Huang YiJin, Yang Wei, Hu JiaJian, Zhu ZhiYun, Qin Hong, Han Wei, Wang HuanMin
Division of Pediatric Oncological Surgery, Department of Pediatrics, Beijing Children's Hospital, Capital Medical University, Beijing, China.
Pediatr Surg Int. 2019 Nov;35(11):1231-1238. doi: 10.1007/s00383-019-04524-y. Epub 2019 Jul 23.
Pancreatoblastoma is a very rare malignant pancreatic tumor in children. Pancreatoblastoma is the most common pancreatic tumor in children less than 10 years of age, accounting for 25% of the pancreatic neoplasm. There were only a few published literatures about the standardized diagnostic and management protocol for PB in the last decade.
To summarize our experience in the management of pancreatoblastoma in children and adolescents with emphasis on the presentation, diagnosis, treatment, and outcomes. A management strategy will also be discussed.
This was a retrospective case-series study of all pancreatoblastoma in patients < 18 years of age who were treated at Beijing children's hospital (BCH) from January 2002-January 2015. The diagnoses of PB were confirmed by histopathology analysis of the resected specimen. The variables being analyzed included patient demographics, age at diagnosis, clinical presentation, tumor size, metastasis if present, tumor markers (AFP), type of surgery, length of follow-up, and outcome. The assessment of the tumor location, size, extent of the tumor, and distant metastasis was made by ultrasound (US), computed tomography (CT), and/or magnetic resonance imaging (MRI).
21 patients with pancreatoblastoma were diagnosed at a median age of 4 years, 7 girls, and 14 boys. The diagnosis of pancreatoblastoma was identified by the histology examination. The most common syndrome was abdominal mass (n = 11), followed by abdominal pain (N = 10), elevated serum AFP levels were noted in almost all cases (17/18), 17 patients with disease initially unresectable on diagnosis accepted neo-adjuvant chemotherapy consisting of CDV, OPEC, PLADO, IEV, and AVCP. All patients underwent surgery, including pancreaticoduodenectomy (Whipple's procedure), the Pylorus-preserving pancreaticoduodenectomy (traverse-Longmire procedure), Spleen-preserving distal pancreatectomy, and distal pancreatectomy with en bloc splenectomy, Roux-en-Y end-to-end pancreatojejunostomy. In all, 13 children were disease free with a median follow-up of 53 months (range 11-156 months).
The pancreatoblastoma in children and adolescents is a malignant tumor. Complete resection combined with chemotherapy is associated with long-term survival. For the unresectable tumor at diagnosis, preoperative chemotherapy was recommended to reduce tumor volume. AFP is critical for diagnosis and monitoring the disease as a tumors marker.
胰腺母细胞瘤是儿童中一种非常罕见的胰腺恶性肿瘤。胰腺母细胞瘤是10岁以下儿童中最常见的胰腺肿瘤，占胰腺肿瘤的25%。在过去十年中，关于胰腺母细胞瘤标准化诊断和管理方案的已发表文献仅有少数几篇。
总结我们在儿童和青少年胰腺母细胞瘤管理方面的经验，重点关注临床表现、诊断、治疗和结果。还将讨论一种管理策略。
这是一项回顾性病例系列研究，研究对象为2002年1月至2015年1月在北京儿童医院（BCH）接受治疗的所有18岁以下胰腺母细胞瘤患者。通过对切除标本的组织病理学分析确诊胰腺母细胞瘤。分析的变量包括患者人口统计学特征、诊断时年龄、临床表现、肿瘤大小、是否存在转移、肿瘤标志物（甲胎蛋白）、手术类型、随访时间和结果。通过超声（US）、计算机断层扫描（CT）和/或磁共振成像（MRI）对肿瘤位置、大小、肿瘤范围和远处转移进行评估。
21例胰腺母细胞瘤患者确诊时的中位年龄为4岁，其中7例为女孩，14例为男孩。通过组织学检查确诊胰腺母细胞瘤。最常见的症状是腹部肿块（n = 11），其次是腹痛（n = 10），几乎所有病例（17/18）血清甲胎蛋白水平均升高，17例诊断时最初无法切除的患者接受了由CDV、OPEC、PLADO、IEV和AVCP组成的新辅助化疗。所有患者均接受了手术，包括胰十二指肠切除术（惠普尔手术）、保留幽门的胰十二指肠切除术（横断-朗迈尔手术）、保留脾脏的远端胰腺切除术、远端胰腺切除术联合整块脾脏切除术、Roux-en-Y端端胰空肠吻合术。共有13名儿童无疾病，中位随访时间为53个月（范围11 - 156个月）。
儿童和青少年胰腺母细胞瘤是一种恶性肿瘤。完整切除联合化疗与长期生存相关。对于诊断时无法切除的肿瘤，建议术前化疗以缩小肿瘤体积。甲胎蛋白作为肿瘤标志物对诊断和监测疾病至关重要。
