Fenwick Stephen W, Wyatt Judith I, Toogood Giles J, Lodge J Peter A
Hepatobiliary and Transplant Unit, St James's University Hospital, Leeds, UK.
Ann Surg. 2004 Feb;239(2):210-9. doi: 10.1097/01.sla.0000109155.89514.42.
To discuss the diagnosis and management of primary carcinoid tumors of the liver in light of our experience and a literature review.
Carcinoid tumors of the liver are rare and pose a diagnostic and management dilemma. This series is the largest reported and the only one to include liver transplantation as a treatment option.
Between March 1994 and May 2002, we treated 8 patients (4 male, 4 female) with primary hepatic carcinoid tumors. Carcinoid syndrome complicated only 1 of the cases. Treatment was by liver resection in 6 patients and orthotopic liver transplantation in 2.
The diagnosis was confirmed histologically with light microscopy and immunohistochemistry in the absence of an alternative primary site. Six patients remain alive and disease free after follow-up of more than 3 years: 39, 43, 45, 50, 50, and 95 months. Two patients are recently postoperative.
Active exclusion of an extrahepatic primary site is essential for the diagnosis of primary carcinoid of the liver. The mainstay of treatment should be liver resection, although liver transplantation may be considered in patients with widespread hepatic involvement. A radical surgical approach is warranted as this disease carries a better prognosis than for other primary hepatic tumors and for secondary hepatic carcinoids.
根据我们的经验及文献复习,探讨肝脏原发性类癌肿瘤的诊断及治疗。
肝脏类癌肿瘤罕见,在诊断和治疗上存在两难问题。本系列是已报道的最大病例组,且是唯一将肝移植作为一种治疗选择的病例组。
1994年3月至2002年5月,我们治疗了8例原发性肝脏类癌肿瘤患者(4例男性,4例女性)。仅1例出现类癌综合征。6例患者接受肝切除术,2例接受原位肝移植术。
在排除其他原发部位后,经组织学光镜检查及免疫组化确诊。6例患者在随访3年以上后仍存活且无疾病复发:随访时间分别为39、43、45、50、50和95个月。2例患者近期接受了手术。
对于肝脏原发性类癌的诊断,积极排除肝外原发部位至关重要。治疗的主要方法应为肝切除术,尽管对于肝广泛受累的患者可考虑肝移植。鉴于该疾病较其他原发性肝脏肿瘤及继发性肝脏类癌预后更好,采取根治性手术方法是必要的。