提高胃肠胰腺神经内分泌肿瘤的诊断率——胃肠激素临床研究进展

胃肠胰腺神经内分泌肿瘤（GEP—NETs）是少见病．且诊断难度大。随着各种生化、影像以及内镜检查技术的发展．其诊断率不断提高。嗜铬粒蛋白A作为GEP—NETs的“通用”肿瘤标志物．其诊断敏感性和特异性为70％～95％。生长抑素受体显像大大提高了肿瘤的定位诊断率和鉴别诊断：内镜超声技术的应用将进一步提高直径〈1cm肿瘤的定位，结合术中超声技术可进一步提高GEP—NETs的定位诊断。针对上述诊断技术不敏感的GEP—NETs．随着对肿瘤胃肠激素研究的进展。将有望开发出更多特异性或敏感性高的诊断方法。     

壶腹部混合性神经内分泌-非神经内分泌肿瘤1例报告

1病例资料患者男性,57岁,以“皮肤、巩膜黄染20 d”为主诉,于2016年12月6日来本院肝胆胰脾外科治疗,伴尿色加深,近期体质量减轻约5 kg。吸烟史50余年,20支/d;饮酒史50余年,350 g/d。入院时查体:皮肤、巩膜轻度黄染,无明显腹部压痛。血常规:白细胞4.29×10⁹/L,红细胞3.96×10¹²/L,血红蛋白119 g/L。     

消化系神经内分泌肿瘤的临床特点

目的:分析消化系统不同部位和病理类型神经内分泌肿瘤的临床特点,以提高对该类疾病的认识.方法:回顾性分析我院1980-06/2007-12病理证实为神经内分泌肿瘤的消化系统病变73例,分为类癌、不典型类癌、小细胞癌和混合性癌,比较不同类型肿瘤的发病年龄特点、分布部位、临床表现、浸润深度及转移情况.结果:典型类癌平均年龄56-38岁,不典型类癌平均年龄61.29岁,混合性肿瘤平均年龄63岁,小细胞癌平均年龄68.8岁.发病部位直肠最常见,依次为胃、贲门、食管、结肠、阑尾和小肠、肝脏、胆囊和胰腺.病变直径小于1 cm的17例均为典型类癌,局限于黏膜及黏膜下层:直径大于2 cm的46例中,只有3例为典型类癌,14例伴有淋巴结转移(30.4%),6例伴有肝或肺部转移(13%).结论:典型类癌组的发病年龄最小,较少远处转移.直肠是典型类癌的好发部位,不典型类癌好发于贲门及结肠.典型类癌多表现为息肉状,其余3种大体表现与癌不易区别.     

贲门下早期神经内分泌癌一例

神经内分泌肿瘤是一类起源于干细胞且具有神经内分泌功能的肿瘤,可发生于呼吸系统、消化系统等各个部位,最常见的是消化系统神经内分泌肿瘤。以往此类肿瘤习惯统称为“类癌”,但肿瘤起源、激素分化活性等生物学行为均未能明确反映。这导致其早期诊断率较低,患者一旦发生转移则预后较差。本文报道一例混合病理类型的贲门下早期神经内分泌癌普通内镜、色素内镜下特征性表现及ESD术前及术后病理表现。     

结直肠神经内分泌肿瘤25例临床分析

目的探讨结直肠神经内分泌肿瘤的临床特征、诊治方法及预后。 方法回顾分析湖北省肿瘤医院自2006年1月至2015年1月收治的25例结直肠神经内分泌肿瘤患者的临床资料。 结果肿瘤部位：阑尾3例,升结肠3例,乙状结肠2例,直肠17例。分级情况：NET（G1）11例,NET（G2）3例,NEC（G3）8例,MANEC 3例。免疫组织化学：Syn、CgA、NSE阳性表达率为96%,68%和92%。预后：结直肠神经内分泌肿瘤出现转移部位最多见为肝脏。9例Ⅰ期患者生存时间均长于5年。6例Ⅱ期患者,3年内死亡1例,5年内死亡3例,余1例仍在随访中。8例Ⅲ期患者,1年生存率75%,3年生存率50%,5年生存率25%。2例Ⅳ期患者,1例存活28月,另1例存活37月。 结论结直肠神经内分泌肿瘤缺乏特征性临床表现,主要通过内镜下活组织病理检查明确诊断。结直肠神经内分泌肿瘤治疗方法主要依靠手术治疗,早期预后良好,预后与分期和分级密切相关。     

