Department of Pediatric Surgery, Children's Hospital Boston, Harvard Medical School, Boston, MA, 02115, USA.
J Pediatr Surg. 2009 Dec;44(12):2267-72. doi: 10.1016/j.jpedsurg.2009.07.078.
The rarity and histopathologic diversity of primary pancreatic neoplasms in children have made it difficult to predict prognosis and to develop optimal management protocols.
A 90-year (1918-2007), single institution, retrospective review of all patients with neoplastic pancreatic masses was performed.
Eighteen patients were identified with 7 distinct histopathologic subtypes. The most common were gastroenteropancreatic neuroendocrine, solid pseudopapillary, and acinar tumors. There were 6 benign and 12 malignant tumors. Six patients had disease outside the pancreas at their initial operation. There were 7 deaths (41%), 2 related to the initial operation, 3 from disease progression, 1 from a small bowel obstruction, and 1 from necrotizing pancreatitis. Five deaths were in patients with extrapancreatic disease found at initial operation. The median duration of follow-up for the 10 survivors was 41 months.
In adults, pancreatic ductal adenocarcinoma is by far the most common histopathologic subtype, with other subtypes more common in children. Stage is an important prognostic factor. Long-term disease-free survival in childhood pancreatic malignancies is achievable with complete surgical resection, prognosis, and adjuvant treatment, depending on the histopathologic type.
儿童原发性胰腺肿瘤的罕见性和组织病理学多样性使得预测预后和制定最佳治疗方案变得困难。
对 1918 年至 2007 年间所有患有胰腺肿瘤的患者进行了一项 90 年（1918-2007 年）、单一机构、回顾性研究。
共发现 18 名患者，有 7 种不同的组织病理学亚型。最常见的是胃肠胰神经内分泌、实性假乳头状和腺泡肿瘤。有 6 例良性肿瘤和 12 例恶性肿瘤。6 例患者在初次手术时存在胰腺外疾病。共有 7 例死亡（41%），其中 2 例与初次手术有关，3 例死于疾病进展，1 例死于小肠梗阻，1 例死于坏死性胰腺炎。5 例死亡患者在初次手术时发现胰腺外疾病。10 例幸存者的中位随访时间为 41 个月。
在成人中，胰腺导管腺癌是最常见的组织病理学亚型，而其他亚型在儿童中更为常见。分期是一个重要的预后因素。通过完全手术切除、预后和辅助治疗，儿童胰腺恶性肿瘤可以实现长期无病生存，具体取决于组织病理学类型。
