小儿胰腺恶性肿瘤11例诊治分析

目的探讨小儿胰腺恶性肿瘤的临床特征和诊治方法。方法选取1992年7月-2004年5月收治的11例小儿胰腺恶性肿瘤。男5例,女6例;年龄3～14岁。7例患儿表现为腹痛,5例表现为腹部包块;其他症状包括黄疸3例,呕吐、厌食和发热各2例。11例患儿中,5例行胰十二指肠切除术,3例行脾及胰体尾切除术,2例行肿瘤局部切除术,1例仅行病理活检术,并对3例患儿分别给予化疗或放疗。结果11例患儿病灶术前至少一项影像学检查定位于胰腺,3例术前B超误诊为后腹膜肿块;术中探查可见5例患儿肿瘤位于胰体部,3位于胰头体部,2例位于胰尾部,1例位于胰头部;瘤体直径4～12cm,平均长径约6.5cm;肿瘤主要侵犯十二指肠和脾脏,均无远处转移。肿瘤类型包括囊实性乳头状肿瘤4例,胰母细胞瘤3例,无功能胰腺内分泌肿瘤2例,腺泡细胞癌及不典型胰腺类癌瘤各1例。术后随访2个月～9a,4例死亡,7例健在,其中1例复发后行胰头部和十二指肠切除处理。结论儿童胰腺恶性肿瘤少见,类型多样,完整切除后能长期生存,预后较成人好,但存在复发的可能,同时化疗和放疗的效果还不确定。     

小儿胰腺实性假乳头状瘤的诊断与外科治疗

目的 总结小儿胰腺实性假乳头状瘤(solid pseudo-papillary tumors of pancreas,SPTP)的诊断及治疗经验.方法 回顾性分析2002年1月至2011年12月9例胰腺实性假乳头状瘤患儿的临床资料.其中,男1例,女8例;中位年龄11岁5个月.结果 小儿SPTP无特异性临床表现,主要临床表现为腹痛或腹部不适,CT检查均提示胰腺囊实性或实性占位.手术证实肿瘤位于胰头、颈部4例,胰体部2例,胰尾部3例.肿瘤直径2.8～16.4 cm,无腹腔或肝脏转移.行胰十二指肠切除术(Child术)2例,肿瘤局部切除术4例,胰腺节段切除、胰肠吻合术1例、胰尾联合脾脏切除术1例,保留脾脏的胰体尾切除1例.9例肿瘤标本在显微镜下均具有SPTP的典型病理学表现.随访3个月～8年,均未发现肿瘤复发及转移.结论 CT是小儿SPTP重要的诊断方法,手术为其主要治疗方式,手术术式的选择应依据术前CT和术中对肿瘤部位、包膜是否完整和是否侵及周围组织的探查来决定.     

保留脾脏的胰体尾部肿瘤切除术治疗小儿胰腺实性假乳头状瘤

目的探讨保留脾脏的胰体尾肿瘤切除术治疗小儿胰腺实性假乳头状瘤的可行性及临床疗效评估。方法回顾性分析2009年1月-2012年12月在天津市儿童医院收治的位于胰体尾部胰腺实性假乳头状瘤患儿4例,均成功行保留脾脏的胰体尾肿瘤切除术。术后均在门诊随访,内容包括腹部B超和CT检查,肿瘤标记物和胰腺外分泌功能,并由专人测量其生长发育情况。结果患儿手术过程顺利,切除肿瘤及部分胰体尾,全部成功保留脾脏,肿瘤直径介于4．0～12．5em,包膜完整,手术过程中无脾脏动、静脉损伤及脾实质撕裂伤,无术中输血。术后3d肠功能恢复后开始进食,恢复过程顺利,住院时间为6～9d。无围手术期出血、胰瘘等并发症发生。病理检查结果均为胰腺实性假乳头状瘤,Ki-67〈5％。所有患儿获得随访,时间为6～48个月,均健康存活,生长发育正常。腹部B超、CT检查未见肿瘤复发,肿瘤标记物及胰腺外分泌功能检查未见异常。结论发生在胰体尾部的胰腺实性假乳头状瘤虽然肿瘤直径较大,但是包膜完整,与胰腺血管关系疏松,易于将脾脏动静脉分开而成功保留脾脏。     

腹腔镜在治疗小儿胰腺疾病中的应用

目的 探讨腹腔镜手术治疗小儿胰腺疾病的临床效果.方法 2005年10月至2010年12月应用腹腔镜手术治疗小儿胰腺疾病9例,其中胰腺假性囊肿1例,行囊肿内引流术;局灶性胰岛细胞增生症1例,行单纯胰腺肿瘤切除;弥漫性胰岛细胞增生症2例,胰腺假乳头状瘤3例,行保留脾脏的胰体尾切除术;胰管扩张2例,行扩张胰管空肠侧侧Roux...     

