Agrons G A, Wagner B J, Davidson A J, Suarez E S
Department of Radiologic Pathology, Armed Forces Institute of Pathology, Washington, DC 20306-6000, USA.
Radiographics. 1995 May;15(3):653-69. doi: 10.1148/radiographics.15.3.7624570.
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.
囊性肾瘤和囊性部分分化型肾母细胞瘤（CPDN）。囊性肾瘤是一种节段性的、纯粹的囊性肿块，其特征是多个分隔完全由分化组织组成，无胚芽成分。CPDN也是一种多房性病变，无结节状实性成分，但其分隔含有胚胎细胞。多房性囊性肿瘤主要影响幼儿期的男孩，其中大量病变含有胚芽（CPDN），以及成年女性，其病变更常见缺乏分隔胚芽（囊性）肾瘤）。通常，肾切除术可治愈，临床病程为良性，但CPDN可能局部复发。虽然囊性肾瘤和CPDN在影像学上无法区分，但未能区分对治疗并无实际影响，因为所有这些肿瘤均需手术切除。然而，鉴别诊断还包括其他可能需要不同治疗策略的小儿囊性肾肿块：因出血和坏死形成囊肿的威尔姆斯瘤、囊性透明细胞肉瘤、囊性中胚层肾瘤、囊性肾细胞癌、多囊性发育不良肾，以及重复肾集合系统中的节段性多囊性发育不良。
