胰腺实性假乳头状瘤的诊断与治疗

目的 总结胰腺实性假乳头状瘤的诊断与治疗经验.方法 回顾性分析天津医科大学附属肿瘤医院2006年1月至2011年10月收治的24例胰腺实性假乳头状瘤患者的临床病理资料.结果 本组患者中男2例、女22例,中位年龄27.5岁(12～51岁).24例患者中11例肿瘤位于胰头,5例位于胰体,4例位于胰尾,3例位于胰体尾,1例位于胰颈体部.肿瘤直径平均为6.4cm(2.0～12.0 cm).该病无特异性临床表现,术前诊断主要依赖于彩超、CT、MR等影像学检查.所有患者均行手术切除治疗,经术后病理证实为胰腺实性假乳头状瘤.术后随访时间为2～55个月,中位随访时间27.2个月,23例患者无瘤生存,l例患者术后复发,带瘤生存. 结论 胰腺实性假乳头状瘤是一种少见的低度恶性胰腺肿瘤,多发于青年女性,完整手术切除可获得较好的预后.     

胰腺实性-假乳头状瘤的多层螺旋CT诊断

目的：探讨胰腺实性-假乳头状瘤的多层螺旋CT表现,提高其术前诊断准确率。方法：对7例胰腺实性-假乳头状瘤患者均行CT平扫、增强动脉期、增强静脉期扫描。结果：肿块位于胰腺头颈部2例,胰体尾部5例,均表现为胰腺囊、实性肿块,3例肿块内见钙化。增强后肿瘤实性部分及包膜在动脉期轻度强化,静脉期有较明显强化,囊变、坏死部分不强化。结论：胰腺实性-假乳头状瘤的多层螺旋CT表现有一定的特异性,对其术前诊断和鉴别诊断具有重要价值。     

胰腺实性假乳头状瘤临床和病理特点分析：附3例报告

目的：探讨胰腺实性假乳头状瘤临床和病理特点,并指导临床诊治。方法：回顾性总结胰腺实性假乳头状瘤3例,结合临床资料进行分析。结果：胰腺实性假乳头状瘤少见,临床症状不典型,细针穿刺诊断或术中快速诊断困难度大,手术治疗后,总体预后良好。结论：临床与影像学典型的病例要考虑胰腺实性假乳头状瘤存在的可能,胰腺实性假乳头状瘤的诊断以外科手术后病理学诊断为标准;瘤体巨大（最大直径>20 cm）并与周围器官紧密粘连时,要注意该肿瘤存在恶性的可能。

     

胰腺导管内乳头状黏液性肿瘤13例临床分析

目的总结胰腺导管内乳头状黏液性肿瘤(IPMT)的诊治经验,提高对该病治疗水平。方法自1985年5月至2004年3月共收治IPMT病人13例,男8例,女5例。所有病人均有不同程度的胰管扩张,8例进行了ERCP检查,发现胰管扩张,胰液溢出5例。结果除1人外,其他病人均接受了手术治疗,手术效果良好。术后病理诊断为胰头导管乳头状黏液性腺癌3例;胰头导管内乳头状黏液性腺瘤伴局部癌变2例;胰头导管内乳头状黏液性腺瘤伴不典型增生2例;胰头导管内乳头状黏液性腺瘤5例。结论IPMT与胰腺导管癌不同,ERCP有助于诊断本病,积极的手术治疗可获得良好的预后。     

胰腺实性假乳头状瘤的CT诊断

目的探讨胰腺实性假乳头状瘤的CT表现及影像学在鉴别诊断中的价值。方法回顾性分析经手术病理证实且临床和CT资料完整的胰腺实性假乳头状瘤患者6例。结果2例胰腺实性假乳头状瘤病灶位于胰头,1例位于胰体,2例位于胰尾,1例胰体尾部均受累,瘤体直径约2．13cm,平均约6．8cm。CT表现：6例均为囊实混合性,圆形或椭圆形。CT平扫实性结构呈低或等密度,动态增强后实性部分早期轻度强化,后期呈渐进性强化。囊变区始终不强化。肿瘤多有完整包膜,很少引起胆总管及胰管扩张,不侵及胰周围组织。结论CT对胰腺实性假乳头状瘤的诊断和鉴别诊断有一定的价值。     