小儿胰腺肿瘤的诊断与治疗(附15例报告)

目的 探讨小儿胰腺肿瘤的诊断和治疗.方法 回顾性分析2002年1月至2010年1月收治的15例胰腺肿瘤,男8例,女7例;年龄45 d至17岁(平均11.3岁).临床表现:腹痛9例,2例合并黄疸;腹部包块6例.B超和CT检查均为胰腺占位性病变.手术方式:腹腔镜胰腺肿瘤切除术6例;胰腺体尾部切除术4例;肿瘤剔除术4例;Whipple术1例.结果 本组患儿无围手术期死亡.病理:胰腺实性乳头状瘤8例,胰腺母细胞瘤4例,胰腺血管瘤1例,胰腺无功能性内分泌癌1例,胰腺癌1例.术后随访1年至8年,患儿全部存活.14例未见复发和转移,1例胰腺癌化疗后2年肝转移,行转移瘤局部切除术后治愈.结论 小儿胰腺肿瘤罕见,临床多见胰腺实性乳头状瘤和胰腺母细胞瘤.腹腔镜和开腹根治性手术均可获得良好的治疗效果.胰腺癌根治性手术后易复发,需长期随访.     

保留十二指肠胰头肿块切除术在儿童胰头肿瘤治疗中的应用

目的 通过对比保留十二指肠的胰头肿块切除术与胰十二指肠切除术治疗儿童胰头肿瘤的术后并发症及治疗效果,探讨保留十二指肠胰头肿块切除术在儿童患者中的应用价值.方法 回顾性地分析了复旦大学附属儿科医院从2007年到2015年,收治并行保留十二指肠的胰头肿块切除术与胰十二指肠切除手术治疗的胰头部肿瘤患儿的临床资料.我们定义保留十二指肠肿块切除术为:①肿块剜除术;②保留十二指肠的胰头切除术(duodenum-preserving pancreatic head resection,DPPHR).胰十二指肠切除术均为保留幽门胰十二指肠切除术(pylorus-preserving pancreaticoduode-nectomy,PPPD).结果 共收集11例患儿临床资料,平均年龄(8.4±3.7)岁.其中7例行保留十二指肠手术,术后病理证实4例为实性假乳头状瘤;3例为胰母细胞瘤;4例行PPPD,3例病理结果为实性-假乳头状瘤;1例为炎性肌纤维母细胞瘤.保留十二指肠组患儿平均肿瘤最长径为:(9.4±4.5)cm,PPPD组最长径为(5.9±2.1)cm,两组肿瘤长径无统计学差异(P=0.17).保留十二指肠组手术平均时间为(6.0±3.5)h,而PPPD组平均手术时间为(7.5±1.3)h,两组手术时间差异无统计学意义(P=0.44).根据国际胰漏小组的定义,4例行保留十二指肠手术的患儿出现A级胰瘘,pp-PD组患儿均无术后胰瘘.根据国际胰腺外科研究小组的定义,11例患儿均未出现临床相关胃排空延迟(B级或C级).除1例炎性肌纤维母细胞瘤失访外,余10例患儿术后经B型超声、CT等影像学检查随访时间为4个月到32个月,均未出现肿瘤复发.临床症状随访时间为6个月到4年,10例患儿均存活,且未诉与疾病相关不适.结论 针对儿童胰头肿瘤,根据术中情况,在肿瘤未侵犯十二指肠且能保证胆道及十二指肠血供的前提下,采取保留十二指肠的胰头肿块切除术,能减少手术创伤,保留胆道及胃肠道的完整性.     

