性肾瘤(CN)(同义词:肾多房囊肿,一种良性实体)的影像学特征。虽然CPDN存在恶性潜能,但迄今为止报道的所有病例仅手术治疗后预后良好。部分肾切除术被认为是安全的,是新生儿期的首选治疗方法。我们报告了1例成功接受部分肾切除术治疗的新生儿CPDN病例。保留肾单位手术后五年多,患肾除上极缩小外,解剖结构正常,无肿瘤复发迹象,肾功能良好。

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