Loncarevic Ivan F, Hering Antje, Posorski Nicole, Linden Tobias, Hoyer Heike, Bucsky Peter
Institute for Human Genetics and Anthropology, Clinical University Hospital, Jena, Germany.
Pediatr Blood Cancer. 2008 Sep;51(3):356-62. doi: 10.1002/pbc.21603.
Adrenocortical tumours (ACT) in children are rare and, if malignant, often associated with poor prognosis. Relevant cytogenetic factors for prognosis are hardly available.
We analysed 14 adrenocortical cancers (ACC) of children by comparative genomic hybridisation (CGH).
The total number of genomic imbalances ranged from 1 to 17 in individual tumour samples. The most common imbalances were +1q (57%), +12p (50%), +12q (50%), +1p (43%), +7q (42%), +9q (42%), +15q (42%), and -4q (57%), -11q (57%), -4p (42%), and -16q (42%). The median number of genomic changes was 5.5 (n = 8) in pT1-pT2 and 15.5 (n = 6) in pT3-pT4 tumours. The median number was 4 in the eight patients, who remain in remission more than 51 months and 15.5 in the six patients, who have died from the disease within 44 months. Moreover, all seven patients with less than 10 individual imbalances were in remission (median follow-up 72 months), while all but one patient with 10 and more individual imbalances (n = 7) have died from the disease (median survival time 30 months). Comparison of the data from children and adults revealed characteristic differences. Gain of 1p and loss of 4p, 4q and 16q are frequent in childhood and rare in adults. Inversely, loss of 1p is rare in childhood but frequent in adult ACT.
The number of CGH imbalances appeared to have a predictive value for overall survival in paediatric ACC.
儿童肾上腺皮质肿瘤（ACT）较为罕见，若为恶性，通常预后较差。几乎没有可用于判断预后的相关细胞遗传学因素。
我们采用比较基因组杂交（CGH）技术分析了14例儿童肾上腺皮质癌（ACC）。
单个肿瘤样本中基因组失衡的总数在1至17之间。最常见的失衡为+1q（57%）、+12p（50%）、+12q（50%）、+1p（43%）、+7q（42%）、+9q（42%）、+15q（42%），以及-4q（57%）、-11q（57%）、-4p（42%）和-16q（42%）。pT1 - pT2肿瘤的基因组变化中位数为5.5（n = 8），pT3 - pT4肿瘤为15.5（n = 6）。8例缓解超过51个月的患者基因组变化中位数为4，6例在44个月内死于该病的患者为15.5。此外，所有7例个体失衡少于10个的患者均处于缓解状态（中位随访72个月），而7例个体失衡为10个及以上的患者中，除1例之外均死于该病（中位生存时间30个月）。儿童和成人数据的比较显示出特征性差异。1p增益以及4p、4q和16q缺失在儿童中常见，在成人中罕见。相反，1p缺失在儿童中罕见，但在成人ACT中常见。
CGH失衡数量似乎对儿童ACC的总生存具有预测价值。