胰腺导管内乳头状粘液性肿瘤的诊治

目的 总结胰腺导管内乳头状粘液性肿瘤（IPMT）的诊治经验,以提高对该疾病的认识和诊治水平。方法 自1993年5月至2000年8月共收治胰腺导管内乳头状粘液性肿瘤患者8例,男性6例,女性2例,年龄33-72岁,平均54岁,患者均行B超和CT检查,均提示有不同程度的胰管扩张,5例发现胰头囊实性占位,5例行ERCP检查,发现十二指肠乳头增大和粘液溢出者3例,血清CA19-9值6.5-108U/ml(平均66.88U/ml),CEA值有1例为32.2ng/L,其余均为小于15ng/L。结果 8例患者中只有3例在入院时诊断为IPMT,除1命名中层得拒绝手术外,7例均进行了手术治疗（胰十二指肠切除术）,术后病理诊断胰头导管内乳头状粘液性腺癌2例、胰头导管内乳头状粘液性腺瘤伴局部癌变2例、胰头导管内乳头状液性腺瘤伴不典型增生1例、胰头导内乳头状粘液性腺瘤2例,术后患者均健在,最长1例已6年,结论 胰腺有囊性占位伴胰管全程扩张的患者应考虑胰腺导管内乳头状粘液性肿瘤,ERCP发现十二指肠乳头有粘液溢出者可确诊该疾病,术前要鉴别良恶性较困难,手术切除是最有效的治疗,术后预后良好。     

腹部医学图像三维可视化系统在胰腺肿瘤诊断和可切除性评估中的作用

目的 研究腹部医学图像三维可视化系统(MI-3DVS)在胰腺肿瘤诊断及可切除性评估中的价值.方法 2008年11月至2009年8月应用MI-3DVS对12例胰腺肿瘤患者的64排螺旋CT扫描数据进行三维重建,对胰腺肿瘤进行辅助诊断及可切除性评估,并与手术所见及病理检查结果进行对照.男性7例,女性5例,年龄14～83岁,平均45岁.其中胰腺癌4例、胰腺实性假乳头状瘤5例、浆液性囊腺瘤2例、胰腺囊肿(导管上皮乳头状增生)1例.结果 12例患者中,术前评估可切除的9例均成功切除肿瘤;2例术前评估不可切除,术中得到证实,仅行姑息手术;未手术1例.3例被外院判为不可切除的胰腺肿瘤患者,三维重建显示巨大肿瘤对相邻大血管为严重压迫,成功切除了肿瘤.结论 MI-3DVS重建对胰腺肿瘤的诊断和可切除性的评估有较高应用价值并具显著临床意义.     

胰腺实性假乳头状瘤的诊断和外科治疗

目的 总结胰腺实性假乳头状瘤的诊断和外科治疗经验.方法回顾性分析2002年1月至2006年10月间20例胰腺实性假乳头状瘤患者诊治的临床资料.结果 20例患者中女性18例,男性2例,平均年龄29.5岁.临床症状无特异性,主要为上腹部疼痛不适、腹胀和腰背痛.术前诊断主要依赖影像学检查,B超发现胰腺低回声实性或囊实性占位,边界清,内部回声不均匀;CT检查肿瘤多为囊实性占位,实质部分为低密度影,边界清楚,增强后肿瘤周边或实质内出现不规则强化.20例患者均接受手术治疗,完整切除肿瘤,术后病理证实为胰腺实性假乳头状瘤.随访时间为4～54个月,平均25.1个月,所有患者均存活,无复发或转移表现.结论胰腺实性假乳头状瘤是一种少见的胰腺肿瘤,多发生于年轻女性,正确的诊断和根治性手术切除可改善其预后.     

