Picado Omar, Ferrantella Anthony, Zabalo Celisse, Rao Krishnamurti, Thorson Chad M, Sola Juan E, Perez Eduardo A
Division of Pediatric Surgery, DeWitt Daughtry Family Department of Surgery, University of Miami Leonard M. Miller School of Medicine, 1120 NW 14th Street, Suite 450K, Miami, FL, 33136, USA.
Pediatr Surg Int. 2020 Mar;36(3):357-363. doi: 10.1007/s00383-020-04617-z. Epub 2020 Jan 27.
Pancreatic tumors are rare in children and limited data are available regarding incidence, treatment, and outcomes. We aim to describe patient and tumor characteristics and to report on survival of these diseases.
Children with pancreatic tumors were queried from the National Cancer Database (2004-2014). The association between treatment and hazard of death was assessed using Kaplan-Meier method and Cox regression model.
We identified 109 children with pancreatic tumors; 52% were male and median age at diagnosis was 14 years. Tumors were distributed as follows: pseudopapillary neoplasm (30%), endocrine tumors (27%), pancreatoblastoma (16%), pancreatic adenocarcinoma (16%), sarcoma (6%) and neuroblastoma (5%). Seventy-nine patients underwent surgery, of which 76% achieved R0 resection. Most patients (85%) had lymph nodes examined, of which 22% had positive nodes. Five-year overall survival by tumor histology was 95% (pseudopapillary neoplasm), 75% (neuroblastoma), 70% (pancreatoblastoma), 51% (endocrine tumors), 43% (sarcoma), and 34% (adenocarcinoma). On multivariable analysis, surgical resection was the strongest predictor of survival (HR 0.26, 95% CI 0.10-0.68, p < 0.01).
Overall survival of children with pancreatic tumors is grim, with varying survival rates among different tumors. Surgical resection is associated with improved long-term survival.
胰腺肿瘤在儿童中较为罕见，关于其发病率、治疗方法及预后的数据有限。我们旨在描述患者和肿瘤特征，并报告这些疾病的生存情况。
从国家癌症数据库（2004 - 2014年）中查询患有胰腺肿瘤的儿童。使用Kaplan - Meier方法和Cox回归模型评估治疗与死亡风险之间的关联。
我们确定了109例患有胰腺肿瘤的儿童；52%为男性，诊断时的中位年龄为14岁。肿瘤分布如下：实性假乳头状肿瘤（30%）、内分泌肿瘤（27%）、胰腺母细胞瘤（16%）、胰腺腺癌（16%）、肉瘤（6%）和成神经细胞瘤（5%）。79例患者接受了手术，其中76%实现了R0切除。大多数患者（85%）接受了淋巴结检查，其中22%有阳性淋巴结。按肿瘤组织学分类的5年总生存率分别为：95%（实性假乳头状肿瘤）、75%（成神经细胞瘤）、70%（胰腺母细胞瘤）、51%（内分泌肿瘤）、43%（肉瘤）和34%（腺癌）。多变量分析显示，手术切除是生存的最强预测因素（HR 0.26，95%CI 0.10 - 0.68，p < 0.01）。
胰腺肿瘤患儿的总体生存情况严峻，不同肿瘤的生存率各不相同。手术切除与改善长期生存相关。
