无功能胰腺神经内分泌癌的诊断与外科治疗

Diagnosis and surgical treatment of nonfunctioning pancreatic neuroendocrine carcinoma

目的提高对无功能胰腺神经内分泌癌( NPNEC)的认识和诊治水平。方法回顾性分析19年间收治的NPNEC 18例的临床资料,包括肝转移组8例及无转移组10例。分析该病的病理特点、临床表现、诊断及治疗。2例行非手术治疗;16例行手术治疗,15例多次行介入化疗。肿瘤标本行神经内分泌细胞标志铬粒素( CgA)、神经特异性烯醇酶( NSE)、突触素( Syn)及相关激素胰岛素( Ins)、胃泌素( Gas)、胰高血糖素( Gluca)、血管活性肠肽( VIP)和生长抑素( Soma)的定性检测。结果年龄、原发瘤大小在组间(肝转移组与无转移组)无明显差别;肿瘤中CgA, NSE及Syn两项以上阳性者见于所有标本,Ins,Gas, Gluca, VIP及Soma 5项全部阴性者占25%。2例非手术治疗者分别于确认后39.61个死亡手术切除至术后首次复发或转移的时间平均为29.8个月。生存期超过3年者8例,超过5年者7例,最长的2例已达7年。结论NPNEC临床表现无特异性;虽无内分泌紊乱综合征,但肿瘤大多可产生激素;即使有肝转移,如能积极手术并辅以介入治疗仍可使患者获得长期生存。

Objective To summarize our experience in diagnosis and surgical treatment of nonfunctioning pancreatic neuroendocrine carcinoma （NPNEC）. Methods The clinical data of 18 cases of NPNEC, including Group A those with liver metastasis, n = 8 ; Group B those without liver metastasis, n = 10, from 1985 to 2004 were retrospectively reviewed. Operations were performed in 16 cases, interventional procedures in 15 cases, and non-operative treatment in 2 cases respectively. The markers of neuroendecrine cell （CgA, NSE, Syn ） and the main peptide hormones （insulin, gastrin, glucagon, VIP, and somatostatin ） were examined in tumor samples with immunohistochemistry method. Results Group A and B were similar with respect to age （ year ） and tumor diameter （ cm ） at diagnosis （ 40.50±12.54 vs 51.60 ±12.85, and 8.45±5. 12 vs 6.26±3.65, respectively; P〉0.05）. Positive expression of 2 or all3 markers of neuroendocrine cell was detected in all 16 samples; negative expression of all 5 peptide hormones was observed in only 25 % of samples. At a mean follow-up of 44.2 months （ range 4 months - 7 years ） , the mean time from resection to relapse or metastasis was 29.8 months ; 7 cases survived more than 5 years, of which 2 have survived for 7 years. Two cases, who subjected non-operative treatment, survired 39,61 months, respectively. Conclusions Clinical manifestations of NPNEC are non-specific. Only a few tumors in NPNEC patients do not yield peptide hormones. Treatment of NPNEC （ including patients with liver metastasis ） with aggressive sttrgical resection followed by interventional methods can result in excellent overall long-term survival.