原发性输尿管肿瘤的诊断和治疗

原发性输尿管肿瘤是泌尿系统少见的和诊断困难的疾患。其病因还不清楚。芳香氨可能是致癌物质,结石可能在罕见的扁平上皮癌成为致病因素。形态学方面可将上皮性肿瘤分为下列几组:1.乳头状肿瘤:乳头状瘤(良性,无浸润性),乳头状癌(有全部恶性特征)。2.原发性浸润性癌。由于乳头状瘤转变为乳头状癌的过程不很清楚,而组织学方面的鉴别又很困难,因而对这两类肿瘤一般是不加以区分的。所谓良性乳头状瘤都具有明显的恶变     

胰腺实性假乳头状瘤的诊断和治疗

目的 总结胰腺实性假乳头状瘤的临床诊治经验,并提出较符合我国国情的诊治流程.方法 回顾性分析2001年1月至2007年3月收治的50例胰腺实性假乳头状瘤患者的临床资料.其中男3例,女47例;中位年龄24岁(13～60岁).术前B超、CT等检查均有特征性影像学表现,血清肿瘤标志物多为阴性.肿瘤位于胰头部23例、胰颈部3例、钩突部2例、胰体部3例、胰尾部18例、原发病灶位置不明1例.48例患者行手术切除肿瘤,1例行剖腹探查术,1例行CT引导下转移病灶穿刺活组织检查术.结果 50例患者中49例获得了"胰腺占位"的术前诊断.32例肿瘤包膜完整的患者有18例、16例肿瘤包膜不完整患者有11例未行冷冻病理检查而直接选择相应术式.32例患者随访3～55个月,均未发现肿瘤复发、转移.结论 胰腺实性假乳头状瘤多发于青年女性,CT检查结果最具诊断价值,外科手术切除肿瘤是首选治疗方式,预后良好.常用的术式为保留十二指肠的胰头切除术与胰体尾+脾切除术.肿瘤包膜是否完整,决定是否行术中冷冻病理检查和指导手术方式的选择.最常见的并发症为胰瘘.     

Paranuclear dot-like immunostaining for CD99: a unique staining pattern for diagnosing solid-pseudopapillary neoplasm of the pancreas

Solid-pseudopapillary neoplasm of the pancreas is a rare tumor of uncertain histogenesis that is characterized by a cystic and solid growth pattern with pseudopapillary structures. The differentiation of solid-pseudopapillary neoplasm from other pancreatic tumors is of great importance. However, it is sometimes difficult because of similarities in morphologic features and immunophenotype. CD99 is a diagnostically useful marker for Ewing sarcoma/primitive neuroectodermal tumor. The aim of this study was to investigate the diagnostic value of CD99 in solid-pseudopapillary neoplasm. We investigated immunohistochemical staining for CD99 in tissue microarray blocks from 55 cases of pancreatic solid-pseudopapillary neoplasm, 51 cases of pancreatic neuroendocrine tumor, and 54 cases of pancreatic adenocarcinoma. Biopsy specimens from 7 solid-pseudopapillary neoplasms, 6 acinar cell carcinomas, and 1 pancreatoblastoma were also investigated. All the solid-pseudopapillary neoplasm cells exhibited paranuclear dot-like immunoreactivity for CD99 regardless of the clinicopathologic or morphologic features. Forty of the 51 pancreatic neuroendocrine tumors were positive for CD99. Staining here was membranous, or membranous and cytoplasmic. Four of the 54 pancreatic adenocarcinomas and 1 pancreatoblastoma showed faint membranous staining. None of the acinar cell carcinomas was reactive for CD99. Our study has identified for the first time that pancreatic solid-pseudopapillary neoplasm exhibits a unique dot-like staining pattern for CD99. This could prove to be the most useful aspect of its immunoprofile for the definitive diagnosis of solid-pseudopapillary neoplasm and differentiation from other pancreatic tumors.     

