Rhabdoid tumour of the kidney: A clinicopathological study of 22 patients from the International Society of Paediatric Oncology (SIOP) nephroblastoma file
Rhabdoid tumour of the kidney: A clinicopathological study of 22 patients from the International Society of Paediatric Oncology (SIOP) nephroblastoma file
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DOI：
10.1046/j.1365-2559.1996.d01-436.x
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发表时间：
1996-04-01
期刊：
影响因子：
6.4
通讯作者：
Delemarre, JFM
中科院分区：
文献类型：
--
作者：
Vujanic, GM;Sandstedt, B;Delemarre, JFM
在2392例儿童肾肿瘤中，我们报告了22例(0.9%)肾横纹肌样肿瘤。患者年龄3周~94个月(中位数7个月)，男女之比为1.2：1。临床表现为腹部包块，4例(18%)患儿伴有高钙血症，1例(4.5%)发展为脑瘤(原始神经外胚叶瘤)。没有一名儿童出现I期疾病，5名(23%)有II期，10名(46%)III期，5名(23%)IV期疾病。两名患者(9%)有双侧肿瘤。组织学上，绝大多数(20/22)的肿瘤表现为经典类型，但也注意到其他组织类型。免疫组织化学染色：波形蛋白阳性12例，CAM5.2阳性8例，上皮膜抗原阳性6例，神经元特异性烯醇化酶阳性4例，S-100蛋白阳性8例，结蛋白阳性1例。在22个肿瘤中，只有12个在报告的病理学家和专家小组之间就肾脏横纹肌样肿瘤的诊断达成了一致。最初被诊断为肾脏横纹肌样瘤的8个肿瘤被发现是其他肾脏肿瘤，在另外10个病例中，专家小组将最初的诊断改为横纹肌样肿瘤。18名儿童(82%)发生转移--8名儿童在确诊时已有转移，10名儿童在初次诊断后2周至9个月内发生转移。肺、腹部、淋巴结、肝、骨和脑均有转移。在19名获得充分随访的儿童中，只有2名(10.5%)II期疾病患者存活，而17名(89.5%)儿童在确诊后2周至20个月死亡。
We present 22 (0.9%) cases of rhabdoid tumour of the kidney diagnosed amongst 2392 renal tumours in children. The patients ages ranged from 3 weeks to 94 months (median 7 months) and the female:male ratio was 1.2 : 1. Clinically, they presented with an abdominal mass but four (18%) children also had hypercalcaemia and one (4.5%) developed a brain tumour (primitive neuroectodermal tumour). None of the children presented with stage I disease, five (23%) had stage II, ten (46%) stage III, and five (23%) stage IV disease. Two (9%) patients had bilateral tumours. Histologically, the vast majority (20/22) of the tumours exhibited a classical pattern but other histological patterns were also noted. Immunohistochemical studies performed in 12 cases showed vimentin positivity in all cases, CAM 5.2 in eight, epithelial membrane antigen in six, neuron specific enolase in four, S-100 protein in eight, and desmin in one case. In only 12 of the 22 tumours was there agreement between the reporting pathologist and the panel on a diagnosis of rhabdoid tumour of the kidney. Eight tumours originally diagnosed as rhabdoid tumour of the kidney were found to be other renal tumours and in another ten cases the initial diagnosis was changed by the panel to rhabdoid tumour. Metastases developed in 18 (82%) of the children-in eight they were present at the time of diagnosis and in 10 they developed from 2 weeks to 9 months after initial diagnosis. Metastases were found in the lung, abdomen, lymph nodes, liver, bone and brain. Of the 19 children with adequate follow-up, only two (10.5%) with stage II disease are alive, while 17 (89.5%) died 2 weeks to 20 months after the diagnosis.