Management of ampullary neoplasms: A tailored approach between endoscopy and surgery

Ampullary neoplasms,although rare,present distinctive clinical and pathological features from other neoplastic lesions of the periampullary region.No specific guidelines about their management are available,and they are often assimilated either to biliary tract or to pancreatic carcinomas.Due to their location,they tend to become symptomatic at an earlier stage compared to pancreatic malignancies.This behaviour results in a higher resectability rate at diagnosis.From a pathological point of view they arise in a zone of transition between two different epithelia,and,according to their origin,may be divided into pancreatobiliary or intestinal type.This classification has a substantial impact on prognosis.In most cases,pancreaticoduodenectomy represents the treatment of choice when there is an overt or highly suspicious malignant behaviour.The rate of potentially curative resection is as high as 90% and in high-volume centres an acceptable rate of complications is reported.In selected situations less invasive approaches,such as ampullectomy,have been advocated,although there are some concerns mainly because of a higher recurrence rate associated with limited resections for invasive carcinomas.Importantly,these methods have the drawback of not including an appropriate lymphadenectomy,while nodal involvement has been shown to be frequently present also in apparently lowrisk carcinomas.Endoscopic ampullectomy is now the procedure of choice in case of low up to high-grade dysplasia providing a proper assessment of the T status by endoscopic ultrasound.In the present paper the evidence currently available is reviewed,with the aim of offering an updated framework for diagnosis and management of this specific type of disease.

Mucins in neoplasms of pancreas,ampulla of Vater and biliary system

Tumors of the pancreas,the ampulla of Vater,and the extrahepatic and intrahepatic bile ducts have significant histological similarities due to the common embryonic origin of the pancreatobiliary system.This obviates the need for discovery of biomarkers with diagnostic and prognostic value for these tumors.Mucins,especially MUC-1,-2,-4 and-5AC,are important candidates for developing into such reliable biomarkers.Increased expression of MUC1 occurs in pancreatic ductal adenocarcinomas and is associated with increased degrees of dysplasia in pancreatic intraepithelial neoplasia(Pan IN).Positive expression of MUC2 in intraductal papillary mucinus neoplasms(IPMN) of the intestinal type indicates high potential progression to invasive carcinoma with de novo expression of MUC1,while absence of MUC2 expression in IPMNs of gastric type implies low potential to malignant evolution.De novo MUC4 expression correlates to the severity of dysplasia in Pan IN and is associated with a poor prognosis in patients with pancreatic ductal adenocarcinomas.In biliary intraepithelial neoplasia(Bil IN),increased expression of MUC1 is associated with higher degrees of dysplasia.Intrahepatic cholangiocarcinomas(ICC) are characterized by increased expression of all glycoforms of MUC1.Positive MUC2 expression in intraductal papillary neoplasm of the bile ducts(IPNB) of the intestinal type indicates high malignant potential with de novo expression of MUC1 in the invasive element.Absent MUC2 expression in any degree of Bil IN may prove useful in differentiating them from IPNB.De novo expression of MUC4 is associated with poor prognosis in patients with ICC or carcinoma of the extrahepatic bile ducts(EHBDC).High de novo expression of MUC5 AC is found in all degrees of Bil IN and all types of IPNB and ICC.The MUC5 AC is useful in the detection of neoplastic lesions of the bile duct at an early stage.Increased expression of mucin MUC1 in carcinoma of the ampulla of Vater associated with unfavorable behavior of the tumor,such as lymph node metastasis,infiltration of the pancreas and duodenum,advanced TNM classification and worse prognosis.Patients withintra-ampullary papillary-tubular neoplasm(IAPN) of the pancreatobiliary immunophenotype did not show MUC2,while those of the intestinal immunophenotype are MUC2 positive.The expression of MUC4 is associated with poor prognosis in patients with carcinoma of the ampulla of Vater favoring metastasis and making them resistant to apoptosis.Moreover,it appears that MUC4 positivity correlates with recurrence of the tumor.Expression of MUC5 AC is associated with the invasive potential of the tumor.

