Factors associated with survival in pediatric adrenocortical carcinoma: An analysis of the National Cancer Data Base (NCDB)
Factors associated with survival in pediatric adrenocortical carcinoma: An analysis of the National Cancer Data Base (NCDB)
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DOI：
10.1016/j.jpedsurg.2015.10.039
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发表时间：
2016-01-01
影响因子：
2.4
通讯作者：
Tracy, Elisabeth T.
中科院分区：
文献类型：
--
作者：
Gulack, Brian C.;Rialon, Kristy L.;Tracy, Elisabeth T.
目的：肾上腺皮质癌（ACC）是一种罕见的儿童肿瘤，与成人疾病有重要区别。我们查阅了国家癌症数据库 (NCDB)，以确定与长期生存相关的因素。方法：查询 NCDB 中 1998 年至 2011 年间诊断为 ACC 的 18 岁以下患者。利用 Kaplan-Meier 分析确定与总生存显着相关的因素。结果：总共纳入 111 名患者（中位年龄：4 岁，69% 女性）。 ACC 在最年轻的队列中更为常见，48% 的病例发生在 3 岁以下的儿童中。中位肿瘤大小为 9.5 厘米（IQR：6.5-13.0），87% 的患者接受了某种形式的手术切除。在有可用数据的儿童中，62 名儿童中有 19 名出现转移。总体 1 年和 3 年生存率分别为 70% 和 64%。年龄、肿瘤大小、肿瘤向周围组织的扩展以及转移性疾病都被发现与生存显着相关。在接受外科手术的患者中，边缘状态也被发现与生存显着相关。结论：年龄、肿瘤大小、肿瘤扩展、转移性疾病和边缘状态与肾上腺皮质癌儿童的长期生存显着相关。 (C) 2016 Elsevier Inc. 保留所有权利。
Purpose: Adrenocortical carcinoma (ACC) is a rare tumor in children with important distinctions from the adult disease. We reviewed the National Cancer Data Base (NCDB) to determine factors associated with long-term survival.Methods: The NCDB was queried for patients less than 18 years of age who were diagnosed with ACC between 1998 and 2011. Kaplan-Meier analysis was utilized to determine factors significantly associated with overall survival.Results: A total of 111 patients were included (median age: 4 years, 69% female). ACC was more common in the youngest cohort, with 48% of cases occurring in children younger than the age of 3. Median tumor size was 9.5 cm(IQR: 6.5-13.0), and 87% of patients underwent some form of surgical resection. Among children with available data, 19 of 62 presented with metastases. Overall 1- and 3-year survival was 70% and 64%, respectively. Age, tumor size, extension of tumor into surrounding tissue, and metastatic disease were all found to be significantly associated with survival. Among patients who underwent a surgical procedure, margin status was also found to be significantly associated with survival.Conclusion: Age, tumor size, extension of tumor, metastatic disease, and margin status are significantly associated with long-term survival in children with adrenocortical carcinoma. (C) 2016 Elsevier Inc. All rights reserved.