儿童内胚窦瘤9例临床分析

目的　分析儿童内胚窦瘤的特点 ,提高儿童内胚窦瘤的诊治水平。方法　对诊断明确的 9例内胚窦瘤患儿临床资料进行分析。结果　 9例患儿发病年龄 3个月至 10岁 4个月 ,<3岁者 7例 ;男 5例 ,女 4例 ;Ⅰ期7例 ,Ⅱ期 1例 ,Ⅲ期 1例 ;原发于睾丸 4例 ,卵巢 2例 ,骶尾部 2例 ,中枢神经系统 1例。临床表现多为原发部位肿物及其压迫引起的相应症状 ,经B超可明确肿物存在。术前甲胎蛋白 (AFP) 7例升高 ,为 4 0 0～ 2 0 2 7mg/L ,无残留病变、转移者术后AFP均可降至正常。 4例睾丸内胚窦瘤Ⅰ期中 3例经根治性手术加术后PVB方案或VAC方案化疗 ,均获得大于 5年的生存期 ;原发卵巢、骶尾部内胚窦瘤 4例中 3例在Ⅱ期前手术、化疗获得至少 2年生存期。结论　小儿内胚窦瘤早期诊断、经手术加术后化疗是提高长期生存率的关键 ,AFP检测及B超联合应用对小儿内胚窦瘤诊断及复发监测有重要意义。     

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。     

化疗联合手术治疗小儿阴道内胚窦瘤的疗效分析

目的总结阴道内胚窦瘤患儿的临床资料,探讨阴道内胚窦瘤。化疗联合局部病灶切除术的疗效。方法回顾性分析2012年11月至2016年5月上海市交通大学附属儿童医院普外科收治的5例阴道内胚窦瘤患儿的临床资料,发病年龄3~14个月,平均(6.8±3.87)个月,根据COG分期均为Ⅲ期,经阴道行膀胱镜检查肿块活检明确病理类型,术前予BEP或VIP方案化疗,化疗后根据影像学检查及经阴道膀胱镜检查确认可手术切除,再行经阴道或经腹部局灶性肿瘤切除术,术后继续化疗。结果5例患儿初始症状均为阴道出血,术前化疗2~5个疗程,化疗后血清AFP水平明显下降[化疗前713.55~18647 ng/mL,平均为(6455.8±6325)ng/mL;化疗后术前4.09~36.4 ng/mL,平均为(17.74±10.6)ng/mL];肿瘤大小明显缩小[化疗前直径1.74~4.33 cm,平均为(3.2±1.09)cm,化疗后术前直径0.8~2.0 cm,平均(1.28±0.43)cm];术后病理检查结果提示2例切缘仍有肿瘤细胞残留,3例切缘未见肿瘤细胞;术后继续化疗2~4个疗程,5例患儿均获得随访,随访17~55个月,平均(29.8±14.85)个月,血清AFP水平正常,影像学检查未见肿瘤复发,均无瘤存活。结论化疗联合经阴道或经腹部局灶性肿瘤手术切除治疗小儿阴道内胚窦瘤可以达到良好的疗效,同时保留了患儿的生育功能。     

儿童内胚窦瘤临床治疗研究

目的 探讨儿童内胚窦瘤的治疗与转归。方法 回顾性分析2000 年4 月至2013 年7 月收治的12 例内胚窦瘤患儿的临床资料。其中男7 例,女5 例;11 例年龄在1~3.3 岁之间,1 例11 岁。Ⅰ期2 例,Ⅱ期4 例,Ⅲ期2 例,Ⅳ期4 例。1 例采用单纯手术,1 例采用手术联合VAC（长春新碱+ 放线菌素D+ 环磷酰胺）方案化疗,10 例采用手术联合以PEB（顺铂+依托泊苷+博来霉素）方案为主的化疗。结果 成功随访11 例,目前存活10 例,生存期为4.5~66 个月。10 例接受了PEB 方案化疗的患儿,8 例达到完全缓解,1 例达到部分缓解,1 例失访。PEB 方案治疗并发症主要包括骨髓抑制及胃肠道反应,未发现远期并发症。结论 手术联合PEB 化疗方案是有效、安全的治疗儿童内胚窦瘤的方法,但需积累更多病例进一步证实。     