肺大细胞神经内分泌癌临床病理分析

目的 探讨肺大细胞神经内分泌癌的临床病理特点及生物学特征,为进一步提高其诊治水平提供依据.方法 对10例肺大细胞神经内分泌癌患者的临床及肿瘤组织病理学、免疫组织化学检查结果进行分析,并复习相关文献.结果 临床及影像学检查无特异性表现.手术标本(8例)呈现境界清楚的结节样肿块,均单发(其中3例侵及胸膜),直径2.5～8.5 cm,切面呈黄白色或灰褐色,伴有出血及坏死.肿瘤组织学特点为呈浸润性生长,边界不清,细胞丰富,常呈巢状、小梁状、片块状及栅栏状排列,并显示器官样或菊形团样结构.瘤细胞通常较大,核质比例降低,胞质呈嗜酸性颗粒状,核多形性,染色质细或呈空泡状,核仁明显,核分裂像多见.免疫组化结果显示:相关指标阳性率为癌胚抗原( CEA)70％、突触素(Syn)60％、神经元特异性烯醇化酶(NSE) 70％、神经细胞黏附分子(CD56) 100％、甲状腺转录因子-1(TTF-1)100％.结论 肺大细胞神经内分泌癌临床及影像学检查无特异性表现,上述病理组织学及免疫组化检查特点有助于确诊.     

4例原发性肝脏神经内分泌肿瘤临床分析及文献复习

目的 总结原发性肝脏神经内分泌肿瘤(PHNEN)患者的病例资料,分析其临床特征,以提高临床诊断和治疗水平。方法 回顾性分析、总结我院诊治PHNEN患者的临床表现、化验检查、影像学特点、病理学检查和治疗转归。结果 4例患者均为女性,中位年龄为56(40,73)岁;2例无不适主诉,2例有腹痛;血清ALT 28.3(18.0～49.8)U/L,TBIL 11.5(9.5～16.3)μmol/L,神经元特异性烯醇化酶(NSE)为44.1(20.2～77.1)ng/ml;腹部CT均提示肝内多发占位,增强扫描动脉期呈环形或不均匀强化,静脉期强化程度降弱;组织病理学检查示3例为神经内分泌癌,1例为神经内分泌瘤G2;1例患者接受对症治疗,采取手术、介入和/或药物等联合治疗另3例患者,随访11～81个月,2例死亡,2例生存。结论 PHNEN临床少见,缺乏特征性临床表现、辅助检查和病理学诊断标志,需排除其他病变后方可考虑诊断。早期发现和联合治疗是改善患者预后的重要策略。     

胰腺神经内分泌肿瘤的诊治进展

胰腺神经内分泌肿瘤(pancreatic neuroendocrine tumors,PNETs)是源于胰腺多能神经内分泌干细胞的一类肿瘤,故又称胰岛细胞瘤,临床少见或罕见,其发病率约为4~5/100万。该病经积极手术治疗后效果较好,但是由于PNETs自身类型多样,症状复杂多变,临床表现各异,在临床诊疗中极易造成误诊和漏诊。因此,早期正确的诊断是有效治疗PNETs的重要前提。该文针对目前胰腺神经内分泌肿瘤的诊治进展作一系统综述。     

食管神经内分泌癌:临床病例回顾性分析

背景:原发性食管神经内分泌癌(NEC)较为罕见,临床表现无特异性,对标准治疗方式亦尚未形成共识。目的:分析食管NEC患者的临床资料,为该病的诊断和治疗提供参考。方法:收集南京军区南京总医院2001年1月~2012年12月住院食管NEC病例26例,对其临床、内镜、影像学、病理学表现以及治疗和随访情况进行回顾性分析。结果:本组食管NEC患者男女比例为4.2∶1,平均年龄60.1岁(46~75岁)。最常见的首发症状为进食梗阻感/吞咽困难(76.9%),肿瘤中心部位以食管中段居多(73.1%),其余为食管下段(26.9%),内镜下多呈溃疡型(46.2%)和隆起型(38.5%)。病理类型以小细胞NEC居多(69.2%),大细胞和混合性NEC分别占3.8%和26.9%,神经内分泌标记物Syn、CgA阳性率分别为80.0%和21.4%。20例(76.9%)患者发生转移,主要为淋巴结和肝转移。23例(88.5%)患者行根治性手术,随访期间(1个月~7年)6例患者复发,其中5例死亡。结论:食管NEC呈侵袭性生长,易发生转移,预后较差。局部治疗与全身性治疗相结合是延长患者生存期的重要手段。     

Neuroendocrine carcinoma of the extrahepatic bile duct: Case report and literature review

