关键词: Gastrinoma Insulinoma Pancreatic masses Pancreatoblastoma Rhabdomyosarcoma Solid pseudopapillary tumor

来  源:   DOI:10.1007/s00431-024-05731-z

Abstract:
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.
BACKGROUND: • 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.
BACKGROUND: • 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:
胰腺肿块在儿科患者中极为罕见,可用数据有限。这种数据的缺乏使得儿童中这些肿瘤的诊断和管理极具挑战性。因此,我们的目的是描述演讲,临床课程,以及我们中心胰腺肿瘤患儿的结局。回顾性分析了2003年至2022年在一家学术独立儿童医院诊断为胰腺肿块的所有儿科患者。数据包括人口统计,临床表现,workup,管理,收集和汇总随后的发病率和死亡率。此外,我们在国家外科质量改善计划-儿科(NSQIP-P)数据库中回顾了胰腺肿瘤切除病例,以确定常见的不良结局和质量改善措施.总的来说,在我们的机构确定了17名患者。诊断包括实性假乳头(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,被排除在随后的分析之外。总的来说,12例患者接受了手术干预,最常见的手术是胰十二指肠切除术和远端胰腺切除术,最后一次接触时,所有12人都还活着。有3人死亡,都是由于与转移性疾病相关的并发症。此外,在NSQIP-P数据集中,儿科患者胰腺手术的术后30天结果非常好,发病率可忽略不计,索引手术后无死亡率。
结论:接受手术切除的胰腺肿瘤患儿似乎具有足够的长期生存率。诊断时的短期结果是极好的,并且主要似乎受到初始表现时转移性疾病的影响。
背景:•胰腺肿块是儿童中的罕见实体,其表现数据有限,管理和手术结果。•实性假乳头状瘤是手术干预后预后良好的儿童中最常见的胰腺肿瘤之一。
背景:•对于没有侵袭性肿瘤类型或转移性疾病的患者,小儿胰腺肿瘤的外科治疗是安全有效的。•我们的病例系列提供了这些胰腺肿瘤的一个值得注意的队列,其中五种肿瘤类型的治疗和结果。
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