Multilocular cystic renal tumor in children: radiologic-pathologic correlation.
Multilocular cystic renal tumor in children: radiologic-pathologic correlation.
复制标题
儿童多房性囊性肾肿瘤：放射学-病理学相关性。
作者：
G. A. Agrons;B. Wagner;A. J. Davidson;E. S. Suárez
多房囊性肾肿瘤是一个术语，包括两种组织学上不同但大体难以区分的病变：囊性肾瘤和囊性部分分化肾母细胞瘤（CPDN）。囊性肾瘤是一种节段性的纯囊性肿块，其特征是完全由分化组织组成的多分隔，没有胚质成分。CPDN也是一种多室病变，无结节性实性成分，但其间隔含有胚胎细胞。多房囊性肿瘤主要影响儿童早期的男孩，大量病变含有囊胚（CPDN），而成年女性的病变更常见的是缺乏间隔囊胚（囊性）肾瘤。通常，肾切除术是治愈的，临床过程是良性的，但CPDN可能局部复发。尽管囊性肾瘤和CPDN不能通过放射学来区分，但由于所有这些肿瘤都是通过手术切除的，因此不能这样做对治疗没有实际影响。然而，鉴别诊断包括其他可能需要不同治疗策略的儿童囊性肾肿块：肾母细胞瘤伴因出血和坏死而形成的囊肿，囊性透明细胞肉瘤，囊性间质肾瘤，囊性肾细胞癌，多囊性发育不良肾，以及复制肾收集系统的节段性多囊性发育不良。
Multilocular cystic renal tumor is a term that encompasses two histologically distinct but grossly indistinguishable lesions: cystic nephroma and cystic partially differentiated nephroblastoma (CPDN). Cystic nephroma is a segmental, purely cystic mass characterized by multiple septations composed entirely of differentiated tissues, without blastemal elements. CPDN is also a multiloculated lesion without nodular solid components, but its septa contain embryonal cells. Multilocular cystic tumors primarily affect boys during early childhood, with a substantial number of the lesions containing blastema (CPDN), and adult women, with lesions that more commonly lack septal blastema (cystic) nephroma). As a rule, nephrectomy is curative and the clinical course benign, but CPDN may recur locally. Although cystic nephroma and CPDN cannot be distinguished radiologically, failure to do so has no practical impact on management, since all of these tumors are surgically removed. However, the differential diagnosis includes other pediatric cystic renal masses that may require different treatment stratagems: Wilms tumor with cyst formation due to hemorrhage and necrosis, cystic clear cell sarcoma, cystic mesoblastic nephroma, cystic renal cell carcinoma, multicystic dysplastic kidney, and segmental multicystic dysplasia in a duplicated renal collecting system.