Department of Pediatric Surgery, Texas Children's Hospital, Baylor College of Medicine, Houston, TX, USA.
J Pediatr Surg. 2012 Dec;47(12):2199-204. doi: 10.1016/j.jpedsurg.2012.09.005.
Malignant pancreatic neoplasms in children and adolescents are rare. The clinical presentation, pathologic characteristics, management, and outcomes at two institutions are discussed.
We retrospectively reviewed all pediatric patients (age <= 18 years) treated for malignant pancreatic neoplasms at two institutions between 1991 and 2011.
Thirty-one patients were identified with median age of 14.7 years (4-18 years). The most common histology was solid pseudopapillary tumor (SPT) (n=22, 71%) followed by neuroendocrine tumors (n=4, 13%), pancreatoblastoma (n=4, 13%), and one unclassified spindle cell neoplasm (3%). Most patients presented with abdominal pain (n=22, 71%). Complications included pancreatic leak, pseudocyst formation, pancreatitis, pancreatic insufficiency, and small bowel obstruction. The overall 1- and 5-year survival was 96% (95% CI 74%-99%) and 78% (95% CI 43%-93%). Median follow-up among patients alive at the end of follow-up was 20 months (<1 month-16.2 years). Patients with SPT had better overall survival compared to patients with neuroendocrine tumors or pancreatoblastomas (Log-rank; p=0.0143).
The majority of pediatric and adolescent patients present with SPTs which are usually resectable and associated with an excellent prognosis. Other histologic subtypes more often present with distant metastases and portend a worse prognosis.
儿童和青少年的胰腺恶性肿瘤较为罕见。本研究讨论了两家机构的临床表现、病理特征、治疗方法和预后。
我们回顾性分析了 1991 年至 2011 年间在两家机构接受治疗的所有胰腺恶性肿瘤的儿科患者（年龄≤18 岁）。
共 31 例患者符合标准，中位年龄为 14.7 岁（4-18 岁）。最常见的组织学类型是实性假乳头状瘤（SPT）（22 例，71%），其次是神经内分泌肿瘤（4 例，13%）、胰腺母细胞瘤（4 例，13%）和 1 例未分类梭形细胞瘤（3%）。大多数患者以腹痛（22 例，71%）为首发症状。并发症包括胰瘘、假性囊肿形成、胰腺炎、胰腺功能不全和小肠梗阻。总体 1 年和 5 年生存率分别为 96%（95%CI 74%-99%）和 78%（95%CI 43%-93%）。在随访结束时存活的患者中，中位随访时间为 20 个月（1 个月-16.2 年）。与神经内分泌肿瘤或胰腺母细胞瘤患者相比，SPT 患者的总体生存率更高（Log-rank 检验；p=0.0143）。
大多数儿童和青少年患者表现为 SPT，通常可切除，预后良好。其他组织学类型常伴有远处转移，预后较差。