Rare tumors of the pancreas

From 1984 to 2001, 486 operations were carried out at the surgical clinic in Gera for pancreatic neoplasms, including 49 patients with rare neoplasms of the pancreas. In 23 patients malignant pancreatic tumors were present (9 solitary metastases, 9 endocrine carcinomas, 2 cystadenocarcinomas, 2 schwannomas and one non-Hodgkin's lymphoma). In 28 benign lesions a resection of the tumor was performed, concerning 8 insulinomas, 8 serous cystadenomas, 3 mucinous cystadenomas and 4 rare cystic tumors. A sarcoidosis, an autoimmune pancreatitis and a radiation fibrosis were diagnosed in 3 patients operated under the suspicion of a malignant pancreatic tumor. By means of own case examples and data from the literature these rare entities are described with their diagnostic and therapeutic special features.     

Peripancreatic paraganglioma: a potential diagnostic challenge in cytopathology and surgical pathology

Paragangliomas are rare neuroendocrine neoplasms arising in extra-adrenal chromaffin cells of the autonomic nervous system. In rare instances, paragangliomas present around and involve the pancreas, thereby mimicking one of the more common primary pancreatic lesions. These neoplasms present considerable diagnostic difficulty not only for the clinician and radiologist but also for the pathologist. We have collected a series of 9 peripancreatic paragangliomas clinically simulating a primary pancreatic lesion. The paragangliomas were diagnosed in 4 men and 5 women with an age range of 37 to 78 years (mean, 50 y). Patients presented clinically either with diffuse epigastric and abdominal pain (7 of 9, 78%) or with an incidental mass (2 of 9, 22%) discovered on routine radiographic imaging. All patients were found to have mass lesions suspicious for a primary pancreatic neoplasm on radiographic examination. The lesions were predominantly located in the body of the pancreas (5 of 9, 56%) and ranged in size from 5.5 to 17.0 cm (mean, 10.0 cm). Five of 9 (56%) neoplasms also demonstrated cystic change. Fine-needle aspiration (FNA) was performed on 6 cases; however, the diagnostic accuracy was low, with 3 of 6 (50%) neoplasms misdiagnosed as pancreatic neuroendocrine tumor (PanNET) (n=1), spindle cell neoplasm (n=1), or pseudocyst (n=1). In addition, 2 of 8 (25%) surgically resected tumors were misdiagnosed by the referring pathologist as a PanNET. Immunohistochemistry was performed on all cases, confirming the characteristic 2-cell populations: chief cells (synaptophysin positive and chromogranin A positive) and sustentacular cells (S-100 protein positive). Follow-up information was available for all patients and ranged from 2 months to 11.6 years (mean, 2.7 y). Three of 9 (33%) patients developed metastatic disease, and 2 of these 3 died of their disease at 2.8 and 4.6 years after diagnosis. In summary, in unsuspected cases, interpretation of FNA and surgical pathology resections can be diagnostically challenging. Awareness and proper recognition of this entity, including differential diagnosis, are imperative in establishing the correct diagnosis. Further, close follow-up of these cases should be considered because of the significant risk of metastatic disease.     

A 20-year review of pediatric pancreatic tumors.

Pancreatic tumors are rare surgical problems in infants and children. A 20-year audit (1971 to 1991) of this institution showed six patients ranging in age from 3 weeks to 16 years who were operated on for pancreatic neoplasms. Five of these tumors were malignant, bringing the reported experience to 71 cases. This series of malignancies included three solid cystic tumors, one insulin-secreting tumor, and one pancreatoblastoma. The clinical presentations varied: three had abdominal pain, one developed hypoglycemia, and one had an abdominal mass with jaundice. In five of the six patients pancreatic pathology was suspected preoperatively. All were treated primarily with pancreatic resection including one pancreatoduodenectomy. No radiotherapy or chemotherapy was used. The perioperative mortality was 0% with a morbidity of 50%. The long-term results are encouraging, with all patients alive after a mean follow-up of 7.8 years. These data suggest that aggressive surgical therapy is warranted in the management of pediatric pancreatic tumors.     