BEP方案治疗儿童阴道内胚窦瘤的效果分析

目的探究BEP方案治疗儿童阴道内胚窦瘤的效果及预后。方法回顾性分析2008—2020年首都医科大学附属北京儿童医院收治的12例阴道内胚窦瘤患者的临床资料。所有患者治疗前经病理确诊。收集患者临床表现、发病年龄、肿瘤大小及有无远处转移等资料。随访内容包括超声检查、CT检查和血清甲胎蛋白(alpha fectoprotein, AFP)水平。化疗方案采用国际标准BEP三联化疗方案:博莱霉素、依托泊苷和顺铂。预后评价采用世界卫生组织通用实体瘤疗效评价标准,分为完全缓解(complete response, CR)、部分缓解(partial response, PR)、疾病稳定(stable disease, SD)和疾病进展(progressive disease, PD)4个等级。结果本研究共纳入12例阴道内胚窦瘤,中位年龄14.5个月(9～21个月),平均肿瘤长径为4.1 cm。12例中有1例发生转移(髂窝淋巴结转移)。单纯化疗者在平均4个疗程后,7例(58.3%)获得CR,5例(41.7%)获得PR,无SD及PD患者。5例PR患者中,3例接受手术获得CR,术后病理证实为坏死肿瘤细胞;2例因拒绝手术继续采用BEP化疗方案,1例达到CR,其中淋巴结转移患者予化疗维持于PR。全部患者AFP水平于平均2.8个化疗周期后降至正常。平均随访101个月,无一例复发。结论阴道内胚窦瘤通过规范化疗可获得很好的治疗效果;BEP作为一线化疗方案,效果明显,多数患者可获得CR,长期随访无复发;对于获得PR的患者,手术治疗可有效去除残余结节,预防复发。     

小儿性腺外内胚窦瘤3例诊断与治疗分析

目的总结小儿原发性腺外内胚窦瘤的诊断与治疗经验。方法回顾性分析我院2003年10月-2011年9月收治的3例性腺外内胚窦瘤患儿的病历资料。结果术前均行PEB或JEB方案新辅助化疗,根治性手术切除,术后病理均为完全缓解,术后继续化疗。随访3个月～8年,2例无病存活,1例原位复发,经术前化疗、再次手术切除及术后化疗后,现随访25个月,无病存活。结论甲胎蛋白水平对小儿性腺外内胚窦瘤诊断及术后复发监测灵敏度较高,化疗与手术结合治疗效果良好。     

儿童内胚窦瘤

恶性生殖细胞肿瘤(EST)占小儿肿瘤3%, 以内胚窦瘤最常见, 预后较差. 近年来使用新的化疗方案和血清AFP监测肿瘤复发, 改变了内胚窦瘤的处理方法, 取得了一定效果.     

