小儿睾丸肿瘤35例报告

３５例小儿睾丸肿瘤中睾丸卵黄囊瘤１６例，畸胎瘤１２例，分析表明，以睾丸无痛性肿块为主要症状，其次为鞘膜积水等，血清ＡＦＰ是诊断小儿卵黄囊瘤的重要肿瘤标记物。３３例采用经腹股沟睾丸切除术，４例睾丸卵黄囊瘤接受淋巴清扫术。１６例卵黄囊瘤病例后化疗１年，２例死于肿瘤转移。     

小儿睾丸恶性生殖细胞瘤的回顾性分析

目的：探索小儿睾丸恶性生殖细胞瘤合理的治疗方案。方法：I期无高危因素18例行睾丸高位切除术后密切随访（A组）;I期有高危因素和Ⅱ期31例分为13例行睾丸切除加腹膜后淋巴结清扫加不规则化疗（B组）,10例行睾丸切除加腹膜后淋巴结清扫加规则化疗（C组）,8例行睾丸切除加规则化疗（D组）。结果：A组、B组间生存率差异有显著性意义（P＜0．05）,B组与C组、D组间生存率差异有显著性意义（P＜0．05）,C组、D组间生存率差异无显著性意义（P＞0．05）。结论：年龄小、分期早预后好,I期有高危因素及Ⅱ期者应行与化疗的综合治疗,有效联合化疗可代替常规腹膜后淋巴结清扫。     

小儿睾丸卵黄囊瘤治疗体会

探讨临床Ⅰ期尤其是〈１岁小儿睾丸卵黄囊瘤治疗方式的选择。临床资料：Ⅰ期１０例，其中〈１岁７例，Ⅱ例４例，均实行高位精索睾丸切除。９例辅以腹膜后淋巴结清扫，Ⅰ期５例，Ⅱ期４例，仅１例有阳性发现，术后均未行规则的化疗。结果；获访１２例中生存９例，死亡３例，均大于１岁，死因为肺和腹膜后转移。     

睾丸肿瘤41例临床分析

目的总结小儿睾丸肿瘤的诊治经验。方法对我院1991年1月～2002年2月收治41例睾丸肿瘤患儿的临床资料进行分析。结果本组发病年龄3个月～6岁,其中<2岁25例,睾丸卵黄囊瘤24例,睾丸畸胎瘤16例,睾丸横纹肌肉瘤1例,均行根治性睾丸切除术,其中17例卵黄囊瘤和1例横纹肌肉瘤行腹膜后淋巴结清扫术,恶性肿瘤均行联合化疗。睾丸畸胎瘤患儿均存活,卵黄囊瘤随访21例,生存16例,死亡5例。结论B超是鉴别睾丸肿瘤有效方法之一,CT扫描对了解有无转移灶有益;甲胎蛋白(AFP)测定对鉴别诊断及预后判断有极大帮助,术后多药联合规则化疗对提高睾丸恶性肿瘤的生存率起重要作用。     

小儿睾丸肿瘤23例诊治体会

目的 探讨小儿睾丸肿瘤的诊治方法.方法 回顾性分析深圳市儿童医院2005 ～ 2009年收治的23例睾丸肿瘤患儿临床资料,年龄2个月至9岁.多以无痛性阴囊肿块就诊.术前常规进行血清AFP、胸部CT、睾丸及腹膜后超声检查,睾丸MRI检查.采用高位精索离断式睾丸切除、睾丸瘤体剔除、腹膜后淋巴结清扫等术式,术中均行冰冻病理切片,术后根据瘤体性质进行相应化疗.随访6个月至5年,监测血清AFP动态变化,以及阴囊、腹股沟、腹膜后超声和胸片检查.结果 23例中,病理检查结果提示卵黄囊瘤11例,畸胎瘤8例,横纹肌肉瘤1例,精原细胞瘤1例,皮样囊肿2例.高位精索离断式睾丸切除术13例,睾丸肿瘤剔除术10例.获随访21例,平均随访时间19个月,卵黄囊瘤Ⅰ期9例,术后化疗1个疗程;Ⅱ期2例,术后1个月复查AFP阳性,PET检查提示阴囊残留复发1例,腹膜后转移1例.畸胎瘤8例,皮样囊肿2例,行保留睾丸的瘤体剔除术,均无瘤存活.结论 睾丸恶性肿瘤应行根治性睾丸切除术,卵黄囊瘤Ⅱ、Ⅳ期应采取手术加化疗.良性睾丸肿瘤可行肿瘤剔除术而保留睾丸组织.     