Rare histological subtype of invasive micropapillary carcinoma in the ampulla of Vater: A case report

BACKGROUND Carcinoma of the ampulla of Vater is an uncommon ampullo-pancreatobiliary neoplasm,and the most common histological type is adenocarcinoma with a tubular growth pattern.Invasive micropapillary carcinoma(IMPC)is an aggressive variant of adenocarcinoma in several organs that is associated with lymph node metastasis and poor prognosis.IMPC was first described as a histological subtype of breast cancer;however,IMPC of the ampulla of Vater is extremely rare,with only three articles reported in the English literature.CASE SUMMARY We have reported a case of IMPC of the ampulla of Vater in an 80-year-old man.Microscopically,the surface area of the carcinoma was composed of tubulopapillary structures mimicking intra-ampullary papillary-tubular neoplasm,and the deep invasive front area exhibited a pattern of IMPC.The carcinoma showed lymphatic invasion and extensive lymph node metastasis.The immunohistochemical study revealed mixed intestinal and gastric/pancreatobiliary phenotypes.CONCLUSION This rare subtype tumor in the ampulla of Vater showed a histologically mixed phenotype and exhibited aggressive behavior.

壶腹部神经内分泌肿瘤的研究进展

壶腹部神经内分泌肿瘤(ampullary neuroendocrine neoplasm,A-NEN)发病率低、分类复杂且临床病理特点多样。随着A-NEN报道数量的增加,研究者对本病的认识正在不断深化。然而,由于一些临床工作者对A-NEN的认识存在不足,使得部分患者未能获得最佳的诊治。A-NEN发病初期的临床表现不典型,许多患者发现时已处于进展期。提高A-NEN的早期诊断率、明确A-NET的内镜治疗指征,以及寻找更有效的抗肿瘤治疗靶点是目前研究的重点。本文以各种类型A-NEN的临床病理特征为出发点,对诊断及治疗的相关进展进行综述,为A-NEN患者的个体化治疗提供参考依据。

局部切除术治疗Vater壶腹部肿瘤临床体会

目的评价局部切除术治疗Vater壶腹部肿瘤的效果。方法对1995年1月～2006年10月行局部切除治疗的19例Vater壶腹部肿瘤患者进行回顾性分析。结果全部病例无手术及住院死亡,术后并发症发生率为15.8%(3/19)。3例良性肿瘤已分别存活3年、6年和8年。16例恶性肿瘤中,1年生存率为87.5%(14/16);3年和5年生存率现已达50.0%(8/16),25%(4/16),其治疗效果满意。结论局部切除手术符合胆汁引流的生理状态,创伤相对小,恢复快,手术合并症及病死率也较低,因早期较小的肿瘤以及良性肿瘤局部恶变而肿瘤被完整切除,患者得到较长生存时间或得以治愈。

Vater壶腹部周围肿瘤54例诊断分析

目的探索有效的Vater壶腹部周围肿瘤的影响学诊断方法。方法回顾性分析2003年1月至2009年12月收治的54例Vater壶腹部周围肿瘤的临床资料。结果 B型超声诊断符合率为31.5%,CT为61.1%,MRI55.6%,经内镜逆行胰胆管造影(ERCP)为100%,超声内镜(EUS)为95.0%,磁共振胰胆管成像(MRCP)为57.4%。B型超声与其他方法的诊断符合率差异有统计学意义(P<0.05)。EUS与ERCP之间差异无统计学意义,但与其他方法的诊断符合率差异有统计学意义(P<0.05)。CT、MRI、MRCP之间的诊断符合率差异无统计学意义(P>0.05)。结论正确选择合适的检查方法及顺序,节约了时间和费用,减少了并发症,提高了手术的安全性和彻底性。

Vater壶腹癌23例报告

目的评估和总结Vater壶腹癌治疗的临床经验。方法回顾性分析23例Vater壶腹癌的临床资料。结果18例（78．3％）行经典的胰十二指肠切除术和保留幽门的胰十二指肠切除术；术后并发胰瘘8例（34．8％），并发胃排空延迟6例（26．1％），两种术式的并发症发生率相近；术后1年生存率为77．3％，术后5年生存率为60．0％。结论Vater壶腹癌术后生存率较高，手术方式与长期生存率、术后胰瘘等发生率无明显关系。

扩大Vater壶腹部切除的根治术治疗壶腹部肿瘤

目的 探讨Vater壶腹部肿瘤行壶腹部扩大切除的根治性手术的方法,以减少非肿瘤器官切除,并符合肿瘤治疗原则。方法 总结1995-1998年手术切除的根治术的经验。经十二指肠后外侧入路,整块切除肝外胆道、胆胰管汇合部,壶腹部及十二指肠乳头,十二指肠乳头封闭术。胆、胰、十二指肠间置空肠或胆、胰空肠Roux-en-Y吻合术。结果 围手术期死亡1例。并发症1例经再手术治愈。随访术后半年以上病人9例,最长生存29个月,无并发症及转移征象。结论（1）该术式符合胰十二指肠解剖关系;（2）按肿瘤治疗原则能达到广泛程度清扫;（3）初行该术式者应掌握胰十二指肠切除术。

