神经内分泌肿瘤肝转移的临床特征与诊治

The clinical characteristic of 43 cases of neuroendocrine tumors with liver metastases

目的对神经内分泌肿瘤（NET）肝转移患者的临床特征及诊治情况进行总结，以加深对该病的认识。方法回顾性分析1996年1月至2010年7月北京协和医院确诊的43例NET肝转移患者的临床资料。结果（1）男：女之比为20：23，中位发病年龄47．5（26～70）岁，发病至确诊的中位时间为4（0～120）个月。（2）69．8％（30／43）的患者以肝转移灶为首发表现而就诊。（3）常规腹部影像学检查（B超、CT、MRI）对原发灶的检出率为65．1％（28／43），联合生长抑素受体显像、正电子发射计算机断层扫描（PET-CT）、超声内镜（EUS）中的1项或多项后检出率可提高到90．7％（39／43）（P=0．004）。（4）69．8％（30／43）的患者通过手术病理确诊。（5）发生肝转移的非功能性和功能性NET分别为28例和15例，原发灶均以胰腺[11例（39．3％）、11例（73．3％）]和胃[6例（21．4％）、2例（13．3％）]多见。（6）治疗方面：88．4％（38／43）的患者接受手术治疗，9．3％（4／43）的患者因仅发现肝转移灶漏诊原发灶而进行了再次手术；非手术治疗主要为长效醋酸奥曲肽、肝脏局部介入治疗、全身化疗及放疗，但随访病例数尚较少，难以评估疗效。结论肝转移是NET的常见甚至首发表现，以非功能性NET居多，其原发灶以消化道最常见。联合生长抑素受体显像、PET．CT、EUS有助于提高原发灶的检出率。手术有助于明确诊断，但术前原发灶的诊断率有待进一步提高，以避免多次手术。

Objective To analyze the clinical characteristics of liver metastases of neuroendocrine tumors （NET） and its treatment outcome, so as to further cognition of NET. Methods The clinical data of patients with liver metastases of NET diagnosed by Peking Union Medical College Hospital during January 1996 to July 2010 were analyzed retrospectively. Results The ratio of male to female was 1：1.15 （20： 23）. The median age at onset of the patients with liver metastases of NET was 47.5 （26-70） years. The median duration from onset to diagnosis was 4 （0-120） months. The liver metastases were the first manifestation in 69. 8% （30/43） cases. The detection rate of primary lesions with routine abdominal imaging （B-type ultrasonography, CT, MRI） was 65. 1% （28/43）, while increased to 90. 7% （39/43） when combined the following one or more special examinations including somatostatin receptor scintigraphy （ SRS ） , PET-CT, endoscopic ultrasound （EUS） （P = 0. 004）. The definite diagnosis methods mainly depended on surgical specimens （69.8% , 30/43）. The ratio of nonfunctional to functional NET with liver metastases was 1.87： 1 （28：15 ）. The primary tumors were most commonly located in pancreas [ 39. 3% （11/28 ） and 73.3% （11/15） ], followed by stomach[ 21.4% （6/28） and 13.3% （2/15） ]. Totally 88.4% （38/43） patients received operation, and 9. 3 % （4/43） patients had reoperation due to missed diagnosis of the primary tumors on earlier operation. Non-surgical treatments included octreotide acetate long-acting release, interventional therapy, chemotherapy and radiotherapy, which were difficult to be evaluated due to less follow-uped cases. Conclusions Liver metastases of NET are common and even the first manifestated symptom. Primary NET with liver metastases is the most commonly nonfunctional and located in digestive system. The detection rates of primary lesions are increased by special examinations including SRS, PET-CT and EUS. Surgical specimens are helpful to the final diagnosis, but it is necessary to improve the preoperative diagnostic rate of primary tumors to avoid repeat surgeries.