FASN在小儿卵黄囊瘤中的表达及临床意义

目的分析小儿卵黄囊瘤的临床特点,探讨脂肪酸合酶(Fatty acid synthase,FASN)在小儿卵黄囊瘤中的表达及意义。方法回顾性分析26例卵黄囊瘤患儿临床资料,运用免疫组化检测26例卵黄囊瘤、17例未成熟畸胎瘤、1例胚胎性癌及12例相关部位正常组织标本中FASN的表达情况。结果26例小儿卵黄囊瘤发病平均年龄2岁5个月,16例单纯以肿块就诊,5例单纯以局部继发症状就诊,5例同时具有肿块及局部症状。FASN在卵黄囊瘤的表达程度(73%)显著高于未成熟畸胎瘤(28%)、胚胎性癌(0%)、正常组织(16%)。FASN在性腺内和性腺外小儿卵黄囊瘤的阳性表达率分别为56%和100%。伴淋巴结或远处转移组和无转移组阳性表达率分别为100%和61%。FASN的阳性表达率与临床分期呈正相关。以上差别均有统计学意义。化疗前后FASN表达率分别为72%和75%,差别无统计学意义。结论小儿卵黄囊瘤起病隐匿,进展迅速,手术治疗联合化疗有效,FASN的表达可为诊治提供参考。     

儿童卵黄囊瘤临床诊断与治疗

小儿卵黄囊瘤(yolk sac tumor，YST)，原发于性腺或性腺外的组织器官，包括卵巢、睾丸、骶尾部、纵隔和腹膜后等，是一种高度恶性的生殖细胞瘤，它进展迅速，容易复发及转移，生存率较低，严重威胁患儿生命[1,2]。对其做到早期诊断，正确及时地选择手术以及术后化疗都是值得探讨的问题。现在将我院自2002年7月至2010年7月收治的22例小儿卵黄囊瘤病例的诊断和治疗经验报告如下。     

高频超声及彩色多普勒对小儿睾丸肿瘤的诊断价值

目的 总结小儿睾丸肿瘤的声像图特点.方法 回顾性分析20例经高频超声及彩色多普勒诊断为睾丸肿瘤的患儿临床资料,其中15例超声检查提示为卵黄囊瘤,5例提示为畸胎瘤.结果 15例超声诊断为卵黄囊瘤患儿中,14例经手术病理确诊卵黄囊瘤,1例手术结果为间质细胞瘤,5例超声检查提示畸胎瘤均与手术病理相符.20例患儿均无区域淋巴...     

睾丸肿瘤60例

目的：探讨小儿睾丸肿瘤的临床病理特点。方法：分析小儿原发性和继发性睾丸肿瘤60例的临床病理资料。其中10例卵黄囊瘤作免疫组化染色。回顾分析国内小儿睾丸肿瘤类型文献资料共203例。结果：60例小儿睾丸肿瘤生殖细胞起源占85．0％，均为单一成分的生殖细胞起源肿瘤，恶性率70．0％，卵黄囊瘤占55．0％。初次就诊时大多数病例为一期。6例有转移者均为卵黄囊瘤。卵黄囊瘤病理形态多样，以内胚窦、网状、玻璃样小体、微囊及腺样结构较为多见。免疫组化染色10例中6例显示甲胎蛋白＋、抗胰蛋白酶＋、细胞角蛋白＋、绒毛膜促性腺激素－、增殖细胞核抗原＋、波形蛋白－，P^53 。结论：小儿睾丸肿瘤与成人不同，3岁以内多见，占85．96％。恶性率较成人低，以卵黄囊瘤和成熟性畸胎瘤多见，预后较成人好。     

