Clinical Genetics Branch, Division of Cancer Epidemiology and Genetics, National Cancer Institute, Rockville, MA 20852, USA.
Fam Cancer. 2009;8(4):451-6. doi: 10.1007/s10689-009-9264-6. Epub 2009 Jul 17.
One of the clinical hallmarks of hereditary cancer susceptibility disorders is a younger-than-usual age at diagnosis. Familial aggregation of testicular germ cell tumor (TGCT) has been reported, but data on whether familial TGCT cases are diagnosed at an earlier age are inconclusive. Here we compared the age at diagnosis of familial TGCT cases with that of population cases in several countries. Familial TGCT is defined as affected individuals from families with >or=2 cases of TGCT. Age at diagnosis of familial cases from the United States, Canada, United Kingdom, Australia and New Zealand, Norway, and Hungary was compared to cases identified in population-based cancer registries from the respective country, using the generalized estimation equation method. Age at diagnosis was statistically significantly younger for familial TGCT cases from North America (P = 0.024), the United Kingdom (P < 0.0001), and Australia and New Zealand (P = 0.0033) compared with population cases. When stratified by histology, the difference in age at diagnosis distribution between familial and population cases was observed for seminoma cases from North America (P = 0.002) and the United Kingdom (P < 0.0001) and non-seminoma cases from the United Kingdom (P = 0.029) and Australia and New Zealand (P = 0.0023). In summary, we found that the age at diagnosis for familial TGCT cases is, on the average, 2-3 years younger than that for the population cases in North America, United Kingdom, and Australia and New Zealand. The younger age at diagnosis might be suggestive of a genetic basis for familial TGCT.
遗传性癌症易感性疾病的临床特征之一是诊断年龄较年轻。已经报道了睾丸生殖细胞肿瘤 (TGCT) 的家族聚集，但关于家族性 TGCT 病例是否更早诊断的数据尚无定论。在这里，我们比较了几个国家的家族性 TGCT 病例和人群病例的诊断年龄。家族性 TGCT 定义为来自 TGCT 病例>或=2 例的家族中的受影响个体。使用广义估计方程方法，将来自美国、加拿大、英国、澳大利亚和新西兰、挪威和匈牙利的家族性 TGCT 病例的诊断年龄与各自国家基于人群的癌症登记处确定的病例进行比较。与人群病例相比，北美 (P = 0.024)、英国 (P < 0.0001) 和澳大利亚和新西兰 (P = 0.0033) 的家族性 TGCT 病例的诊断年龄明显更年轻。按组织学分层，在北美 (P = 0.002) 和英国 (P < 0.0001) 的精原细胞瘤病例以及英国 (P = 0.029) 和澳大利亚和新西兰 (P = 0.0023) 的非精原细胞瘤病例中，观察到家族性和人群病例之间诊断年龄分布的差异。总之，我们发现家族性 TGCT 病例的诊断年龄平均比北美的人群病例年轻 2-3 岁，英国和澳大利亚和新西兰的人群病例年轻。更早的诊断年龄可能提示家族性 TGCT 的遗传基础。