Neuroendocrine carcinoma (NEC) of the extrahepatic bile duct is rare, and only 22 cases have been reported. Only two of these were large-cell NEC (LCNEC); the vast majority were small-cell NEC. Here, we report a third case of LCNEC of the extrahepatic bile duct. A 76-year-old male presented to a local hospital with painless jaundice. Imaging studies revealed a tumor at the hepatic hilum. The patient underwent right hepatic lobectomy, bile duct resection, and cholecystectomy. The resection specimen showed a 5.0-cm invasive neoplasm involving the hilar bile ducts and surrounding soft tissue. Histologically, the tumor consisted of nests of medium to large cells with little intervening stroma. The tumor invaded a large portal vein branch. All four excised lymph nodes were positive for metastasis, and metastatic deposits were also present in the gallbladder wall. The tumor was diffusely positive for synaptophysin and focally positive for chromogranin A. Approximately 70%-80% of the tumor cells were positive for Ki-67, indicating strong proliferative activity. A diagnosis of LCNEC was made. A few bile ducts within and adjacent to the invasive tumor showed dysplasia of the intestinal phenotype and were focally positive for synaptophysin and chromogranin A, suggesting that the dysplastic intestinal-type epithelium played a precursor role in this case. A postoperative computer tomography scan revealed rapid enlargement of the abdominal and retroperitoneal lymph nodes. The patient died 21 d after the operation. NEC of the bile duct is an aggressive neoplasm, and its biological characteristics remain to be better defined.     

胆管内乳头状肿瘤的研究现状

胆管内乳头状肿瘤(IPNB)是一组少见的胆管肿瘤性疾病,有不同的分类标准,预后好于传统胆管癌。由于发病率低,临床医师对其缺乏认识。综述了近年来IPNB的病因与发病机制、病理特点、临床特征、鉴别诊断、治疗及预后等方面研究进展,以期提高临床医师对IPNB的诊疗水平。     

Large-cell neuroendocrine carcinoma of the distal bile duct

Large-cell neuroendocrine carcinoma (LCNEC) in the distal bile duct is very rare and different from common distal bile duct adenocarcinoma. A 77-year-old man was admitted with obstructive jaundice. Severe stenosis of the distal bile duct was revealed by percutaneous transhepatic cholangiography. Subtotal stomach-preserving pancreaticoduodenectomy was performed. A tumor measuring 1.8 cm in diameter was located in the distal bile duct. Both histopathological and immunohistochemical examination of the resected specimen revealed features of LCNEC of the bile duct. The patient developed multiple liver metastases, lung metastases, and local recurrence and died of disease 3 months after the operation. The clinical behavior of LCNEC in the distal bile duct appears to be highly aggressive with early metastases and a fatal outcome.     

Neuroendocrine carcinoma of the extrahepatic bile duct: A case report

Neuroendocrine carcinoma(NEC) originating from the gastrointestinal hepatobiliary-pancreas is a rare, invasive, and progressive disease, for which the prognosis is extremely poor. The patient was a 72-year-old man referred with complaints of jaundice. He was diagnosed with middle extrahepatic cholangiocarcinoma(cT 4N1M0, c Stage Ⅳ). He underwent a right hepatectomy combined with extrahepatic bile duct and portal vein resection after percutaneous transhepatic portal vein embolization. Microscopic examination showed a large-cell neuroendocrine carcinoma according to the WHO criteria for the clinicopathologic classification of gastroenteropancreatic neuroendocrine tumors. Currently, the patient is receiving combination chemotherapy with cisplatin and etoposide for postoperative multiple liver metastases. Although NEC is difficult to diagnose preoperatively, it should be considered an uncommon alternative diagnosis.     

细支气管肺泡癌75例临床分析

目的 总结经病理证实的75例细支气管肺泡癌（BAC）的临床特征和诊断方法.方法 对我院2001年1月～2010年6月收治的75例BAC进行回顾性分析.结果 75例BAC患者男女比例为1∶1.34,平均年龄56.9岁,吸烟者占12％; CT检查58例表现为孤立结节型,8例为弥漫结节型,9例为炎症浸润型;26例患者行CT或超声引导下经皮肺穿刺活检,22例获得确诊,9例行支气管镜检查,仅1例确诊,52例为手术后组织活检确诊.结论 BAC的临床特征不同于其他类型肺癌,CT检查有助于BAC的诊断,而经皮肺穿刺活检则能提高BAC诊断的正确率.     

Sonographic diagnosis of intraluminal bile duct neoplasm: a report of 3 cases

Intraluminal growing tumors of the bile duct are uncommon causes of jaundice. The sonographic appearance of 2 hilar cholangiocarcinomas or Klatskin tumors and a benign extrahepatic biliary cystadenoma is described. Compared to contrast studies of the bile ducts, sonography better defined the intraductal character of the neoplasms. However, the ultrasound appearance did not allow differentiation between the adenocarcinomas and the benign cystadenoma.     