小儿慢性胰腺炎12例

目的总结小儿慢性胰腺炎的临床经验,探讨其诊断与手术治疗方法。方法回顾性分析12例慢性胰腺炎患儿的临床资料,其中男性9例,女性3例。平均年龄11.4岁,病程17.1个月。均因反复发作上腹痛入院。B超、CT或MRCP显示存在胰腺钙化2例,胰管扩张4例,胰管结石4例,胰腺假性囊肿5例。6例1期患儿予保守治疗;6例2期患儿予手术治疗,其中胰管切开取石、胰管空肠侧侧吻合术3例,胰体尾、脾切除术2例,腹腔引流术1例。结果 6例保守治疗患儿症状完全缓解后出院。6例手术治疗患儿中,除1例胰头部结石嵌顿未能取尽外,其余均恢复良好。12例患儿均获得6个月至5年随访,平均随访时间2.4年,其中3例复发腹痛,其余无临床症状。结论小儿慢性胰腺炎多依靠影像学检查确诊。合并胰管结石者首选胰管切开取石、大口径胰管空肠吻合术,可有效缓解疼痛,创伤小,并发症少。     

儿童Ⅲ型胰腺外伤六例治疗体会

目的 探讨儿童Ⅲ型胰腺外伤的治疗方法.方法 回顾性分析2010年1月至2015年6月收治的6例Ⅲ型胰腺外伤患儿的临床资料.本组均为男孩,年龄6岁4个月～11岁5个月.入院时均有不同程度腹痛,伴呕吐2例,伴发热1例;血淀粉酶(695.83±264.07) U/L,尿淀粉酶(4 163.67±5 546.53)U/L.CT检查,均提示胰管损伤可能,其中3例分别合并脾挫伤、左肾上腺挫伤加肺挫伤、左肾上腺挫伤.患儿均先给予生长抑素、禁食、静脉营养等保守治疗,但症状控制不满意.待血流动力学稳定后行逆行胰胆管造影(ERCP)加内镜鼻胰管引流术或胰管支架引流术.结果 6例ERCP造影提示1例胰头部、4例胰体部、1例体尾部主胰管损伤.行13次ERCP引流,2例经7次ERCP引流治愈;另4例6次ERCP引流症状控制不佳,3例穿刺引流,1例开放引流后治愈.6例随访胰管均修复、恢复通畅;2例超声检查示胰腺有局部细小囊性改变,无临床症状,淀粉酶恢复正常.结论 单独ERCP引流治疗儿童Ⅲ型胰腺外伤疗效不满意,有待更多临床总结;结合经皮穿刺引流和开放引流效果确切.儿童Ⅲ型胰腺外伤使用药物治疗加确切引流就能取得良好预后,应尽量避免复杂手术.     

小儿腹腔镜保脾胰腺体尾部切除手术七例

目的 探讨儿童胰腺体尾部病变行保留脾脏和脾血管的腹腔镜胰体尾切除手术的可行性与安全性.方法 以2015年1月至2020年12月湖南省儿童医院收治的7例胰腺体尾部实性假乳头状瘤患者为研究对象,7例均采用保留脾脏与脾动脉或脾动脉、脾静脉的方法切除胰腺体尾部,保留胰腺头颈部,保留脾脏和脾动脉、动静脉;2例保留脾脏和脾动脉.结...     

小儿胰腺乳头状囊性实性肿瘤

目的：探讨小儿胰腺乳头状囊性实性肿瘤的发病率、临床病理、免疫组化、诊断和治疗特点。方法：手术及病理诊断11例。肿瘤标本进行HE、PAS染色及免疫组化染色，抗体选用神经元特异性烯醇化酶（NSE）、α-1抗胰蛋白酶（α-1AT）、S-100、嗜铬粒素A(CgA)、细胞角蛋白（CK）、上皮膜抗原（EMA）、胰岛素。结果：11例患儿中，男1例，女10例，年龄9-14岁，平均年龄11.1岁。该肿瘤占儿童胰腺实体瘤的33.3％。多以腹痛、腹块为首发症状，肿瘤多位于胰头，瘤体较大，平均直径8.7cm。6例行肿瘤切除术，2例行远端胰腺切除术，3例行胰十二指肠切除术。11例患儿生存至今，随访1-15年。镜下观察：11例肿瘤均以实性区、囊性区及乳头状突起为其特征。免疫组化：α-1抗胰蛋白酶标志阳性率100％，神经元特异性烯醇化酶66.7％、S-10028.6%,PAS染色均呈阳性。结论：胰腺乳头状囊性实性肿瘤位居儿童胰腺实体瘤第二位，多发生于女孩，具有良性临床病程，手术治愈率高，预后良好。胰腺囊实性肿瘤起源尚有争议，有等进一步研究。     