61例儿童睾丸卵黄囊瘤的诊断与治疗

目的：分析儿童睾丸卵黄囊瘤的诊断与治疗,以提高其临床诊治水平。方法回顾性分析本院1995年至2014年收治的61例儿童睾丸卵黄囊瘤患儿的临床资料。结果61例均以阴囊包块就诊,其中60例无痛;61例体查均有阴囊沉重感,透光试验均为阴性;3例提睾反射消失,6例误诊为鞘膜积液,4例初诊腹股沟疝,2例睾丸炎症,1例误诊为腺瘤。术前AFP值均增高,超声检查提示实性包块,CDFI提示84．8％睾丸肿块血流丰富。阴囊X线片均未见确切钙化影。60例行瘤睾高位切除术,1例行睾丸肿瘤剥除术,11例加行腹股沟区淋巴结清扫术。病理检查多见疏网状、腺泡样以及乳头样结构。2009年以前术后化疗采用博来霉素＋长春新碱,近5年采用PEB（顺铂＋足叶以带＋博来霉素）方案、PVB（顺铂＋长春新碱＋博来霉素）方案或二者交替化疗。术后1～2个月随访AFP值多降至正常,无一例复发或死亡。结论儿童睾丸卵黄囊瘤多因无肿痛性肿块就诊,体查包块有沉重感,AFP值升高,超声检查可见实质性包块。胸片和腹部超声可协助肿瘤临床分期。手术方案主要为高位瘤睾切除术,术中冰冻切片能协助术者选择手术范围,术后配合化疗,患儿临床预后效果好。     

Treatment of a stage I testicular yolk sac tumor with vascular invasion

There is controversy concerning the treatment of stage I yolk sac tumor of the testis, particularly of those with histological factors that indicate a high risk of relapse. Usually orchiectomy alone is sufficient and adjuvant chemotherapy is unnecessary. Retroperitoneal lymphadenectomy is indicated for patients with persistently high alpha-fetoprotein. Once recurred, treatment at that time is thought to be curative. However, postoperative chemotherapy may be necessary for patients with a tumor expressing histological factors that predict possible relapse. In this paper we report on a case of a 2 year old boy whose tumor invaded the testicular veins. The patient suffered from recurrent disease but was successfully treated by chemotherapeutic regimens including cisplatin and retroperitoneal lymphadenectomy. The importance of the histological factors in making a decision on the treatment strategy for stage I testicular yolk sac tumor is discussed.     

小儿卵黄囊瘤的诊断与治疗

目的 总结18例儿童卵黄囊瘤手术治疗和化疗的临床经验,分析探讨与预后的关系.方法 回顾性分析我院1992年4月至2009年12月收治的18例儿童卵黄囊瘤的病例资料.男12例,女6例,依据临床表现、影像学所见和血AFP水平升高,做出临床诊断.原发部位:睾丸10例,卵巢3例,骶尾部4例,腹部1例.结果 11例完全切除及BEP方案化疗;5例未完全切除、化疔;1例完全切除、未化疗;1例未完全切除也未行化疗.随访8～24个月,监测影像检查及血AFP水平变化,除1例放弃治疗外,全部患儿存活良好,未出现复发或转移.结论 在儿童卵黄囊瘤的治疗中手术与化疗相结合可以达到良好的疗效.

Abstract：

Objective To review our experience of managing yolk sac tumors in children. Methods Clinical data of eighteen patients with yolk sac tumors admitted in our department from April 1992 to December 2009 were reviewed retrospectively. There were 12 boys and 6 girls. The diagnoses were based on diagnostic imaging and elevated serum AFP levels. Of the 18 cases, 10 were located in testes,3 in ovaries,4 in sacrococcygeal region and 1 in the abdomen. Results Eleven patients were treated with complete excision and BEP chemotherapy; 5 pateints underwent subtotal resections and chemotherapy; one complete excision without chemotherapy; one patient refused treatment. Followedup of 8-24 months revealed that all children, except for the untreated case, survived uneventfully, without any sign of recurrence or metastasis. Conclusions The combination of surgery and chemotherapy can achieve good prognosis in children with yolk sac tumors.     

Clinical stage I nonseminomatous germ cell tumors of the testis in childhood and adolescence: an analysis of 31 cases

