Kadan-Lottick Nina S, Kawashima Toana, Tomlinson Gail, Friedman Debra L, Yasui Yutaka, Mertens Ann C, Robison Leslie L, Strong Louise C
Yale University, New Haven, Connecticut 06520, USA.
Pediatr Blood Cancer. 2006 Apr;46(4):476-81. doi: 10.1002/pbc.20465.
Twin concordance studies help evaluate the contribution of genetic factors in childhood cancers, but previous reports have primarily focused on leukemia because of the rarity of other malignancies. In the current report, a large cohort of childhood cancer survivors was used to: (1) describe twin concordance patterns for a range of cancers, (2) calculate the standardized incidence rates of cancers in twins, and (3) describe clinical features and outcomes of concordant twins.
Cancer family history was obtained on the 211 twins participating in the Childhood Cancer Survivor Study (CCSS) (14,352 participants surviving > or =5 years after a malignancy diagnosed at <21 years during January 1, 1970-December 31, 1986) to calculate probandwise twin concordance rates and standardized incidence ratios (SIRs) using Surveillance, Epidemiology, and End-Results data.
Seven monozygotic twin pairs were concordant for cancer (six for leukemia, one for non-Hodgkin lymphoma), yielding probandwise concordance rates of 9.5%, 20.7%, and 20.0% for all cancer, leukemia, and non-Hodgkin lymphoma (NHL), respectively. No concordance was observed among dizygotic twins or for dissimilar cancers. The SIR in monozygotic twins was 23.3 (95% CI = 11.1-48.9) for all cancer, 112.4 (95% CI = 50.5-250.1) for leukemia, and 40.5 (5.7-287.5) for NHL. Concordant twins were similar in age at diagnosis and vital status.
Twin concordance for cancer is largely restricted to monozygotic twins and hematological malignancies, consistent with in utero malignancy transmission demonstrated by others. Our data support clinical monitoring of the twins of cases with hematological malignancies, and does not contribute evidence for genetic factors in other cancers.
双胞胎一致性研究有助于评估遗传因素在儿童癌症中的作用，但由于其他恶性肿瘤较为罕见，以往的报告主要集中在白血病上。在本报告中，我们使用了一大群儿童癌症幸存者来：（1）描述一系列癌症的双胞胎一致性模式，（2）计算双胞胎中癌症的标准化发病率，以及（3）描述一致性双胞胎的临床特征和结局。
获取了参与儿童癌症幸存者研究（CCSS）的211对双胞胎的癌症家族史（14352名参与者在1970年1月1日至1986年12月31日期间在21岁之前被诊断为恶性肿瘤后存活≥5年），以使用监测、流行病学和最终结果数据计算先证者双胞胎一致性率和标准化发病率比（SIRs）。
7对同卵双胞胎患癌一致（6对患白血病，1对患非霍奇金淋巴瘤），所有癌症、白血病和非霍奇金淋巴瘤（NHL）的先证者一致性率分别为9.5%、20.7%和20.0%。在异卵双胞胎或不同癌症之间未观察到一致性。同卵双胞胎中所有癌症的SIR为23.3（95%CI = 11.1 - 48.9），白血病为112.4（95%CI = 50.5 - 250.1），NHL为40.5（5.7 - 287.5）。一致性双胞胎在诊断时的年龄和生存状态相似。
癌症的双胞胎一致性主要限于同卵双胞胎和血液系统恶性肿瘤，这与其他人所证明的子宫内恶性肿瘤传播一致。我们的数据支持对血液系统恶性肿瘤病例的双胞胎进行临床监测，并且没有为其他癌症中的遗传因素提供证据。