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。     

小儿不可切除性恶性实体瘤区域性动脉化疗的探讨

目的 探讨经导管的区域性动脉灌注化疗对小儿全身各部位的不可切除性恶性实体瘤治疗的可行性和疗效.方法 1999年7月至2006年12月对15例不同部位的小儿不可切除性恶性实体瘤行经导管的区域性动脉灌注化疗33次(每例1～4次).患儿年龄5个月至12岁(中位年龄3.6岁).男6例,女9例.病种包括:横纹肌肉瘤5例(盆腔1例,阴道2例,膀胱1例,盆腔臀部1例),内胚窦瘤4例(骶尾部3例,大网膜1例),恶性畸胎瘤2例(卵巢、小脑各1例),腹腔促结缔组织增生性小圆细胞肿瘤1例,肠系膜根部胰母细胞瘤1例,小脑室管膜瘤1例,上臂皮肤纤维肉瘤1例.其中13例为术前治疗、2例为手术时有肉眼残留的术后治疗.方法 为对肿瘤的供血动脉行经导管的灌注化疗.对盆腔、膀胱、子宫阴道肿瘤选用双侧髂内动脉,骶尾部畸胎瘤选用双侧髂内动脉加骶中动脉,腹腔内肿瘤选用肠系膜上动脉,上肢肿瘤选用肱动脉分支,小脑肿瘤选用椎动脉,药物为顺铂80 mg/m2、吡柔比星40 mg/m2,长春地辛3 mg/m2,溶于生理盐水120 ml,灌注时间60 min,间隔4周重复.间歇期加用静脉途径的全身化疗(长春地辛、异环磷酰胺、依托泊苷).结果 全组15例的33次动脉灌注化疗后均无肝、肾、心和听力功能障碍,但有恶心呕吐和Ⅰ～Ⅱ度骨髓抑制,1例小脑室管膜瘤出现轻度偏瘫,4周后恢复.术前治疗的13例均肿瘤明显缩小、局限,可完全切除.术后治疗的2例均无复发.随访24～100个月,无瘤生存13例,失访1例,死亡1例.结论 经导管的区域性动脉灌注化疗可用于各种部位的不可切除性儿童恶性实体肿瘤,具有较强的肿瘤杀灭作用,副作用小,与静脉途径的全身化疗可交替应用,实现双途径的多药联合化疗,可使不可切除的肿瘤变为可完全切除,或杀灭术后残留的肿瘤、避免复发转移.     

32例小儿未成熟畸胎瘤临床诊疗特点回顾分析

目的回顾分析儿童未成熟畸胎瘤的临床诊疗特点。方法收集我院2010年3月-2014年9月收治的32例经手术及病理确诊为未成熟畸胎瘤患儿的临床资料,其中男10例,女22例,年龄9 h~103个月。肿瘤发生于卵巢9例,睾丸6例,腹膜后9例,骶尾部5例,纵膈1例,胃部2例。全部患儿均行肿瘤完全切除,术后接受化疗14例(Ⅱ级、Ⅲ级各7例),随访患儿生存情况。结果(1)32例患儿,男女比例为1:2.2,发病中位年龄5个月,平均年龄24.8个月,1岁以下占65.6%。肿瘤发生部位以性腺最常见(卵巢多于睾丸)16例(50.0%),其次为腹膜后9例(28.1%)、骶尾部5例(15.6%),其他部位仅2例(6.3%)。(2)血清甲胎蛋白(AFP)比值大于83.17者3例(Ⅱ级1例,Ⅲ级2例)。(3)影响血清AFP阳性率的因素:病理分级Ⅰ级和Ⅲ级间血清AFP阳性率差异有显著性(P=0.0265),而不同性别、年龄及不同部位的未成熟畸胎瘤患儿血清AFP阳性表达率差异无显著性(P>0.05)。(4)所有患儿均经手术完全切除肿瘤,病理检查确诊未成熟畸胎瘤,Ⅰ级11例,Ⅱ级14例,Ⅲ级7例,术后接受化疗14例(Ⅱ级7例、Ⅲ级7例)。(5)随访时间3个月~42个月,平均随访时间20.6个月,仅2例(2/32)出现复发,1例为卵巢畸胎瘤术前有破溃者,1例为纵膈畸胎瘤术后未予化疗者,其余均获得完全缓解(30/32,93.8%)。结论(1)未成熟畸胎瘤诊断后应及早行肿瘤完整切除手术,Ⅰ级伴AFP正常者,通常仅需要手术完整切除肿瘤;Ⅱ级伴AFP阳性、术中见肿瘤包膜不完整、侵袭周围组织或肿瘤破溃、瘤体未完整切除者,术后建议进一步联合化疗;Ⅲ级的患儿在术后都应及时辅以化疗。规范的综合治疗下多能获得良好的临床预后。(2)未成熟畸胎瘤患儿血清AFP测定对于术前鉴别肿瘤的良恶性、指导手术时机及制定综合治疗方案、术后动态随访监测肿瘤有无复发、转移等生物学行为及疗效判断具有重要意义。     

