Schmidt D, Harms D, Lehner-Geisser M
Pathol Res Pract. 1984 Nov;179(2):242-9. doi: 10.1016/S0344-0338(84)80139-9.
Typical nephroblastoma (Wilms'tumor) is uncommon within the first 6 months of life. Renal tumors most commonly found in this age constitute of two groups which have a better and worse prognosis, respectively, than typical nephroblastomas. The group of tumors with a better prognosis encompasses congenital mesoblastic nephroma (CMN), fetal rhabdomyomatous nephroblastoma and cystic, partially differentiated nephroblastoma. The group of tumors with a worse prognosis consists of rhabdoid tumor of the kidney and bone metastasizing renal tumor (clear cell sarcoma) of childhood. Adequate therapy for these two neoplasms has as yet to be developed. Among the low-grade malignant tumors congenital mesoblastic nephroma can be successfully treated with simple nephrectomy. There, are however, variants of CMN which may differ in clinical behavior from the typical form of CMN. These variants include the cellular CMN2 and, possibly, the malignant mesenchymal nephroma of infancy. A case of cellular congenital mesoblastic nephroma is presented here. Its clinical and pathologic features are discussed.
典型肾母细胞瘤(威尔姆斯瘤)在出生后6个月内并不常见。这个年龄段最常见的肾肿瘤分为两组,其预后分别比典型肾母细胞瘤好和差。预后较好的肿瘤组包括先天性中胚层肾瘤(CMN)、胎儿横纹肌样肾母细胞瘤和囊性、部分分化的肾母细胞瘤。预后较差的肿瘤组包括肾横纹肌样瘤和儿童骨转移性肾肿瘤(透明细胞肉瘤)。针对这两种肿瘤的充分治疗方法尚未开发出来。在低级别恶性肿瘤中,先天性中胚层肾瘤可通过单纯肾切除术成功治疗。然而,CMN存在一些变体,其临床行为可能与典型的CMN形式不同。这些变体包括细胞性CMN2,以及可能的婴儿恶性间叶性肾瘤。本文介绍了一例细胞性先天性中胚层肾瘤病例,并讨论了其临床和病理特征。