Surgical method choice and coincidence rate of pathological diagnoses in transduodenal ampullectomy: A retrospective case series study and review of the literature

BACKGROUND Transduodenal ampullectomy(TDA) is not in wide clinical use due to its low radical effect and a high recurrence rate of tumors. However,TDA is still an effective treatment method; it has great clinical value in cases of duodenal benign tumors,precancerous lesions,and benign and malignant borderline tumors,and can avoid the risks associated with pancreaticoduodenectomy with larger resection range and greater thoroughness than endoscopic papillectomy.AIM To investigate the surgical method choice and the coincidence rate of pathological diagnoses in TDA for ampullary neoplasms.METHODS Ten patients with ampullary neoplasms underwent TDA based on the fact that their endoscopic biopsy results suggested benign lesions,and the endoscopic ultrasound(EUS)-assessed tumors were resectable. All cases underwent duodenal ampullary lesion endoscopic biopsy,intraoperative frozen-section pathological examination,and postoperative pathological examination.RESULTSThis study included seven patients with benign tumors and three with malignant tumors(1 pTis,2 pT1),according to the postoperative pathology results. The coincidence rate of the postoperative pathology results with the intraoperative frozen-section biopsy results was 100%(10/10),and the coincidence rate with the endoscopic biopsy results was 70%(7/10) based on pathological characteristics.The endoscopic biopsy false-negative rate was 30%(3/10). All patients were followed for 6 to 70 mo without tumor recurrence or metastasis.CONCLUSION The coincidence rate of postoperative pathology results,intraoperative frozensection pathology results,and endoscopic biopsy results is the restraining factor of TDA clinical application. Endoscopic biopsy results and EUS have importance relevance to surgical planning. Intraoperative frozen-section pathology results have a significant influence on the choice of surgical procedure.

Vater壶腹周围恶性肿瘤11例诊治体会

目的总结Vater壶腹周围恶性肿瘤的临床诊治方法。方法 2004年1月至2009年9月间,对渐进性无痛性黄疸病人通过B型超声、CT、磁共振胰胆管成像(MRCP)、血清学检查及肿瘤标记物检测,利用经内镜逆行胰胆管造影(ERCP)技术筛选出Vater壶腹周围恶性肿瘤。结果诊断Vater壶腹周围恶性肿瘤11例,其中6例行标准Whipple手术,行姑息减黄术2例,行内镜胆道内支架放置术(ERBD)3例;治疗效果明显,无胰漏、吻合口漏、出血等明显并发症发生。结论 ERCP技术在Vater壶腹周围恶性肿瘤的诊治中有其独到的价值,但仍然存在不足之处。

Comparison of clinicopathological characteristics between resected ampullary carcinoma and carcinoma of the second portion of the duodenum

BACKGROUND Few studies compared the oncological and biological characteristics between ampullary carcinoma(AC) and cancer of the second portion of the duodenum(DC-Ⅱ), although both tumors arise from anatomically close locations.AIM To elucidate differences in clinicopathological characteristics, especially the patterns of lymph node metastasis(LNM), between AC and DC-Ⅱ.METHODS This was a retrospective cohort study of 80 patients with AC and 27 patients with DC-Ⅱ who underwent pancreaticoduodenectomy between January 1998 and December 2018 in two institutions. Clinicopathological factors, LNM patterns, and prognosis were compared between the two groups.RESULTS The patients with AC and DC-Ⅱ did not exhibit significant differences in 5-year overall survival(66.0% and 67.1%, respectively) and 5-year relapse-free survival(63.5% and 62.2%, respectively). Compared to the patients with DC-Ⅱ, the rate of preoperative biliary drainage was higher(P = 0.042) and the rates of digestive symptoms(P = 0.0158), ulcerative-type cancer(P < 0.0001), large tumor diameter(P < 0.0001), and advanced tumor stage(P = 0.0019) were lower in the patients with AC. The LNM rates were 27.5% and 40.7% in patients with AC and DC-Ⅱ,respectively, without significant difference(P = 0.23). The rates of LNM to hepatic nodes(N-He)and pyloric nodes(N-Py) were significantly higher in patients with DC-Ⅱ than in those with AC(metastasis to N-HE: 18.5% and 5% in patients with DC-Ⅱ and AC, respectively;P = 0.0432;metastasis to N-Py: 11.1% and 0% in patients with DC-Ⅱ and AC, respectively;P = 0.0186)CONCLUSION Although there were no significant differences in the prognosis and recurrence rates between the two groups, metastases to N-He and N-Py were more frequent in patients with DC-Ⅱ than in those with AC.

