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儿童胰腺肿块:二十多年单中心经验。

Pancreatic masses in children: a single-center experience over two decades.

机构信息

Division of Pediatric Surgery, Loma Linda University Children's Hospital, Loma Linda, CA, USA.

Division of Pediatric General Surgery, Stanford University, Palo Alto, CA, USA.

出版信息

Eur J Pediatr. 2024 Oct;183(10):4467-4476. doi: 10.1007/s00431-024-05731-z. Epub 2024 Aug 15.

Abstract

UNLABELLED

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.

CONCLUSIONS

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.

WHAT IS KNOWN

• 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.

WHAT IS NEW

• 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.

摘要

背景

胰腺肿块在儿科患者中极为罕见,相关数据有限。这使得儿童胰腺肿瘤的诊断和治疗极具挑战性。因此,我们旨在描述本中心胰腺肿瘤患儿的临床表现、临床病程和结局。

方法

对 2003 年至 2022 年期间在一家学术型独立儿童医院诊断为胰腺肿块的所有儿科患者进行回顾性分析。收集并汇总了人口统计学、临床表现、检查、治疗以及随后的发病率和死亡率等数据。此外,我们还在国家外科质量改进计划-儿科(NSQIP-P)数据库中回顾了胰腺肿瘤切除术的病例,以确定常见的不良结局和质量改进措施。

结果

本机构共发现 17 例患者。诊断包括实性假乳头状瘤(9 例)、胃泌素瘤(1 例)、横纹肌肉瘤(2 例)、胰腺胚胎瘤(2 例)和胰岛素瘤(1 例)。2 例患者未行组织病理学诊断,故排除在后续分析之外。总体而言,12 例患者接受了手术干预,最常见的手术方式为胰十二指肠切除术和胰体尾切除术,所有 12 例患者在最后一次随访时均存活。3 例死亡,均归因于转移疾病相关并发症。此外,NSQIP-P 数据库中胰腺手术的 30 天术后结局非常好,发病率低,索引手术后无死亡。

结论

可手术切除的胰腺肿瘤患儿似乎有足够的长期生存。诊断时的短期结局良好,主要似乎受初始表现时转移疾病的影响。

儿童胰腺肿瘤少见,其临床表现、治疗和手术结局的数据有限。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。

手术治疗无侵袭性肿瘤类型或转移疾病的儿童胰腺肿瘤患儿是安全有效的。本病例系列为这些胰腺肿瘤提供了一个显著的队列,深入了解了其中五种肿瘤类型的临床表现、治疗和结局。

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