Leuschner I, Harms D, Schmidt D
Abt. Paidopathologie, Universität Kiel, Federal Republic of Germany.
Med Pediatr Oncol. 1991;19(1):33-41. doi: 10.1002/mpo.2950190107.
Ten renal cell carcinomas in children under 15 years were investigated. The average age was 122.5 months and the girls predominated in our cases (7 girls, 3 boys). By using the classification of Thoenes et al., Pathol Res Pract 181: 125-143, 1986 a predominance of clear cell-eosinophilic tumor cell type and of the tubulopapillary growth pattern was found. Immunohistochemistry revealed a heterogeneity of cytokeratin expression. By using the monoclonal antibodies Cam 5.2 and KL 1, cytokeratins were found in 7 cases each. The other 4 cytokeratin antibodies used were less sensitive. The expression of cytokeratin 13 in 3 cases suggested a more complex histogenesis than assumed. Vimentin was found in 3 tumors, but an association to a higher grade (G) of malignancy was not found in these cases. One tumor expressed the Tamm-Horsfall-protein, which is predominantly found in the distal tubule of the normal kidney. In summary the results of immunohistochemistry characterized the great heterogeneity of these tumors. Follow-up information was available in 9 cases. All patients with G I- and G II-tumors were free of disease after an average time of 39.6 months (mean 27 months). Two of the 3 cases with G III-tumors died after 9 and 15 months, despite additional chemo- or radiotherapy. Therefore tumors of grade I and II of the Thoenes classification seem to have a good prognosis.
对15岁以下儿童的10例肾细胞癌进行了研究。平均年龄为122.5个月，在我们的病例中女孩占多数（7名女孩，3名男孩）。采用托内斯等人（《病理学研究与实践》181:125 - 143,1986）的分类方法，发现透明细胞 - 嗜酸性肿瘤细胞类型和管状乳头样生长模式占优势。免疫组织化学显示细胞角蛋白表达具有异质性。使用单克隆抗体Cam 5.2和KL 1时，在7例病例中均发现了细胞角蛋白。所使用的其他4种细胞角蛋白抗体敏感性较低。3例中细胞角蛋白13的表达提示其组织发生比预期更复杂。在3个肿瘤中发现了波形蛋白，但在这些病例中未发现与更高恶性等级（G）相关。1个肿瘤表达了Tamm - Horsfall蛋白，该蛋白主要存在于正常肾脏的远端小管中。总之，免疫组织化学结果表明这些肿瘤具有很大的异质性。9例有随访信息。所有I级和II级肿瘤患者在平均39.6个月（平均27个月）后均无疾病。3例III级肿瘤患者中有2例在9个月和15个月后死亡，尽管进行了额外的化疗或放疗。因此，托内斯分类中的I级和II级肿瘤似乎预后良好。
