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胰腺神经内分泌肿瘤肝转移作为儿童肝移植的极罕见适应证。病例报告及文献复习。

Metastases of pancreatic neuroendocrine tumor to the liver as extremely rare indication for liver transplantation in children. Case report and review of the literature.

机构信息

The Department of Pediatric Surgery and Organ Transplantation, The Children's Memorial Health Institute, Al. Dzieci Polskich 20, 04-730 Warsaw, Poland.

The Department of Pediatric Surgery and Organ Transplantation, The Children's Memorial Health Institute, Al. Dzieci Polskich 20, 04-730 Warsaw, Poland.

出版信息

Clin Res Hepatol Gastroenterol. 2016 Sep;40(4):e33-7. doi: 10.1016/j.clinre.2015.12.001. Epub 2016 Feb 2.

DOI:10.1016/j.clinre.2015.12.001
PMID:26850362
Abstract

Neuroendocrine tumors (NET) are extremely rare in children (0.75 cases per 100,000 children and adolescents a year) and the majority of these tumors are benign or present low grade of malignancy. According to the American registry Surveillance, Epidemiology, and End Results (SEER) Program of the National Cancer Institute, less than 2% of all neuroendocrine tumors in children occur in the pancreas, making it a rare site for these tumors. The majority of them are found in children over 10years of age, especially those with malignant potential. Treatment of NET consists of different methods: surgery, somatostatin analogues and chemotherapy. Radical surgical resection remains the standard of treatment; however, it is not always feasible because of distant metastases. The authors present a case report of pancreatic NET with multiple metastases to the liver. The patient was treated with pancreatic resection and liver transplantation for liver metastases. Prior to liver transplantation, the patient was treated with somatostatin analogues, sunitinib and chemotherapy. Management of liver metastases with liver transplantation is discussed.

摘要

神经内分泌肿瘤(NET)在儿童中极为罕见(每年每 10 万名儿童和青少年中有 0.75 例),且这些肿瘤大多数为良性或低度恶性。根据美国国家癌症研究所的美国注册处监测、流行病学和最终结果(SEER)计划,儿童中不到 2%的神经内分泌肿瘤发生在胰腺,这使得胰腺成为这些肿瘤的罕见部位。它们中的大多数发生在 10 岁以上的儿童中,特别是那些具有恶性潜能的儿童。NET 的治疗包括不同的方法:手术、生长抑素类似物和化疗。根治性手术切除仍然是治疗标准;然而,由于远处转移,并非总是可行。作者报告了一例胰腺 NET 伴肝多发转移的病例。该患者接受了胰腺切除术和肝移植治疗肝转移。在肝移植前,该患者接受了生长抑素类似物、舒尼替尼和化疗治疗。讨论了肝移植治疗肝转移的管理。

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