A 20-year single-institution experience of clinical stage I nonseminomatous germ cell tumors of the testis (NSGCTT) in childhood and adolescents was reviewed in relation to clinical characteristics, treatment modalities, and survival. Thirty-one patients with clinical stage I NSGCTT were seen between 1980 and 2000: 14 children and 17 adolescents. Yolk sac tumors and/or teratomas occurred in the children, whereas mixed histologies, including embryonal carcinoma, were predominant in the adolescents. After orchiectomy, the children were assigned to surveillance and the adolescents to active treatment: 16 underwent retroperitoneal lymph node dissection (RPLND) and 1 had adjuvant cisplatin-based chemotherapy because of a high-risk histology. Three of the 14 children (21.4%) relapsed 3, 7, and 8 months after orchiectomy: all 3 had yolk sac tumors and presented with increased alpha-fetoprotein levels. No patients had retroperitoneal relapse; two recurred locally and one in the lung. All three children were treated with cisplatin-based chemotherapy with or without surgery. Among the 16 adolescents undergoing RPLND, 4 (25%) had nodal metastases. Three of the 12 patients (25%) who had negative nodes at RPLND relapsed in the lung 3, 7, and 8 months after RPLND. All were treated with cisplatin-based chemotherapy with or without surgery. Five-year relapse-free and overall survival rates for the whole series were 80.6% and 100%, respectively. This series enabled the authors to pinpoint several important aspects of stage I NSGCTT in children and adolescents. In particular, almost all the childhood cases had the same yolk sac tumor histology, the children tended to have localized disease, and an increased alpha-fetoprotein level had a very high predictive value, suggesting that follow-up should include AFP measurements. A conservative approach is the best option in children, while adolescent NSGCTT behaves like the adult disease and management must include similar treatment strategies.     

Clinical significance of serum alpha-fetoprotein subfractionation in pediatric diseases

Serum alpha-fetoprotein (AFP) subfraction profile is a predictive indicator for the discrimination of hepatic malignancies, benign liver diseases and yolk sac tumor in adults. In the present study, AFP subfractions were examined in AFP-positive sera from 59 patients of less than 15 years of age. Fractionation of AFP was carried out by lectin affinity crossed-line immunoelectrophoresis. Concanavalin A, Lens culinaris hemagglutinin and phytohemagglutinin E were used as lectins. Fifty-four of 59 (91.5%) AFP subfraction profiles in patients with pediatric diseases were classified into three common types: (1) benign liver disorder, (2) hepatic malignancy and (3) yolk sac tumor. An atypical AFP subfraction profile resembling hepatic malignancy type was found in 5 of 59 (8.5%) infants. It was concluded that estimation of serum AFP subfraction profiles facilitates differential diagnosis of various AFP-positive pediatric diseases, such as hepatoblastoma. hepatoma, hepatic cirrhosis, hepatitis or germ cell tumors.     

Testicular germ cell tumors in children: management and outcome in a series of 20 patients

Testicular germ cell tumors occurring during childhood are extremely rare. This study reports the clinical presentation, pathological diagnosis, treatment methods and outcome in a series of 20 boys, aged between 3.5 months and 16 years (median: 1.5 years; 19 were prepubertal), who were treated between 1963 and 2003. Histologically, mature teratoma was present in seven, immature teratoma in four and yolk sac tumor in nine. Nineteen patients were stage I; only one patient was stage IV. Of the 11 teratomas, 10 were treated by orchiectomy and one by testis-sparing tumor excision only. All 11 patients have survived and show no evidence of disease between 10 and 28 years after surgery. The nine patients with yolk sac tumor were managed by orchiectomy, in two plus retroperitoneal lymphadenectomy, and in eight plus chemotherapy. One patient is in remission for 10 months, seven are alive with no evidence of disease for 5.5-23 years, and one patient died from a T-cell acute lymphoblastic leukemia, 2 years after the end of treatment of the testicular tumor. A gradual switch towards less invasive treatment has been observed over the years. This study confirms the excellent cure rates obtained in children with testicular germ cell tumor, provided diagnosis is prompt and treatment accurate.     

小儿睾丸卵黄囊瘤细胞的体外培养及生物学特性研究

目的 采用组织培养技术在体外培养取自人体的睾丸卵黄囊瘤组织,探讨小儿睾丸卵黄囊瘤组织的体外培养方法及生物学特性.方法 采用组织块培养法培养1例睾丸卵黄囊瘤患儿手术的标本,从形态学、细胞生长动力学、肿瘤内分泌、染色体分析、细胞DNA分析等方面初步研究了小儿睾丸卵黄囊瘤细胞的生物学特性.结果 小儿睾丸卵黄囊瘤细胞无论形态学观察,还是功能学测定,均符合卵黄囊瘤细胞的特征,染色体众数39～97条,并具有体外分泌AFP功能.其特有表现为:细胞贴壁生长,呈短梭形或多角形,细胞密集时可多层重叠生长,肿瘤细胞表面有明显的微绒毛,细胞核形态怪异,细胞倍增时间较长.结论 小儿睾丸卵黄囊瘤细胞在体外生长稳定,增殖活跃,肿瘤细胞的纯度较高,可以用于后续的实验研究.