壶腹癌并病理性胆肠瘘的术前诊断和治疗:附12例报告

目的探讨壶腹癌并病理性胆肠瘘的临床表现，以提高其术前诊断率。方法回顾性分析近9年间以壶腹部癌收治人院，术中诊断合并病理性胆肠瘘12例患者的临床资料。结果所有患者术前临床无阻塞性黄疸的症状及体征。B型超声检查提示壶腹部占位并肝内外胆管扩张，胆囊萎缩，与周围组织粘连，轮廓不清。胆肠瘘均在术中确诊，胆囊十二指肠瘘7例，胆囊结肠瘘5例，瘘口直径为0．4～2．8cm。4例行根治；8例姑息性手术。术后病理证实均为壶腹癌。结论对于超声检查有上述发现的老年患者，若临床无阻塞性黄疸的表现，往往反映病理性胆肠瘘存在。

壶腹部肿瘤的局部切除(附17例报告)

目的 评价局部切除术在壶腹部肿瘤治疗中的作用。方法 1987年 11月～1998年 12月为 17例乏特氏壶腹部肿瘤患者行肿瘤局部切除 ,其中良性腺瘤 3例 ,腺癌 14例。全部病例均经病理证实。分别采用 2种手术方法行肿瘤切除 ,16例行经十二指肠切开肿瘤切除术 ,1例行经胆总管下段肿瘤切除术。结果 本组术后发生胆道感染 3例 ,无手术死亡。 17例中 12例 (70 .6 % )获得随访 ,随访时间 4～ 6 1个月 ,中位时间 12个月。本组良性肿瘤 3例中 ,1例 17个月因其它疾病死亡 ,2例随访 38,7个月后仍健康存活。恶性 14例中随访 9例 (6 4 3 % ) ,生存≥ 1年者 6例 (6 6 .7% ) ,生存 2年 4例 (44 .4% ) ,生存 5年 2例(2 2 .2 % ) ;另 3例现已分别存活 9,8,6个月 ,仍健在。结论 局部切除术具有对病人创伤小 ,出血少 ,恢复快 ,对生理干扰轻的特点。但需有经验的高年资医生进行手术。适合非胰头部起源的壶腹周围肿物、壶腹周围良性肿瘤或病变 ,此外在病人年龄较大或伴有较严重的其它器官疾病的壶腹周围恶性肿瘤病人 ,在无周围浸润及淋巴结转移情况下也可行局部切除。对非典型性增生 ,术前。

壶腹及周围小病变CT与超声及病理对照分析

目的通过CT与超声及病理对照分析，评价CT及US对壶腹及周围较小病变的诊断价值．方法25例均先行超声检查，再行CT平扫加动态增强扫描，动态增强扫描的方法是：静脉团注65％安其格纳芬100mL后立即快速、4mm层厚及局部放大连续扫描．结果该组病例手术及病理结果为胰头癌9例、壶腹癌8例、胆管癌3例、十二指肠乳头炎2例、胰头炎性包块3例．25例定位诊断准确率CT为88．0％，超声为68．0％，定性诊断准确率CT为68．0％，US为56．0％，其中CT和US对胰头及壶腹定位、定性准确性较高，CT对十二指肠乳头炎及腹头炎性包决定位诊断较准确，但定性诊断的准确性差．US对胆总管下端肿瘤定位、定性均较准．该组25例病变大小均在1．2cm～3．0cm之间．结论壶腹及其周围小病变早期诊断有一定难度，CT与US是一种经济、方便且较准确的方法，如果密切结合临床表现，采用正确的检查方法及多种影像改变综合分析有助于提高诊断准确性．

十二指肠乳头肿瘤局部切除术32例临床分析

目的探讨十二指肠乳头肿瘤局部切除术的效果。方法自2000年1月至2009年12月,采用局部切除术治疗32例Vater壶腹部肿瘤,其中良性肿瘤10例,恶性肿瘤22例。对病人的临床表现、手术后并发症和生存情况进行观察。结果本组无手术死亡;术后发生并发症8例(25.0%);10例良性肿瘤经局部切除治愈;1例死亡;5年生存率为37.5%(12/32)。结论局部切除术治疗Vater壶腹部肿瘤创伤小,恢复快,效果满意,对于部分病人,尤其是老年病人和高危病人,是较好的手术方式。

