小儿胰腺实性-假乳头状瘤(附2例报告)

目的 提高对小儿胰腺实性-假乳头状瘤的认识。方法通过介绍2例小儿胰腺实性-假乳头状瘤的诊治经验，结合复习有关本病的文献报道加以总结。结果本组2例患儿，均行手术治疗，效果满意。病理证实为胰腺实性-假乳头状瘤。通过有关文献复习，提高对本病的认识。结论胰腺实性-假乳头状瘤多见于青少年和年轻女性，小儿约占1／3，较罕见。临床主要表现左上腹隐痛，偶有急性胰腺炎史。B超和CT扫描可发现胰腺囊实性占位性病变。手术依据肿瘤大小、部位与周围浸润程度，可选择肿瘤局部切除；胰体、尾切除；胰十二指肠切除。可达到满意的治疗效果。SPT具有独特的临床病理学特点，目前认为是一种原始胰腺上皮性肿瘤，有低度恶性倾向，预后良好。     

小儿胰腺囊肿的外科治疗

目的 通过分析小儿胰腺囊肿的诊断和治疗，提供完善诊断和决定治疗方案的思路。方法 本组16例患儿中，腹部外伤12例，有腹痛等炎症表现3例，1例为胆总管囊肿切除术后反复腹痛。均经B超或CT证实胰腺存在囊肿样改变，行囊肿外引流11例，行囊肿-空肠Roux-Y吻合术4例，1例因病理证实为胰腺实体，囊性假乳头状瘤而行囊肿和脾切除及脾自体移植术。结果 治愈15例，因合并伤死亡1例，B超或CT随访无囊肿或肿瘤复发。结论 腹部外伤是胰腺囊肿样改变及囊肿形成的最主要原因；B超及CT检查为主要诊断手段；囊肿外引流术可作为早期控制症状的手术选择。     

小儿胰腺囊实性乳头状瘤的超声分析:附4例报告

胰腺囊实性乳头状瘤为一种少见的胰腺肿瘤,近年来被逐步认识。为提高对小儿胰腺囊实性乳头状瘤的诊断水平,现将经手术病理证实的4例资料回顾性分析如下。     

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。     

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.     

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.     

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。     

儿童囊性肾瘤伴 DICER1基因突变1例并文献复习

目的分析1例儿童囊性肾瘤伴DICER1基因突变患儿的临床资料并复习相关文献, 以提高对此类疾病的认识。方法收集2020年11月1例儿童囊性肾瘤伴DICER1基因突变患儿的临床资料, 总结临床特点、影像学表现、手术经过、病理和基因结果。通过PubMed、中国知网、万方等数据库检索相关文献, 检索时间截至2021年5月。结果本例患儿术前B超、CT及MRI均提示左肾巨大囊实性肿块, 边界尚清, 患儿接受左瘤肾切除术, 术中未发现肿大淋巴结, 术后病理考虑囊性肾瘤, 患儿血液及肿瘤组织均发现DICER1基因突变, 随访6个月无肿瘤残余及复发。中文文献尚无儿童囊性肾瘤伴DICER1基因突变的病例报告, 英文文献中, 同时检测到胚系和体细胞突变的仅有6例报道。结论儿童囊性肾瘤较为少见, 其发生机制与DICER1基因突变有关, 手术切除是主要治疗方式, DICER1基因突变可致多种肿瘤, 术后长期随访监测非常重要。     

小儿先天性巨输尿管症诊治1例并文献复习

目的报道1例小儿先天性巨输尿管症的诊断及治疗体会并复习文献。方法根据临床症状及B超、泌尿系尿路水成像、肾核素扫描等影像学检查,对CM进行诊断;治疗方案为手术,手术方式为腹腔镜膀胱输尿管裁剪整复加Lich-Gregoir输尿管膀胱抗反流再植术。结果术后6个月复查,患者症状消失,患肾积水减轻,无膀胱输尿管反流。结论 CM的诊断主要依据临床症状及影像学检查,手术是治疗本病的有效方法。     

腹腔镜胰腺肿瘤切除术治疗小儿胰腺实性假乳头状瘤的探讨

胰腺实性假乳头状瘤(solid pseudopapillary tumor of pancreas,SPTP)是一种少见的胰腺肿瘤,约占所有原发性胰腺外分泌肿瘤的1％～2％,常发生于年轻女性(85％的患者小于30岁)[1],男女比例约为1∶10.与其他小儿胰腺恶性病变相比,SPTP通常是可切除的,并且完全手术切除后可达到95％的治愈率.迄今为止,有关儿童患该病的报道不多,尤其应用腹腔镜技术治疗小儿胰腺实性假乳头状瘤的报道更少[2,3].本文通过回顾性分析2010年8月至2010年10月我们收治的3例胰腺实性假乳头状瘤患儿的临床资料,研究腹腔镜胰腺肿瘤切除术治疗小儿胰腺实性假乳头状瘤的技术和效果,探讨该技术的可行性和有效性.     