Abstract：

Objective To establish a method for the culture of testicular yolk sac tumor in childhood and then investigate biological characteristics in vitro. Methods One specimen from testicular yolk sac tumor was cultured in vitro. Testicular yolk sac tumor cell lines were studied morphologically and subjected to karyotype analysis, DNA analysis, and tumor formation evaluation. Results Morphological observation and functional analysis show that cell lines have characteristics of testicular yolk sac tumor. The number of chromosomes varied from 39 to 97. Turours were immunostained positively for AFP in vitro and found to form multiple layers with microvilli. The nuclei were variable and bizarre in size and shape. Conclusions Testicular yolk sac tumor cell lines were cultured proliferated in a stable manner in vitro, which provides a convenient and economical object for basic researches on yolk sac tumor in future.     

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??Objective??To analyze the clinical characteristics and outcomes of children with stage I testicular germ cell tumors??TGCT????and to discuss the treatment strategy for this disease after radical inguinal orchiectomy. Methods??Clinical data of 41 patients with stage I TGCT at Shanghai Children’s Medical Center??Shanghai Jiaotong University School of Medicine between June 2003 to December 2013 were retrospectively analyzed. Their clinical characteristics??therapy and outcomes were analyzed. Results????1??The median age at diagnosis was 18??3 to 43??months old. Among them??38 children??92.7%?? were younger than 3 years old.The pathological subtype included 35 cases of yolk sac??85.4%????4 immature teratoma??9.7%?? and 2 mixed germ cell tumors??4.9%??.??2??Serum AFP levels were elevated in 38 patients??92.7%??. AFP reduced to normal in 27 cases??71.1%??4 weeks after surgery and in 35 cases??92.1%?? after 2 courses of chemotherapy respectively??but 2 cases of them relapsed with AFP increasing again.??3??Forty out of 41 patients underwent surgery and 3??4 courses of chemotherapy with cyclophosvnamide??vincristine and dactinomycin D??VAC??. One patient received surgery alone. The median follow-up period was 64 months. One of the 32 patients who received adjuvant chemotherapy relapsed and then lost follow-up without treatment. The recurrence rate was 3.1% and the 5-year overall survival was 100%. No complication related to chemotherapy occurred during the follow-up. The only one patient treated with surgery alone relapsed at 5 months and achieved complete response after salvage treatment. Conclusion????1??The relationship between the decrease of serum AFP and the prognosis is uncertain and needs to be further studied.??2??Short course chemotherapy with mild toxic side effects can reduce the recurrence rate effectively?? so it can be used as a necessary treatment for patients with high risk of relapse??and it can also be used as a selective treatment for patients with low risk of recurrence.     

儿童盆腔卵黄囊瘤的64层螺旋CT诊断与临床病理对照分析

目的分析儿童盆腔卵黄囊瘤的临床、病理及64层螺旋CT(MSCT)特点,以提高对本病的诊断水平。方法回顾性分析34例经手术病理检查证实的盆腔卵黄囊瘤儿童的临床、病理、MSCT及多平面重建资料。术前均行盆腔MSCT平扫及增强扫描。结果本组34例中,女性21例,男性13例;患儿年龄3个月至7岁3个月,平均(18±15)个月龄。临床表现主要为腹部或骶尾部肿块以及腹痛。33例甲胎蛋白(AFP)明显升高,1例(占2.9%)AFP值为2.2 ng/mL。34例患儿中,18例来源于盆腔腹膜腔内,16例位于腹膜腔外向骶尾部生长,其中9例主体部分位于盆腔,7例主体部分位于骶尾部。MSCT均表现为卵圆形或不规则形囊实性肿块,增强后肿瘤的实性部分明显强化,肿块最大径为2.8~10.7 cm。AFP值与肿块大小及部位无显著相关性。结论盆腔卵黄囊瘤的临床及MSCT表现有一定特征,CT能较准确描述肿瘤内部结构与血供,结合血清AFP检查,有利于盆腔卵黄囊瘤的正确诊断、制订术前手术方案及判断术后复发情况。