Endodermal sinus tumors in the head and neck region

Extragonadal germ cell tumors of the head and neck region account for only 5% of all benign and malignant germ cell tumors. Endodermal sinus tumors (EST) of the head and neck region are rare. We report three patients with EST of head and neck region over a period of 10 years; the primary sites of tumor were orbit, maxillofacial region and retroauricular region. Histopathological examination revealed malignant teratoma with predominant endodermal sinus pattern in two, and pure EST in one patient. Serum alpha fetoprotein (AFP) was elevated in all three patients. Two patients had initial surgery but did not receive adjuvant chemotherapy, as the parents refused it. Partial remission was achieved in the other patient who received chemotherapy (cisplatin, bleomycin and vinblastin) and the patient died of infection after four courses of chemotherapy. Med. Pediatr. Oncol. 29:303–307, 1997. © 1997 Wiley-Liss, Inc.     

Endodermal sinus tumor. Histological changes induced by chemotherapy

The case of a child affected at birth sacrococcygeal teratoma is reported. Twenty-eight months following surgical resection, the tumor relapsed locally and liver metastases occurred. A biopsy of the sacrococcygeal mass was performed and histologic examination proved it to be an endodermal sinus (yolk sac) tumor. Chemotherapy consisting of etoposide and high-dose carboplatin was started. When a second operation was performed the mass had disappeared and the macroscopically involved areas of liver were removed. Histologic examination demonstrated that the complete necrosis of the tumor was partially replaced by a fibrous scar tissue. The child underwent adjuvant chemotherapy and now is in complete remission fourteen months after the last operation.     

Emergence of mature teratoma following treatment of sacrococcygeal endodermal sinus tumor: CT and MR imaging with pathological correlation

We report a case of pseudoretroconversion in which, clinically and pathologically, it appeared that an endodermal sinus tumor retroconverted to mature teratoma. An 8-month-old white female infant with a sacrococcygeal tumor, diagnosed by biopsy as endodermal sinus tumor (yolk sac tumor), was treated with chemotherapy. The residual tumor after treatment was mature teratoma confirmed by imaging and by pathological examination. We present the computerized tomography (CT) and magnetic resonance imaging (MRI) findings with pathological correlation.     

Endodermal sinus (yolk sac) tumor in infants and children. A clinical and pathologic study: an 11 year review

We reviewed the clinical features, treatment, and results of children with gonadal and extragonadal yolk sac (endodermal sinus) tumors seen in the King Faisal Specialist Hospital and Research Centre between 1976 and 1987. There were nine children (seven girls and two boys) with ages ranging from 7 months to 12 years (median of 3.5 years). Sites of origin included the vagina (two cases), face (two cases), sacrum (two cases), mediastinum (one case), ovary (one case), and testicle (1 case). All children had elevated alpha-fetoprotein (AFP) at diagnosis. One girl had complete surgical excision of an ovarian tumor at the time of diagnosis, and one boy had surgical excision of the testis. In the remaining seven children, the tumor was unresectable. Surgery was limited to a biopsy in six children. All patients received different combinations of chemotherapy, including vincristine (VCR), actinomycin D (Act-D), cyclophosphamide (Cyclo), adriamycin (Adria), bleomycin (Bleo), cis-platinum (CDDP), vinblastine (VBL), and VP-16. Of the nine patients, one was lost to follow-up while in remission, five died, one was lost to follow-up, and three are alive and disease-free at 15, 55, and 67 months from diagnosis. This review demonstrates an unusual preponderance of the extragonadal form of endodermal sinus tumor among our patients.     

