Mathew P, Douglass E C, Jones D, Valentine M, Valentine V, Rowe S, Shapiro D N
Department of Experimental Oncology, St. Jude Children's Research Hospital, Memphis, TN 38105, USA.
Med Pediatr Oncol. 1996 Jul;27(1):3-7. doi: 10.1002/(SICI)1096-911X(199607)27:1<3::AID-MPO2>3.0.CO;2-K.
The der(16)t(1;16)(q21;q13) chromosomal abnormality has been reported rarely in Wilms' tumor. This abnormality has also been found in several other pediatric and adult neoplasms, and may imply a poor prognosis in at least some of these solid tumors. To investigate its clinical significance in Wilms' tumor, we examined the records of 65 consecutive children with Wilms' tumor whose tumor cells were successfully karyotyped. The t(1;16) was present in seven patients (10%) whose ages ranged from 2.5 to 4.7 years (median 3.5 years) at diagnosis. Six of the seven patients were female. All four stages of Wilms' tumor were represented (two patients had stage IV disease). No patient had bilateral disease. All tumors were of "favorable histology." All seven patients are alive and off therapy with a median follow-up of 3.2 years (range, 2 to 8.5 years). One patient with this abnormality developed brain metastases within 4 months of completion of therapy. Comparison of these patients with the remaining 58 Wilms' tumor patients revealed no significant differences with regard to disease stage, histology, survival, or relapse-free survival. Cytogenetic evidence of der(16)t(1;16)(q21;q13) in Wilms' tumor does not appear to portend an adverse clinical outcome, although only a larger study that includes molecular detection methods can provide more conclusive evidence.
16号衍生染色体t(1;16)(q21;q13)染色体异常在肾母细胞瘤中鲜有报道。这种异常也在其他几种儿童和成人均肿瘤中被发现，并且在至少部分这些实体瘤中可能预示预后不良。为了研究其在肾母细胞瘤中的临床意义，我们检查了65例连续的肾母细胞瘤患儿的记录，这些患儿的肿瘤细胞成功进行了核型分析。t(1;16)存在于7例患者（10%）中，诊断时年龄范围为2.5至4.7岁（中位年龄3.5岁）。7例患者中有6例为女性。肾母细胞瘤的所有四个分期均有代表（2例患者为IV期疾病）。没有患者有双侧疾病。所有肿瘤均为“预后良好的组织学类型”。所有7例患者均存活且停止治疗，中位随访时间为3.2年（范围为2至8.5年）。1例有这种异常的患者在治疗完成后4个月内发生脑转移。将这些患者与其余58例肾母细胞瘤患者进行比较，发现在疾病分期、组织学、生存率或无复发生存率方面没有显著差异。肾母细胞瘤中der(16)t(1;16)(q21;q13)的细胞遗传学证据似乎并不预示不良临床结局，尽管只有一项包括分子检测方法的更大规模研究才能提供更确凿的证据。