胰腺囊实性乳头状瘤的临床特点

目的探讨小儿胰腺囊实性乳头状瘤(SCPT)的诊断与治疗的特点。方法回顾分析近5年收治的7例SCPT患儿临床资料。男2例,女5例;平均11.5岁。病例均有诱因腹痛来诊,71.4%为腹部外伤,28.6%为过饱饮食后腹痛,查体以腹部包块为主。7例均经手术治疗,3例胰头肿瘤中2例行胰十二指肠切除术,1例仅行病理活检,4例胰尾肿瘤全部切除。结果7例均一期愈合出院。随访4个月~4.5年,近期效果良好。术后病理确诊。结论SCPT为低度恶性肿瘤,患儿平时无明显症状,有诱因后可引起突然腹痛,腹部可触及肿物为其特点,SCPT应早期手术切除肿瘤,术后近期效果满意。     

Solid-cystic papillary tumor of the pancreas in children

Solid-cystic papillary tumor (SCPT) of the pancreas is a rare neoplasm in children. The current study attempted to estimate the incidence and possible pathological origin of the tumor. Clinicopathologic features of the children under the age of 16 years with pancreatic tumors managed in a single institution between January 1993 and November 1999 were reviewed. Representative blocks of SCPT specimens were immunostained for neuron-specific enolase (NSE) chromogrannin, synaptophysin, insulin, glucagon, somatostatin, and gastrin. There were three SCPTs, two pancreatic endocrine tumors, and one acinar cell carcinoma during the study period. The estimated yearly incidence in the referral area of 2 million population is about 0.01 pediatric SCPT per year per 100,000 population. The children underwent surgical removal of the tumors. Postoperatively, they were followed up for 6 months to 4 years and were well. Immunostaining for NSE, chromogranin, synaptophysin, insulin, somatostatin, and gastrin was negative in all cases. SCPT may thus be the most common pancreatic tumor in the Asian pediatric population. The pathological origin of the tumor remains unclear and requires further investigations. Accepted: 30 October 2000     

小儿神经节瘤的诊断与治疗

目的：为准确地诊断和治疗小儿神经节细胞瘤。方法：观察24例小儿神经节细胞瘤的超声，CT，核磁共振（MR）影像检查所见，总结其临床特点，并对该肿瘤进行病理分型。结果：术产经彩超，CT，MR检查，17例确定诊断，7例误诊为其他肿瘤，患儿年龄偏大，一般状态好，瘤体增长速度缓慢，骨穿，尿3－甲基，4－羟基苦杏仁酸（VMA）为阴性，依据神经节细胞与神经纤维成分的多少及有无神经母细胞，病理分为三型；A型以神经纤维成分为主，神经节细胞占少数，此型多见；B型两种成分大致相等；C型除上述两成分外，还可见到神经母细胞手术完整切除19例，5例部分残留，术中副损伤以腔静脉破裂多见，术后随访19例，16例正常，3例死亡，其中2例起源于肾上腺，结论：神经节细胞瘤虽为少见，但了解其影像学特点，术前可以作出诊断。     

Pancreatoblastoma in childhood: clinical course and therapeutic management of seven patients

BACKGROUND: To analyze the clinical course of pancreatoblastoma in children and to propose management and therapy. PROCEDURE: Retrospective review of seven cases of pancreatoblastoma treated in France over a 20-year period and literature review. RESULTS: Median age of patients was 6 years. The tumor was well encapsulated in one patient and had direct extension to adjacent tissues in two others. Three patients had regional lymph node involvement and one had liver metastases. The tumor arose in the head of the pancreas in three children, in the tail and body in two and in the tail and in the body, one each. Elevation of AFP serum levels was noted in four out of 6 cases. Five tumor resections were performed, one initially and 4 after neoadjuvant chemotherapy, and cisplatin plus doxorubicin seemed effective. Two children received post-operative irradiation because of incomplete resection. In all, four children are disease free with a median follow-up of 50 months ( range, 5--120 months) : one had a complete removal of tumor at diagnosis and no further treatment, three had unresectable tumor at diagnosis and received neoadjuvant chemotherapy with cisplatin and doxorubicin. One of them also received post-operative irradiation. CONCLUSIONS: Pancreatoblastoma is a curable tumor. Examination of serum AFP levels may be useful for diagnosis and to follow the course of the disease. Complete resection is the treatment of choice. However, tumor is often unresectable at diagnosis and preoperative chemotherapy is needed to reduce tumor volume. We suggest a regimen that include cisplatin and doxorubicin. In patients with incompletely resected disease, postoperative radiation may be indicated.     

