Blonski Wojciech C, Reddy K Rajender, Shaked Abraham, Siegelman Evan, Metz David C
Gastroenterology Division, University of Pennsylvania Health System, 3400 Spruce Street, 3 Ravdin Building, Philadelphia, PA 19104, USA.
World J Gastroenterol. 2005 Dec 28;11(48):7676-83. doi: 10.3748/wjg.v11.i48.7676.
Neuroendocrine tumors are divided into gastrointestinal carcinoids and pancreatic neuroendocrine tumors. The WHO has updated the classification of these lesions and has abandoned the term "carcinoid". Both types of tumors are divided into functional and non-functional tumors. They are characterized by slow growth and frequent metastasis to the liver and may be limited to the liver for long periods. The therapeutic approach to hepatic metastases should consider the number and distribution of the liver metastases as well as the severity of symptoms related to hormone production and tumor bulk. Surgery is generally considered as the first line therapy. In patients with unresectable liver metastases, alternative treatments are dependent on the type and the growth rate. Initial treatments consist of long acting somatostatin analogs and/or interferon. Streptozocin-based chemotherapy is usually reserved for symptomatic patients with rapidly advancing disease, but generally the therapy is poorly tolerated and its effects are short-lived. Locoregional therapy directed such as hepatic-artery embolization and chemoembolization, radiofrequency thermal ablation and cryosurgery, is often used instead of systemic therapy, if the disease is limited to the liver. However, liver transplantation should be considered in patients with neuroendocrine metastases to the liver that are not accessible to curative or cytoreductive surgery and if medical or locoregional treatment has failed and if there are life threatening hormonal symptoms. We report a case of liver transplantation for metastatic neuroendocrine tumor of unknown primary source and provide a detailed review of the world literature on this controversial topic.
神经内分泌肿瘤分为胃肠道类癌和胰腺神经内分泌肿瘤。世界卫生组织已更新了这些病变的分类，并摒弃了“类癌”这一术语。这两种类型的肿瘤都分为功能性和非功能性肿瘤。它们的特点是生长缓慢，常转移至肝脏，且可能长期局限于肝脏。肝转移瘤的治疗方法应考虑肝转移灶的数量和分布，以及与激素产生和肿瘤大小相关的症状严重程度。手术通常被视为一线治疗方法。对于无法切除肝转移瘤的患者，替代治疗取决于肿瘤类型和生长速度。初始治疗包括长效生长抑素类似物和/或干扰素。基于链脲佐菌素的化疗通常用于病情快速进展的有症状患者，但一般来说，该治疗耐受性差且效果短暂。如果疾病局限于肝脏，常采用诸如肝动脉栓塞和化疗栓塞、射频热消融和冷冻手术等局部区域治疗来替代全身治疗。然而，对于无法进行根治性或减瘤手术、内科或局部区域治疗失败且存在危及生命的激素症状的肝转移性神经内分泌肿瘤患者，应考虑肝移植。我们报告了一例原发灶不明的转移性神经内分泌肿瘤肝移植病例，并对关于这一有争议话题的世界文献进行了详细综述。