Intraductal neoplasm of the intrahepatic bile duct: clinicopathological study of 24 cases

AIM: To investigate the clinicopathological features of intraductal neoplasm of the intrahepatic bile duct (INihB). METHODS: Clinicopathological features of 24 cases of INihB, which were previously diagnosed as biliary papillomatosis or intraductal growth of intrahepatic biliary neoplasm, were reviewed. Mucin immunohistochemistry was performed for mucin (MUC)1, MUC2, MUC5AC and MUC6. Ki-67, P53 and β-catenin immunoreactivity were also examined. We categorized each tumor as adenoma (low grade), borderline (intermediate grade), and malignant (carcinoma in situ , high grade including tumors with microinvasion). RESULTS: Among 24 cases of INihB, we identified 24 tumors. Twenty of 24 tumors (83%) were composed of a papillary structure; the same feature observed in intraductal papillary neoplasm of the bile duct (IPNB). In contrast, the remaining four tumors (17%) showed both tubular and papillary structures. In three of the four tumors (75%), macroscopic mucin secretion was limited but microscopic intracellular mucin was evident. Histologically, 16 tumors (67%) were malignant, three (12%) were borderline, and five (21%) were adenoma. Microinvasion was found in four cases (17%). Immunohistochemical analysis revealed that MUC1 was not expressed in the borderline/adenoma group but was expressed only in malignant lesions (P = 0.0095). Ki-67 labeling index (LI) was significantly higher in the malignant group than in the borderline/adenoma group (22.2 ± 15.5 vs 7.5 ± 6.3, P 0.01). In the 16 malignant cases, expression of MUC5AC showed borderline significant association with high Ki-67 LI (P = 0.0622). Nuclear expression of β-catenin was observed in two (8%) of the 24 tumors, and these two tumors also showed MUC1 expression. P53 was negative in all tumors. CONCLUSION: Some cases of INihB have a tubular structure, and are subcategorized as IPNB with tubular structure. MUC1 expression in INihB correlates positively with degree of malignancy.     

胆管腺瘤临床分析11例

目的:探讨胆管腺瘤的临床病理特点及诊断治疗方法.方法:对我院经病理证实的11例胆管腺瘤的临床资料进行回顾性分析.结果:11例胆管腺瘤患者有9例女性,2例男性,平均年龄62.2岁.主要临床表现为腹痛、黄疸、发热.术前确诊困难,必须依靠病理组织学检查,恶变率高,肿瘤根治性切除是主要的治疗方法.所有患者均经病理证实为胆管腺瘤,其中乳头状腺瘤8例,管状乳头状腺瘤3例.其中6例伴有癌变,4例伴有不同程度的不典型增生.11例患者中,1例患者行活检术,10例患者行肿瘤根治性切除术.所有患者随访期间均未见明显复发迹象.结论:胆管腺瘤可广泛累及胆道系统,术前诊断较困难,恶变率高,易被误诊,确诊必须依靠病理组织学检查,肿瘤根治性切除是有效治疗方法,预后较好.     

Primary hepatic neuroendocrine carcinoma:clinical analysis of 11 cases

BACKGROUND:Primary hepatic neuroendocrine carcinoma (PHNEC) is extremely rare,and fewer than 300 cases have been reported in the English/Chinese-language literature,therefore it is difficult to make a proper diagnosis and determine a therapeutic approach.METHODS:Eleven PHNEC patients were admitted to our hospital between January 1996 and May 2008.Laboratory examination,digestive endoscopy,B-ultrasonography,CT,MRI,or PET-CT were performed on the patients for preoperative diagnosis.All patients received liver...     

进展期胆囊癌侵犯肝门胆管致梗阻性黄疸15例

目的:探讨进展期胆囊癌侵犯肝门胆管致梗阻性黄疸时的外科治疗价值.方法:回顾性分析15例进展期胆囊癌侵犯肝门胆管致梗阻性黄疸病例的外科治疗情况,并与同期行胆道引流或支架治疗以及保守治疗的15例患者的生存情况进行比较.结果:Kaplan-Meier生存分析显示扩大根治术组中位生存时间为16.0mo±6.1mo,癌灶切除胆肠吻合组中位生存时间为6.0mo±1.1mo胆道引流组中位生存时间为6.00mo±0.77mo保守治疗组中位生存时间为2.00mo±0.45moGehan比分检验对各组生存时间比较,发现扩大根治术组显著长于其余各组(P<0.01),癌灶切除胆肠吻合组与介入胆道引流组无统计学差异(μ=0.015,P=0.09).术后黄疸下降及营养改善,癌灶切除胆肠吻合组显著优于胆道引流组,但肝脏酶学的恢复两组间无差异.结论:扩大根治术能显著提高进展期胆囊癌侵犯肝门胆管致梗阻性黄疸患者的生存时间胆囊及肝方叶癌灶切除胆肠吻合的姑息性手术对于严格选择的病例亦能达到提高生活质量和改善预后的目的.