Half-Life of Alpha-Fetoprotein in Patients with a Teratoma, Endodermal Sinus Tumor, or Hepatoblastoma

To evaluate the AFP-concentralion postoperatively and/or during chemotherapy treatment, a retrospective study was carried out on 14 patients with a teratoma, endodermal sinus tumor (EST), or hepatoblastoma. Eleven patients were operated. Eight of the 11 patients showed postoperatively a linear decline in their AFP concentration. The half-life was about 6 days. In the group of patients that underwent a noncomplete resection, both a linear and a nonlinear declining pattern was seen. The patients that underwent a complete resection showed only a linear decline in the AFP concentration. No relationship was found between the reduction pattern of the AFP and the development of recurrences. The development of a recurrence was accompanied by an increasing AFP concentration.     

Endodermal sinus tumor of the vagina

Malignant germ cell tumors of the vagina represent a critical site for local treatment. We report on our experience with two vaginal endodermal sinus tumors. Treatment with PEB regimen induced both tumor regression and alpha fetoprotein normalization. One patient had a residual lesion that was biopsied twice and no viable tumor cells were ever found. Both patients remained alive and disease-free, without having had surgical procedures except for biopsies. Platinum-based chemotherapy is able to achieve complete remissions and should be considered for vaginal endodermal sinus tumors.     

Successful treatment of an endodermal sinus tumor of the vagina by chemotherapy alone: a rare case of an infant diagnosed by pathological examination of discharged tumor fragment

A 7-month-old infant was noted to have vaginal bleeding that was accompanied by a discharged tumor fragment. The histological diagnosis was endodermal sinus tumor. Her serum alpha-fetoprotein (AFP) was increased to 358.7 ng/mL, and magnetic resonance imaging showed a 1.8 x 1.0 cm tumor in the vagina. She received combination chemotherapy with cyclophosphamide, pirarubicin, carboplatin, and etoposide. The tumor in the images disappeared and the serum level of AFP returned to the normal range after 2 cycles. Treatment was complete without surgical or radiological therapy. More than 45 months after the completion of chemotherapy, she is alive without signs of recurrence.     

Yolk sac tumor with a unique uniform hepatoid pattern histology

Abstract Background: Yolk sac tumors (YST) exhibit several different histological subtypes and the mechanisms of cellular differentiation and prognosis of each subtype remain unknown.
Results: We report two infants with sacrococcygeal YST; one represented a typical histological subtype and the other showed a hepatoid subtype with uniform histology. The isoform of alpha-fetoprotein (AFP) in the patient with the hepatoid pattern was examined by lectin-affinity Immunoelectrophoresis and represented as a YST. but not hepatocellular, subtype. The patient with typical YST responded well to VAB-6 combination chemotherapy. However, this regimen was only partially effective to the patient with the pure hepatoid histological subtype, and an etoposide with ifosfamide and cisplatin (VIP) regimen as a salvage chemotherapy combined with complete tumor resection was useful to achieve complete remission (CR). Both of the patients have been in CR for more than four years.     

Vaginal endodermal sinus tumor

Malignant germ cell tumors are rare tumors of childhood accounting less than 3% of pediatric malignancies, and endodermal sinus tumor (EST) is the most common histological subtype. The vagina is an extremely rare site for germ cell tumors (GCT). A one-year female was admitted with history of bleeding pervagina. She had pallor and a mass was palpable anteriorly on rectal examination. Magnetic Resonance Imaging (MRI) showed a tumor mass at the junction of cervix and vagina and biopsy was suggestive of a vaginal EST. The serum alpha fetoprotein (AFP) was elevated. She underwent vaginohysterectomy. The tumor was excisedin toto and patient received four courses of cisplatin, etoposide and bleomycin. At one year of follow up, patient was symptom free and serum AFP remained at normal level. EST of the vagina is a rare, highly malignant GCT that exclusively involves children less than 3 years of age. It is both locally aggressive and capable of metastasis. Even though more conservative surgery is advised to maintain sexual and reproductive function, at times, radical surgery is mandatory depending on the infiltration of the tumor to the surrounding structures. Simple tumor excision is not sufficient, as residual cells induce recurrence and make chemotherapy ineffective. The serum AFP level is a useful marker for diagnosis and monitoring the recurrence of vaginal EST in infants.