高频超声联合X片在儿童消化道畸形中的诊断价值

目的：探讨高频超声联合X线检查在儿童消化道畸形中的诊断价值。方法对60例经手术证实的消化道畸形患儿进行超声及X线片图像的回顾性分析。结果60例患儿中，单纯经超声诊断为肥厚性幽门梗阻8例，肠旋转不良5例，肠重复畸形6例。单纯经X线检查诊断为食管闭锁4例，肛门闭锁伴直肠会阴瘘7例；经高频超声联合X线检查诊断为消化道重复畸形13例，幽门水肿伴胃食管反流4例，环形胰腺7例，先天性巨结肠6例。结论高频超声联合X线检查可提高儿童消化道畸形的诊断率，二者互为补充。     

Diagnosis and differential diagnosis of heart tumors in infancy and childhood

The clinical features of 10 children, in whom the diagnosis of a cardiac tumor has been made, are reviewed. There were nonspecific systolic murmurs in 9 patients, depression of the S-T segment in the ECG of 7 children, and premature extrasystoles or supraventricular tachycardia in 4 children. X-ray always showed mild to moderate enlargement of the heart, but an abnormal contour could be detected only in 2 of the patients. While findings of cardiac catheterization were unspecific, the diagnosis could be made angiographically in 8 cases. Invasive studies, however, are now only indicated, if a proper diagnosis cannot be made by means of echocardiography. The diagnosis should be suspected in children with a nonspecific murmur, cardiomegaly and ECG-changes. Usually the diagnosis of a cardiac tumor is an indication for operation.     

恶性实体瘤患儿血清白细胞介素2受体的表达及其意义

目的探讨恶性实体瘤患儿可溶性白细胞介素2受体(SIL-2R)与正常儿童的表达差异及手术前后不同时期表达水平进行动态观察。方法选择15例恶性实体瘤、10例健康儿童,分别检测术前1d、术后2d、10d的血清SIL-2R含量。结果恶性实体瘤患儿SIL-2R表达水平明显高于正常儿童(P<0.05),术后第2天SIL-2R表达水平高于术前,术后10天SIL-2R表达水平明显高于术前及术后第二天(P<0.05)。结论SIL-2R对小儿恶性实体瘤的诊断,预后有参考价值。     

Clinical analysis of childhood pancreatoblastoma arising from the tail of the pancreas

Pancreatoblastoma is a rare pancreatic tumor. In this study, 3 cases of childhood pancreatoblastoma that arise from the tail of the pancreas were reported. Abdominal pain and vomiting were observed in 1 case considering the huge size of the tumor. The other 2 patients, who were previously well, complained of a mass in the abdomen after a casual physical examination. Elevated serum α-fetoprotein levels were noted in all cases. Imaging findings indicated a well-defined heterogeneous large mass in the left retroperitoneal space. Exploratory laparotomy revealed a large mass, arising from the tail of the pancreas. Surgery alone with complete excision of the masses was performed. Immunohistochemical staining showed that only α-fetoprotein was positive in all cases. All of these 3 cases have a good outcome in the follow-up without adjuvant chemotherapy. These data suggest that the diagnosis of pancreatoblastoma is difficult and should be suspected at palpation of an abdominal mass. α-Fetoprotein may serve as a tumor marker for preoperative diagnosis and postoperative recurrence. Pancreatoblastoma arising from the tail of the pancreas is a curable tumor, and adjuvant chemotherapy may not be necessary if the tumor can be excised completely.     

小儿肝间叶性错构瘤诊断和治疗

目的 探讨小儿肝间叶性错构瘤的临床特征与诊治方法.方法 结合文献,回顾性分析1998年2月至2007年10月南京军区普通外科研究所收治的8例肝间叶性错构瘤患儿的临床表现、诊断、治疗和预后.8例患儿术前均行超声与CT或(和)MRI检查,术中经快速冷冻切片病理检查,行肿瘤局部切除术5例,肝叶切除术3例.结果 术前根据影像学检查结果诊断为肝间叶性错构瘤4例,肝脏肿瘤(性质待查)1例,误诊3例.术中冷冻切片确诊5例.术后8例均经常规病理确诊.术中出血5～800 ml,平均出血量为119 ml.术后除1例患儿出现肺部感染经对症治疗痊愈外,其余无并发症.住院时间为7～21 d,平均12 d.随访10～126个月,平均68.5个月,均健康无瘤存活,未见复发或恶变.结论 术前影像学检查有助于诊断和治疗,但由于该病罕见,仍存在较高的误诊率,外科手术是首选治疗方法,可选择肿瘤局部切除术或规则性肝切除术.术中出血与术后感染是可能出现的并发症.肿瘤完整切除后,多预后良好,偶有复发或恶变,需长期随访,定期复查.