儿童胰腺肿块:二十年来的单中心经验。
Pancreatic masses in children: a single-center experience over two decades.
发表日期:2024 Aug 15
作者:
Joyce J L H McRae, Humza Thobani, Marla A Sacks, Steven L Raymond, Adil Shah, Andrei Radulescu, Faraz A Khan
来源:
EUROPEAN JOURNAL OF PEDIATRICS
摘要:
胰腺肿块在儿科患者中极为罕见,可用数据有限。数据的缺乏使得儿童这些肿瘤的诊断和治疗极具挑战性。因此,我们的目的是描述我们中心患有胰腺肿瘤的儿童的表现、临床病程和结果。对 2003 年至 2022 年间在一家学术独立儿童医院诊断出胰腺肿块的所有儿科患者进行了回顾性分析。收集和汇总的数据包括人口统计、临床表现、检查、管理以及随后的发病率和死亡率。此外,我们回顾了国家外科质量改进计划 - 儿科 (NSQIP-P) 数据库中的胰腺肿瘤切除病例,以确定常见的不良结果和质量改进措施。我们机构总共确认了 17 名患者。诊断包括实性假乳头状瘤 (n = 9)、胃泌素瘤 (n = 1)、横纹肌肉瘤 (n = 2)、胰母细胞瘤 (n = 2) 和胰岛素瘤 (n = 1)。两名患者没有组织病理学诊断,因此被排除在后续分析之外。总体而言,12 名患者接受了外科手术,最常见的手术是胰十二指肠切除术和远端胰腺切除术,并且在最后一次接触时已知所有 12 名患者都还活着。共有 3 人死亡,均死于与转移性疾病相关的并发症。此外,NSQIP-P 数据集中的儿科患者胰腺手术术后 30 天的结果非常好,指数手术后的发病率可以忽略不计,也没有死亡病例。适合手术切除的胰腺肿瘤儿童似乎有足够的长期生存率。诊断时的短期结果非常好,并且主要受到初次就诊时是否存在转移性疾病的影响。• 胰腺肿块在儿童中是一种罕见的实体,有关其表现、治疗和手术结果的数据有限。 • 实性假乳头状瘤是儿童最常见的胰腺肿瘤之一,手术干预后预后良好。 • 对于没有侵袭性肿瘤类型或转移性疾病的儿童胰腺肿瘤患者,手术治疗是安全有效的。 • 我们的病例系列提供了一组值得注意的胰腺肿瘤,深入了解其中五种肿瘤类型的表现、治疗和结果。© 2024。作者获得 Springer-Verlag GmbH 德国(Springer 旗下公司)的独家许可自然。
Pancreatic masses are extremely rare in pediatric patients, with limited data available. This lack of data makes the diagnosis and management of these tumors in children extremely challenging. Therefore, we aimed to describe the presentations, clinical course, and outcomes of children with pancreatic tumors at our center. A retrospective analysis was performed of all pediatric patients diagnosed with pancreatic masses between 2003 and 2022 in an academic freestanding children's hospital. Data including demographics, clinical presentation, workup, management, and subsequent morbidity and mortality were collected and aggregated. Furthermore, we reviewed cases of pancreatic tumor resections in the National Surgical Quality Improvement Program - Pediatric (NSQIP-P) database to identify common adverse outcomes and measures for quality improvement. In total, 17 patients were identified at our institution. Diagnoses included solid pseudopapillary (n = 9), gastrinoma (n = 1), rhabdomyosarcoma (n = 2), pancreatoblastoma (n = 2), and insulinoma (n = 1). Two patients did not have a histopathologic diagnosis and were excluded from subsequent analysis. Overall, 12 patients underwent surgical intervention, with the most common procedures being pancreaticoduodenectomy and distal pancreatectomy, and all 12 were known to be alive at last contact. There were 3 deaths, all due to complications related to metastatic disease. Furthermore, 30-day postoperative outcomes in the NSQIP-P dataset for pancreatic surgeries in pediatric patients are excellent, with negligible morbidity and no mortalities after the index surgery.Children with pancreatic tumors amenable to surgical resection appear to have adequate long-term survival. Short-term outcomes at diagnosis are excellent and mainly appear to be influenced by the presence of metastatic disease at initial presentation.• Pancreatic masses are a rare entity in children with limited data on their presentation, management and surgical outcomes. • Solid Pseudopapillary tumors are one of the most common pancreatic tumors in children with a fair prognosis after surgical intervention.• Surgical management of pediatric patients with pancreatic tumors is safe and effective in patients who do not have aggressive tumor types or metastatic disease. • Our case series provides a notable cohort of these pancreatic tumors with insight into the presentation, management and outcomes of five of these tumor types.© 2024. The Author(s), under exclusive licence to Springer-Verlag GmbH Germany, part of Springer Nature.