无功能性胰腺神经内分泌肿瘤的MRI表现及治疗

[摘要] 目的 探讨无功能性胰腺神经内分泌肿瘤（non-Punctioning pancreatic neuroendocrine neoplasms,NF-pNENs）的MRI表现及治疗措施,提高诊疗水平。方法 回顾性分析11例经病理学证实的无功能性胰腺神经内分泌肿瘤的MRI及临床资料。结果 11例肿瘤均为单发,7例呈类圆形,4例呈不规则分叶状。7例边界较清晰,4例局部侵犯导致边界不清。肿瘤最大直径约23～137 mm,平均60.1 mm。其中实性5例,囊实性5例,囊性1例。MRI平扫肿瘤实性成分呈不均匀T1WI较低T2WI较高信号,囊变、坏死区为T1WI低T2WI高信号,DWI均为不均匀高信号。增强实性部分7例明显持续强化,3例呈中等持续强化,1例轻度延迟强化。9例见完整或不完整的包膜结构较明显强化,3例见胆总管和（或）主胰管增宽,3例肝脏转移,2例腹膜后淋巴结转移,2例腹腔少量积液。11例均经手术治疗,9例达到根治性切除。术后7例接受全身化疗,4例仅接受中医药治疗。术后随访6～63个月,3例死亡,平均生存时间35.7个月;8例仍存活。结论 MRI有利于反映NF-pNENs的病理学变化,对其诊断具有重要价值。手术切除是NF-pNENs的主要治 疗手段,一旦确诊应争取根治性切除。     

胰腺囊性肿瘤的诊断与治疗

目的探讨胰腺囊性肿瘤的诊断与外科治疗。方法回顾性研究北京大学第一医院外科1994—2004年所收治45例胰腺囊性肿瘤的临床资料。结果本组病例涉及8种不同囊性肿瘤，男12例，女33例，平均年龄49．1岁，临床表现无特异性。B超及CT检查提示囊性或囊实性占位，ERCP、EUS等可提供鉴别诊断线索。根据肿瘤位置及性状选择局部切除术13例，Whipple术12例，胰体尾切除术18例，全胰腺切除术1例，单纯活检术1例。无围手术期死亡，术后胰瘘2例经保守治疗痊愈。随访38例，平均32．6个月。随访期内死亡4例，其中3例死于肿瘤复发，其余病人无瘤生存。结论胰腺囊性肿瘤包括多种亚型，临床表现均无特异性，诊断须综合各项检查结果，诊断明确者应积极手术治疗，切除率高，预后较好。     

超声内镜与CT对胰腺及壶腹部周围肿瘤的术前诊断价值

目的评估超声内镜(endoscopic ultrasonography,EUS)和CT对胰腺及壶腹部周围肿瘤的术前诊断价值。方法回顾性分析33例胰腺及壶腹部肿瘤患者术前EUS、CT资料,与手术探查及术后病理结果对照,从肿瘤大小、部位等角度筛选出影响EUS准确性的因素。结果 EUS在判断胰周脂肪浸润、胆管扩张、胰周脏器侵犯、血管侵犯等方面敏感性、特异性与CT的差异无统计学意义(P>0．05);EUS在胰管扩张及淋巴结转移方面的诊断价值优于CT(P=0．039和P=0．004); EUS判断胰周脂肪浸润和胰周脏器侵及的准确性与肿瘤大小有关(P=0．015和P=0．022),判断胰管扩张的准确性与肿瘤部位有关(P<0．001)。结论 EUS对胰腺及壶腹部周围肿瘤诊断的临床价值很高,结合CT检查有助于加强对患者术前评价的认识。     

