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肝移植治疗神经内分泌肿瘤患者:2 例罕见病例报告及文献复习。

Liver Transplant for Patients With Neuroendocrine Tumor: A Report of 2 Exceptional Cases and Literature Review.

机构信息

From the Department of General Surgery Division of Hepato-Pancreatico-Biliary Surgery and Organ Transplantation Surgery, Tehran University of Medical Sciences, Tehran, Iran.

出版信息

Exp Clin Transplant. 2023 Jul;21(7):578-585. doi: 10.6002/ect.2023.0110.

Abstract

Patients with neuroendocrine tumors with unresec-table liver involvement can benefit from liver transplant. There is a specific set of guidelines for neuroendocrine tumors with liver metastasis that involve less than 50% of the liver. However, beyond those guidelines, there are reports of exceptional criteria patients who benefited from liver transplant. Here, we present 2 unusual cases of patients with exceptional circumstances and with neuroendocrine tumors who underwent liver transplant. The first case describes a patient with an extremely rare neuroen-docrine tumor of the proximal common bile duct that caused liver biliary cirrhosis. The patient underwent tumor resection and liver transplant concurrently. The second case describes a patient with a neuroendocrine tumor of unknown primary origin with more than 50% hepatic involvement who received a liver transplant after downstaging. In our center, patients with unresectable hepatic metastases from neuroendoc-rine tumors are currently selected for liver transplant based on well-established criteria. However, these 2 cases did not meet the criteria for consideration of liver transplant; thus, multidisciplinary team sessions were held to discuss these 2 cases. After a period of nonsurgical treatment and evaluation of the tumor behavior, we selected the patients as candidates for liver transplant based on the favorable tumor behavior and favorable response to treatment. For both patients, we did not observe any signs of tumor recurrence during follow-up. The outcomes were acceptable, and the patients tolerated treatment well. Considering the favorable tumor pathology (G1 phase and low Ki67 index), we suggest that more studies should be conducted to evaluate the outcomes of patients with low-grade tumors and that the criteria for patients with low-grade tumors could be extended based on such future data.

摘要

无法手术切除肝转移的神经内分泌肿瘤患者可以从肝移植中获益。对于肝转移瘤少于 50%的神经内分泌肿瘤,有一套特定的指南。然而,超出这些指南之外,也有报道称存在一些特殊标准的患者从肝移植中获益。在这里,我们报告了 2 例具有特殊情况的神经内分泌肿瘤患者接受肝移植的不寻常病例。第一个病例描述了 1 例极为罕见的近端胆总管神经内分泌肿瘤,导致肝胆汁性肝硬化。患者同时进行了肿瘤切除和肝移植。第二个病例描述了 1 例起源不明的神经内分泌肿瘤患者,肝脏受累超过 50%,在降期后接受了肝移植。在我们中心,目前根据既定标准选择无法手术切除的神经内分泌肿瘤肝转移患者进行肝移植。然而,这 2 例病例不符合考虑肝移植的标准;因此,我们举行了多学科团队会议来讨论这 2 例病例。经过一段时间的非手术治疗和肿瘤行为评估,我们根据肿瘤的良好行为和对治疗的良好反应选择这 2 例患者作为肝移植候选者。在随访期间,我们未观察到这 2 例患者有任何肿瘤复发的迹象。结果是可以接受的,患者对治疗耐受良好。考虑到肿瘤的良好病理学(G1 期和低 Ki67 指数),我们建议应进行更多研究来评估低级别肿瘤患者的结果,并根据未来的数据扩展低级别肿瘤患者的标准。

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