PDF(Pubmed)
Abstract：
Complete surgical resection in the context of a multimodal approach has been associated with excellent long-term survival in children diagnosed with pancreatoblastoma (PB). Traditionally, curative intent surgery for PB implies standard pancreatic resections such as pancreaticoduodenectomies and distal pancreatectomies with splenectomies, surgical procedures that may lead to significant long-term pancreatic functional deficiencies. Postoperative pancreatic functional deficiencies are particularly interesting to children because they may interfere with their development, considering their long life expectancy and the significant role of pancreatic functions in their nutritional status and growth. Thus, organ-sparing pancreatectomies, such as spleen-preserving distal pancreatectomies and central pancreatectomies, are emerging in specific tumoral pathologies in children. However, data about organ-sparing pancreatectomies\' potential role in curative-intent PB surgery in children are scarce. Based on the literature data, the current review aims to present the early and late outcomes of pancreatectomies in children (including long-term deficiencies and their potential impact on the development and quality of life), particularly for PB, and further explore the potential role of organ-sparing pancreatectomies for PB. Organ-sparing pancreatectomies are associated with better long-term pancreatic functional outcomes, particularly central pancreatectomies, and have a reduced impact on children\'s development and quality of life without jeopardizing their oncological safety. The long-term preservation of pancreatic functions should not be disregarded when performing pancreatectomies for PB in children. A subset of patients with PB might benefit from organ-sparing pancreatectomies, particularly from central pancreatectomies, with the same oncological results as standard pancreatectomies but with significantly less impact on long-term functional outcomes.
摘要：
在诊断为胰腺母细胞瘤（PB）的儿童中，在多模式方法的背景下进行完整的手术切除与出色的长期生存率相关。传统上,PB的治愈性手术意味着标准的胰腺切除术，如胰十二指肠切除术和远端胰腺切除术伴脾切除术，可能导致严重的长期胰腺功能缺陷的外科手术。术后胰腺功能缺陷对儿童特别有趣，因为它们可能会干扰他们的发育，考虑到他们的预期寿命和胰腺功能在他们的营养状况和生长中的重要作用。因此，保留器官的胰腺切除术，例如保留脾脏的远端胰腺切除术和中央胰腺切除术，正在出现在儿童的特定肿瘤病理中。然而,关于保留器官的胰腺切除术在儿童治愈性PB手术中的潜在作用的数据很少。根据文献数据，本综述旨在介绍儿童胰腺切除术的早期和晚期结果（包括长期缺陷及其对发育和生活质量的潜在影响），尤其是PB,并进一步探讨保留器官的胰腺切除术对PB的潜在作用。保留器官的胰腺切除术与更好的长期胰腺功能结局相关，尤其是中央胰腺切除术，并减少对儿童的发展和生活质量的影响，而不会危及他们的肿瘤安全。在儿童中进行PB的胰腺切除术时，不应忽视胰腺功能的长期保存。一部分PB患者可能受益于保留器官的胰腺切除术，尤其是胰腺中央切除术，肿瘤学结果与标准胰腺切除术相同，但对长期功能结局的影响明显较小。