MRI结合MRCP对乳头型壶腹癌的诊断价值

目的:探讨乳头型壶腹癌的MRI和磁共振胰胆管成像(MRCP)表现及诊断价值。材料与方法:回顾性分析经病理组织学检查证实的17例乳头型壶腹癌的MRI及MRCP表现,分析胆总管壶腹部病灶、胆管扩张形态、梗阻端形态以及胆管、胰管与十二脂肠之间的关系。结果:17例均清晰显示十二脂肠乳头、壶腹部局限性强化的充盈缺损团块影,肿瘤直径为1～4.2cm,平均2.1cm。所有病例均可见肝内胆管扩张、胆囊增大、胆总管扩张和12例胰管的扩张,1例伴胰体尾部的明显萎缩。结论:MRI结合MRCP不仅能准确确定梗阻平面,还能显示十二脂肠乳头部壶腹癌强化的肿块影,可作为乳头型壶腹癌的首选检查方法。

335例壶腹周围癌手术分析

目的 我院近十年来开展的335例壶腹周围癌的手术情况,以期能为逐步提高其外科手术治疗的安全性和疗效提供有益帮助。方法 分析1990年1月至1999年12月间在我院普外科施行手术的335例壶腹周围癌患者的临床资料,其中胰头癌237例,壶腹部癌98例。结果 胰头癌与壶腹部癌手术切除率分别为13.5％与66.3％,总手术切除率为29％,根治性手术死亡率为7.69％与5.45％,手术并发症发生率分别为26.9％与32.7％。本组手术并发症主要是:胰瘘、胆瘘、出血、腹腔感染、吻合口梗阻。结论 要最大限度地提高壶腹周围癌的手术切除率和长期生存率,降低手术死亡率、并发症发生率,除了提高早期诊断率以外,更重要的是一支专业手术组医师梯队的建立。

壶腹部癌的局部切除治疗——附11例报告

目的 :评价局部切除治疗壶腹部癌的效果。方法 :1996年 5月 - 2 0 0 0年 12月 ,16例壶腹部癌的患者进行局部切除术 ,获随访 11例 ,其中术后组织病理证实为高分化腺癌 7例 ,其中中分化腺癌 2例 ,低分化腺癌 2例。 11例均行十二指肠切开肿瘤切除术。结果 :本组无手术死亡 ,术后均获随访 ,随访时间 6～ 5 3个月。 3例分别于术后 9、18、2 7个月死于其他病 ,1例 2 1个月后复发死亡。其余 7例均在随访中 ,平均生存期 2 3 4个月。术后 1年生存 8例 (72 7% ) ,2年为 5例 (4 5 5 % ) ,4年为 2例(18 2 % )。结论 :局部切除具有手术操作简单、创伤小、恢复快、手术并发症及死亡率低等特点。适应于肿瘤较小、无壶腹周围浸润及淋巴结转移 。

壶腹周围肿瘤的再探查手术治疗(附58例报告)

目的:总结58例壶腹周围肿瘤再次手术探查的经验体会。方法:回顾性分析58例壶腹周围肿瘤病例再次手术的临床及预后资料。结果:再次手术距首次手术时间为0.75个月至9年,中位时间3个月。经术前影像学评估无肿瘤转移和胰周大血管包裹后由胰腺外科专业组医师再次手术,再切除率为91.4%(53/58),有3例联合肠系膜上静脉切除重建。手术死亡率为3.4%(2/58),术后并发症发生率为12.1%(7/58)。平均手术时间为(5.3±1.9)h;平均术中出血量为(1140±970)ml。随访表明:17例周围淋巴结转移术后存活9～18个月,平均(14.4±5.3)个月;无转移患者存活12～42个月,平均(28.2±15.8)个月。13例患者存活8～42个月,至今尚健在。结论:术前准确的影像学评估和手术医师的专业化是再探查切除肿瘤的关键,提高手术安全性可使再次手术切除肿瘤获得良好预后。

壶腹周围肿瘤染色体9p21缺失图谱的构建

目的:进一步限定壶腹周围肿瘤染色体9p21区域的缺失范围.方法:选择染色体9p21区域5个微卫星多态性标记,通过聚合酶链反应、聚丙烯酰胺凝胶电泳和银染法,检测35例壶腹周围肿瘤及其外周血杂合性丢失(LOH)状况.结果:50%(4/8)胰腺癌有至少一个微卫星位点的LOH,其中D9S974(37.5%)和D9S942(28.6%)丢失频率较高,并且有连续性丢失现象.62.5%(5/8)壶腹癌在部分或全部位点出现LOH,其中D9S942(42.9%)丢失频率最高,其次为IFNA(37.5%)和D9S171(37.5%).14.2%(1/7)胰岛素瘤有一个位点LOH.结论:壶腹周围肿瘤染色体9p21最小共同缺失区位于D9S974和D9S942位点之间,其中可能存在一个新的涉及该肿瘤发生的相关抑癌基因.