Melanotic Neuroectodermal Tumor of Infancy: A Molecular Genetic Study

Melanotic neuroectodermal tumor of infancy is a rare but well-recognized entity in pediatric pathology. However, the relationship of this tumor to other pediatric small cell tumors with neuroectodermal features (such as neuroblastoma, Ewing sarcoma/peripheral primitive neuroectodermal tumor, and desmoplastic small round cell tumor) is undetermined. Molecular genetic studies of melanotic neuroectodermal tumor of infancy have not been reported. We studied three typical cases of melanotic neuroectodermal tumor of infancy in an attempt to link this tumor to other small cell tumors with well-characterized molecular genetic changes. Tests performed included: detection of MYCN gene amplification and deletion of 1p (all 3 cases), and presence of the t(11;22)(q24;q12) and the t(11;22)(p13;q12) translocations (2 of 3 cases). None of these tests yielded positive results. Thus, there is no genetic basis at present to link melanotic neuroectodermal tumor of infancy to neuroblastoma, Ewing sarcoma/peripheral primitive neuroectodermal tumor, or desmoplastic small round cell tumor. Received June 30, 1997; accepted September 19, 1997.     

Imaging of pediatric abdominal soft tissue tumors: A COG Diagnostic Imaging Committee/SPR Oncology Committee White Paper

This paper provides imaging recommendations for pediatric abdominal tumors that arise outside of the solid viscera. These tumors are rare in children and have been categorized in two groups: abdominal wall and peritoneal tumors (desmoid tumor and desmoplastic small round cell tumor) and tumors that arise from the gastrointestinal tract (gastrointestinal stromal tumor and gastrointestinal neuroendocrine tumor). Authors offer consensus recommendations for imaging assessment of these tumors at diagnosis, during follow-up, and when off-therapy.     

Management of childhood pancreatic disorders: a multidisciplinary approach

INTRODUCTION: Data on therapeutic endoscopy and radiologic interventions for the management of childhood pancreatic disorders are relatively limited. This study focuses on the multidisciplinary approach to the management of pancreatitis in children. PATIENTS AND METHODS: Children with pancreatic disorders were studied from January 1992 to May 2001. Acute pancreatitis (AP) was diagnosed by clinical evaluation, serum amylase more than three times normal, and morphologic abnormalities of the pancreas on imaging. Children with recurrent abdominal pain, pancreatic calcification or ductal stones on imaging, and pancreatic ductal changes on endoscopic retrograde cholangiopancreatography (ERCP) were diagnosed with chronic pancreatitis (CP). Patients were treated by gastroenterologists, surgeons, and interventional radiologists. Pancreatic exocrine insufficiency was diagnosed in appropriate settings. RESULTS: Fifteen children--6 with AP (posttrauma, 3; gallstone disease, 1; and viral, 1), 7 with CP, and 2 with pancreatic exocrine insufficiency--were diagnosed. Local complications observed in children with AP included pseudocyst in three, and infected acute fluid collection, right-sided pleural effusion, and ascites in one patient each. Complications of AP were managed with percutaneous catheter drainage (n = 3; pseudocyst, 2; infected fluid collection, 1), additional pancreatic duct stenting (n = 2), surgical drainage (n = 1), and octreotide for pleural effusion (n = 1). Signs of CP included abdominal pain (n = 7), obstructive jaundice resulting from lower common bile duct stricture (n = 2), and bleeding from gastroduodenal artery pseudoaneurysm (n = 1). Pancreatic duct stenting relieved pain in one patient, and steel coil embolization arrested bleeding from the pseudoaneurysm. Common bile duct strictures were managed by surgical bypass (n = 2), one of which required preoperative endoscopic bile duct stenting for management of cholangitis. Two other patients with CP required no intervention. CONCLUSION: A multidisciplinary approach of radiologic and endoscopic interventions and surgery are complimentary to each other in achieving successful outcomes of complicated childhood pancreatitis.     

