胰腺实性假乳头状瘤（SPN）是儿童胰腺肿瘤中最常见的一种疾病。然而，这些疾病很少见，每年的发病率为0.1-0.2/1,000,000，人们对其最佳治疗方法所知甚少。本分析旨在增加对儿童SPN发生和治疗策略的了解。我们分析了德国罕见儿科肿瘤登记簿（STEP）中记录的0-18岁患有SPN的儿童的诊断、治疗和结果数据。共识别出38名患者，诊断时中位年龄为14.5岁（范围：8-18岁），女性占多数（81.6%）。肿瘤最常见的部位是胰腺尾部。在组织病理学和免疫组化检查中，假乳头状、实性和囊性病变以及β-连环蛋白、孕激素受体和细胞周期蛋白D1的表达最常见。所有患者均接受了手术切除。大多数患者接受了开放性切除，主要是尾部切除术用于尾部区域的肿瘤，胰十二指肠切除术用于头部区域的肿瘤。术后主要的影响是内源性胰腺功能不全（23.7%），特别是胰头部位的SPN。在随访期间没有出现复发，尽管有两名患者进行了显微镜残留切除。儿童胰腺SPN是低度恶性肿瘤，通常治疗结果良好。然而，治疗可能导致相关的长期后遗症。为了预防复发，建议进行完全的手术切除，并尽可能保留健康的胰腺组织。为了优化治疗，跨学科专家的协作是必不可少的。这些肿瘤的分子遗传分析可能有助于改善对其发生机制的理解。 • 儿童胰腺实性假乳头状瘤（SPN）是非常少见的疾病。 • 对肿瘤发生的了解很有限，并且儿科患者的治疗和随访没有具体的指南。 • 我们对大量儿童SPN患者进行了综合评估，包括其特征、治疗和结果。 • 我们提出了儿童SPN诊断、治疗和随访的建议，这些建议是基于我们的分析和已有的经验。 © 2023. 作者。

Solid pseudopapillary neoplasms (SPNs) are the most common entity among pediatric pancreatic tumors. Still, these are rare tumors with an annual incidence of 0.1-0.2/1,000,000, and little is known about their optimal treatment. This analysis aimed to increase knowledge about the occurrence and treatment strategies of SPN in childhood. Data regarding diagnostics, treatment, and outcome of children aged 0-18 years with SPN recorded in the German Registry for Rare Pediatric Tumors (STEP) were analyzed. Thirty-eight patients were identified with a median age of 14.5 years at diagnosis (range: 8-18) and a female preponderance (81.6%). The most frequent location of the tumor was the pancreatic tail. In histopathological and immunohistochemical examination, pseudopapillary, solid, and cystic lesions as well as expression of beta-catenin, progesterone receptors, and cyclin D1 were the most common findings. All patients underwent surgical resection. Most patients underwent open resection, predominantly tail resection for tumors in the tail region and pylorus-preserving pancreaticoduodenectomy for tumors in the head region. The main postoperative sequela was exogenous pancreatic insufficiency (23.7%), especially with SPN in the pancreatic head. No recurrence occurred during follow-up, although two patients underwent resection with microscopic residue.SPN of the pancreas in childhood are low-grade malignancies with usually favorable treatment outcomes. However, therapy can lead to relevant long-term sequelae. To prevent recurrence, complete surgical resection is recommended, sparing as much healthy pancreatic tissue as possible. Interdisciplinary collaboration between specialists is essential to optimize treatment. Molecular genetic analysis of these tumors could improve understanding of their genesis.• Solid pseudopapillary neoplasms (SPNs) of the pancreas are very rare tumors in childhood. • Little is known about tumorigenesis, and there are no specific guidelines for treatment and follow-up in pediatric patients.• Characteristics, treatment, and outcome were comprehensively assessed in a large cohort of pediatric patients with SPN. • We propose recommendations for diagnosis, treatment, and follow-up of children with SPN, based on our analysis and considering published experience.© 2023. The Author(s).