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[先天性中胚层肾瘤伴多发囊肿]

[Congenital mesoblastic nephroma with multiple cysts].

作者信息

Hamzaoui Mourad, Essid Afif, Gasmi Manef, Ben Attia Moncef, Houissa Taoufik

机构信息

Service de Chirurgie Pédiatrique A, place Bab Saadoune, 1006 Tunisie.

出版信息

Prog Urol. 2003 Jun;13(3):466-9.

Abstract

Mesoblastic nephroma is a rare, benign congenital disease with a clinical presentation similar to that of Wilms tumour. It was described for the first time by BOLANDE in 1967. The authors report the case of a 6-month-old infant admitted for investigation of an abdominal mass. The radiological assessment (ultrasonography. CT) concluded on a right intrarenal retroperitoneal tumour occupying almost all of the abdominal cavity, predominantly cystic suggesting a diagnosis of non-metastatic Wilms tumour. Radical ureteronephrectomy was performed. The outcome was favourable with a follow-up of 2 years. Histological examination corrected the diagnosis to mesoblastic nephroma by showing a fibrous tumour composed of spindle cells resembling muscle cells, associated with intracystic haemorrhage. The authors emphasize the clinical and radiological features of this tumour, its treatment (exclusively surgical) and its good prognosis (98% survival).

摘要

中胚叶肾瘤是一种罕见的良性先天性疾病,临床表现与肾母细胞瘤相似。1967年由博兰德首次描述。作者报告了一名6个月大婴儿因腹部肿块入院检查的病例。影像学评估(超声、CT)结果显示右肾内腹膜后肿瘤几乎占据整个腹腔,以囊性为主,提示诊断为非转移性肾母细胞瘤。遂行根治性输尿管肾切除术。随访2年,结果良好。组织学检查显示为纤维性肿瘤,由类似肌肉细胞的梭形细胞组成,并伴有囊内出血,从而将诊断纠正为中胚叶肾瘤。作者强调了该肿瘤的临床和影像学特征、治疗方法(仅手术治疗)及其良好的预后(生存率98%)。

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