Multidisciplinary management of surgical disorders of the pancreas in childhood

OBJECTIVES: To describe the frequency and range of pancreatic disorders in children requiring surgical intervention and to highlight the importance of multidisciplinary management. METHODS: An audit of all children under 17 years of age referred with surgical disorders of the pancreas or pancreatitis to a regional pediatric gastroenterology unit in the United Kingdom during a 10-year period. A retrospective chart review of clinical features, pathology and outcome was undertaken. RESULTS: Surgical intervention was required for the following pancreatic disorders: persistent hyperinsulinemic hypoglycemia of infancy (n = 4), pancreatic tumors (n = 5), pancreaticobiliary malunion (n = 12), pancreatic trauma (n = 6) and pancreatitis (n = 10). The indications for surgery in acute pancreatitis were a persistent pseudocyst (n = 1) and treatment of an underlying cause of pancreatitis (n = 4); in chronic pancreatitis, surgery was used to treat symptomatic pancreatic duct strictures (n = 4). One child died of a progressive lymphoma but all others who underwent surgery are alive and well. All 33 children with acute pancreatitis, including four with pancreatic necrosis, survived. CONCLUSIONS: Surgery for pancreatic disorders in children is rarely required but may be necessary a) for definitive management of primary pancreatic pathology, b) to treat sequelae of acute or chronic pancreatitis and c) to treat an underlying cause of pancreatitis. There is a broad spectrum of potential pathologies. These patients are best managed by a multidisciplinary team approach.     

儿童Ⅳa型胆管扩张症的外科治疗探讨

目的探讨儿童Ⅳa型胆管扩张症临床特点及手术治疗。方法选取2015~2017年复旦大学附属儿科医院普外科收治的胆管扩张症患儿且临床资料较完整者,共135例,按Todani分型,Ⅰ型96例,Ⅱ型9例,Ⅲ型1例,Ⅳ型29例。按照纳入标准分组:Ⅳa型29例为A组;Ⅰc型肝内外胆管扩张且互相连续患儿36例设为B组,Ⅰa、Ⅰb型仅肝外扩张患儿56例设为C组,合计121例。回顾性分析患儿术前临床症状、影像学诊断、术中发现、手术过程及预后。结果各组中女性均多于男性,组间差异无统计学意义。以磁共振胰胆管成像(MRCP)为标准,B型超声肝内胆管扩张检出率为30.7%(20/65)。所有B型超声报告均提示第一肝门前囊性占位或胆总管扩张。术前伴随胰腺炎共25例(20.7%)。二次入院患儿共10例,3例经消炎治疗后好转,7例再次手术。术后随访中可见肝内胆管扩张持续存在患儿4例,其中2例无不适症状,予保守治疗,2例患儿行二次手术治疗,手术1个月后扩张胆管消失。结论术中应积极探查肝门处狭窄环及近端扩张胆管,并适当做肝门整形。儿童Ⅳa型胆管扩张症大多预后较好,妥善处理肝外胆道后,肝内胆管扩张术后大多能改善。胰腺炎与囊肿分型无关,术前伴发水肿型胰腺炎患儿多可一期手术。     

Diagnostic and therapeutic endoscopic retrograde cholangiopancreatography in children: a large series report

OBJECTIVES: Our goal is to evaluate the indications, findings, therapies, safety, and technical success of endoscopic retrograde cholangiopancreatography (ERCP) in children. METHODS: Our database was searched for patients 17 years of age or younger undergoing ERCP between January 1994 and March 2003. Additional information was obtained by chart review. The safety and technical success of ERCP were examined. Complications were classified by the consensus criteria. RESULTS: A total of 245 patients (95 M, 150 F; mean age 12.3 years) underwent 329 examinations. Indications included biliary pathology (n = 93), pancreatic pathology (n = 111), and chronic abdominal pain of suspected biliary or pancreatic origin (n = 41). The ERCP findings were bile duct stone(s) (n = 29), benign biliary stricture (n = 19), primary sclerosing cholangitis (n = 7), anomalous pancreaticobiliary union (n = 8), choledochal cyst (n = 5), bile duct leak (n = 6), malignant biliary stricture (n = 2), biliary atresia (n = 1), chronic pancreatitis (n = 44), pancreas divisum (n = 26), pancreatic duct stricture with (n = 6) or without (n = 9) leak, pancreatic tumor (n = 1), periampullary adenoma (n = 2), and sphincter of Oddi dysfunction (n = 65). Endoscopic therapies were performed in 71% of the procedures and included sphincterotomy, stone extraction, stricture dilation, endoprosthesis placement, snare papillectomy, and cystoduodenostomy. Thirty-two (9.7%) post-ERCP complications occurred and included cholangitis in 1 patient and pancreatitis in 31. The pancreatitis was graded mild in 24, moderate in 5, and severe in 2. No mortality related to ERCP occurred. CONCLUSIONS: Diagnostic and therapeutic ERCP results are similar in children and adults except for a lower incidence of malignant disease in children. Technical success rates are high. However, ERCP-related pancreatitis is not uncommon, and the risk and benefits should be carefully reviewed before proceeding. Outcome data are necessary and is currently being accumulated at our institution.