胰腺囊性肿瘤的诊治研究

目的 初步探讨胰腺囊性肿瘤的诊治策略.方法 回顾性分析我院近十年来收治的21例胰腺囊性肿瘤的病例资料.结果 21例胰腺囊性肿瘤患者中胰腺浆液性囊腺瘤11例,黏液性囊腺瘤6例,胰腺导管内乳头状黏液性肿瘤2例,胰腺实性假乳头状瘤2例;其中仅7例患者伴有相应的症状和体征,其他均为健康体检时所发现.所有患者均经手术治疗,其中8例行胰十二指肠切除术、5例行胰体尾切除术、1例行腹腔镜下胰尾切除术、2例行胰腺肿瘤摘除术、4例行胰腺节段切除术;1例囊腺癌患者仅可行姑息手术,其余20例患者随访11～96个月,均未出现肿瘤复发或转移.结论 任何影像学检查都不能准确判断胰腺囊性肿瘤的具体病理类型;及时手术探查是防止肿瘤癌变的重要手段,除了已经获得明确诊断、肿瘤较小且无明显症状的浆液性囊腺瘤之外,都应该积极手术治疗.     

动态增强MSCT对胰腺导管内乳头状黏液癌的诊断分析

目的 分析胰腺导管内乳头状黏液癌(IPMC)的多层螺旋CT(MSCT)表现,以提高其诊断水平.方法 对经手术病理证实的12例IPMC的MSCT资料进行回顾性分析.结果 12例患者中主胰管型7例,表现为主胰管不规则明显扩张,胰管内呈较均匀低密度,多数可见管壁小结节,CT呈稍高密度;分支胰管型4例,表现为分叶状单发或多发囊性低密度病变,腔内见分隔及结节样突起或肿块,呈稍高密度,2例CT的MPR图像显示病灶与主胰管相通;混合型1例,表现为胰头部囊状病灶,内有附壁结节,合并主胰管轻度扩张,内呈较均匀低密度.8例病灶主胰管不同程度扩张(4~68 mm),7例胰腺不同程度萎缩,4例显示十二指肠乳头膨大,2例见不规则点状钙化.增强扫描囊内分隔强化较明显,附壁结节强化稍弱且不均匀,以胰腺期较明显.结论 MSCT薄层扫描对IPMC诊断价值较大,结合MPR、CPR图像能够较清晰显示胰腺 IPMC的病理特征,有利于显示胰管壁小结节及胰管扩张、十二指肠乳头改变,多数可与胰腺导管内良性乳头状黏液性肿瘤鉴别.     

The prevalence of pancreatic intraepithelial neoplasia in pancreata with uncommon types of primary neoplasms

Pancreatic ductal adenocarcinoma is thought to develop through a series of genetic events through its purported precursor lesion, pancreatic intraepithelial neoplasia (PanIN). Little, however, is known regarding the role of possible precursor lesions in the development of other primary neoplasms of the pancreas. This study investigated the prevalence of PanIN, as defined by recent consensus statements, in pancreata with uncommon types of primary neoplasms. All pancreata resected at the University of Virginia from June 1, 1991 to March 1, 2005 for neoplasia not diagnosed as conventional ductal adenocarcinoma were reviewed and classified according to the World Health Organization's classification schema for tumors of the exocrine and endocrine pancreas. All slides from these cases were then assessed for PanIN, which was classified according to the criteria of the most recent consensus statement. Three acinar cell carcinomas (ACCs), 18 mucinous cystic neoplasms (MCNs), 24 pancreatic endocrine tumors (PETs), 12 serous cystadenomas (SCs), and 3 solid-pseudopapillary tumors (SPTs) were identified. PanIN was identified in the pancreata of 3 of 3 ACCs, 17 of 18 MCNs, 16 of 24 PETs, 10 of 12 SCs, and 2 of 3 SPTs. The degree of PanIN was noted to trend with patient age. Although the high prevalence of PanIN in pancreata concomitantly harboring certain uncommon neoplasms of the pancreas could signify its role as a precursor lesion for those neoplasms, its high prevalence throughout our series may simply be the result of a coincidental, prevalent finding seen in all pancreata, especially with aging. Because of the ubiquitous nature of PanIN, it should not be used histologically to assist in the diagnosis and subclassification of pancreatic neoplasia.