Gonzalez-Crussi F, Sotelo-Avila C, Kidd J M
Hum Pathol. 1981 Jan;12(1):78-85. doi: 10.1016/s0046-8177(81)80244-4.
Eight cases of primary mesenchymal renal tumor in infants under one year of age were studied. The patients' ages ranged from one day to seven months; all but one were diagnosed within the first three months of life. There were four typical congenital mesoblastic nephromas. Two malignant mesenchymal, one intermediate case of difficult classification designated as "cellular variant" of congenital mesoblastic nephroma, and one hemangioendothelioma. All patients were alive and free of tumor 3 9/12 to 18 10/12 years after surgery. Of the four infants with congenital mesoblastic nephroma, one was treated by tumor excision alone; nearly 13 years later this patient was free of tumor. A high degree of cellularity and a high nucleus-cytoplasm ratio were features that characterized the tumor diagnosed as "cellular variant" of congenital mesoblastic nephroma. A sarcomatous gross appearance with cavitation necrosis was seen in the two instances of malignant mesenchymal nephroma; one of these metastasized to the lung, whereas in the other, though no metastases developed, all the histologic details of the previous case were reproduced, including distinct foci of necrosis and a high mitosis rate. Mesenchymal renal tumors in young infants constitute a set more heterogeneous than has been previously assumed. They should not be considered uniformly benign. As a group, they span the whole spectrum between benign, morphologically quiescent lesions, clinically and pathologically intermediate or indeterminate ones, and outright malignant tumors with a high risk of distant spread.
对8例1岁以下婴儿的原发性肾间叶肿瘤进行了研究。患者年龄从1天至7个月不等;除1例患者外,其余均在出生后的前3个月内确诊。其中有4例典型的先天性中胚层肾瘤。2例为恶性间叶肿瘤,1例分类困难的中间型病例被指定为先天性中胚层肾瘤的“细胞变异型”,还有1例为血管内皮瘤。所有患者术后3又9/12至18又10/12年仍存活且无肿瘤。4例先天性中胚层肾瘤患儿中,1例仅接受了肿瘤切除术;近13年后该患者无肿瘤。高细胞密度和高核质比是诊断为先天性中胚层肾瘤“细胞变异型”肿瘤的特征。2例恶性间叶肾瘤可见肉瘤样大体外观伴空洞性坏死;其中1例发生肺转移,而另1例虽未发生转移,但重现了前1例的所有组织学细节,包括明显的坏死灶和高有丝分裂率。幼儿肾间叶肿瘤构成的一组病变比以往认为的更为异质性。它们不应被一概视为良性。作为一个整体,它们涵盖了从良性、形态学上静止的病变,临床和病理上为中间型或不确定型的病变,到具有远处转移高风险的完全恶性肿瘤的整个范围。