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。     

小儿骶尾部内胚窦瘤的治疗

目的探讨骶尾部内胚窦瘤的治疗和预后。方法回顾性分析1999年1月-2005年12月收治12例骶尾部内胚窦瘤患儿。该组病例术前经B超、CT或MRI检查发现骶尾部肿瘤,经检测血清甲胎蛋白均升高,采用手术切除全部或大部分肿瘤,所有患儿均采用BEP（顺铂十足叶乙甙＋博来霉素）方案化疗。结果该组病例术前经B超或CT检查发现骶尾部肿瘤,经检测血清甲胎蛋白（AFP）均升高,采用手术切除全部或大部分肿瘤,病理证实为内胚窦瘤。患儿均采用BEP（顺铂＋足叶乙甙＋博来霉素）方案化疗。随访6年,生存9例,死亡3例,复发5例。结论以顺铂为基础的联合化疗可改善内胚窦瘤疗效,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,选择恰当的手术方式并辅以联合化疗可提高患儿长期生存率。     

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。     

小儿卵巢肿瘤34例临床分析

目的：了解影响小儿卵巢肿瘤生存率的因素，探讨合理的治疗方案以提高治愈率。方法：３４例小儿卵巢肿瘤患儿为１９７３～１９９５年所收治，年龄４～１４岁，右侧１８例，左侧１６例，其中３例合并蒂扭转。均经手术及病理证实。良性２４例（７０．６％），恶性１０例（２９．４％）。结果：２９例获随访。２４例良性畸胎瘤术后１例复发；２例恶性畸胎瘤术后无瘤生存期分别为６年和２年；３例内胚窦瘤术后均死于肿瘤广泛转移。结论：对于良性卵巢肿瘤主张行患侧附件切除。对于Ⅰ、Ⅱ期恶性肿瘤除行患侧附件切除外，应剖视对侧卵巢，并进行活检、术后化疗及严密随访。对Ⅲ、Ⅳ期恶性卵巢肿瘤强调作根治性手术，尽可能切除原发灶及所有转移病灶，同时术后化疗，以达长期缓解。     

儿童原发性恶性非肾母细胞瘤性肾脏肿瘤诊治特点

目的 探讨儿童原发性恶性非肾母细胞瘤性肾脏肿瘤的临床诊治特点.方法 回顾性分析1993年4月至2008年1月问收治的11例儿童原发性恶性非肾母细胞瘤性肾脏肿瘤患儿的临床资料.根据临床表现及术前影像学检查并于术前行穿刺活检,治疗方法主要为术前介入和/或全身化疗、手术切除、术中热灌注化疗和术后化疗.结果 肾细胞癌6例,无瘤长期(平均32个月)生存率66.7%.中胚性肾瘤3例,均获3年以上无瘤生存.肾透明细胞肉瘤1例,术后复发死亡.肾横纹肌样瘤1例,术后化疗2个月复发,结论儿童原发性恶性非肾母细胞瘤性肾脏肿瘤发病率低,临床表现与肾母细胞瘤相似,术前诊断较为困难,其中肾细胞癌发病年龄多为年长儿,而肾透明细胞肉瘤、中胚层肾瘤和肾横纹肌样瘤则多见于小婴儿.经过多项系统性治疗,中胚层肾瘤预后较佳,肾细胞癌次之.     

小儿胸膜肺母细胞瘤的诊断与治疗分析

目的初步探讨小儿胸膜肺母细胞瘤的临床特征、诊断与治疗方法。方法回顾性分析上海市儿童医院2013年10月至2017年6月收治的6例胸膜肺母细胞瘤患儿临床资料,其中男3例,女3例;年龄范围1岁2个月至10岁。5例患儿行肿瘤大部分切除(其中部分肺叶切除术1例,肺叶修补术4例),1例行肿瘤完整切除。6例均获得有效随访,随访时间1~34个月。结果 6例患儿中5例首诊被误诊,其中3例首诊为肺炎,1例首诊为胸腔积液,1例首诊为先天性肺囊性腺瘤样畸形。6例患儿随访结果分别为:临床痊愈1例;带瘤生存1例(目前正规接受化疗中);术后1个月死亡3例;术后2个月死亡1例。结论小儿胸膜肺母细胞瘤临床表现无特异性,Ⅰ型胸膜肺母细胞瘤易误诊为先天性肺囊性腺瘤样畸形,术后预后相对理想;早期发现、早期手术、后续正规化疗是治疗的主要方法。     

小儿卵巢肿瘤67例诊治分析

目的探查小儿卵巢肿瘤的特点及治疗方法。方法回顾性分析本院1998—2007年手术治疗的67例卵巢肿瘤患儿的临床资料。结果67例中,良性肿瘤改变60例,其中成熟性畸胎瘤51例,未成熟性畸胎瘤3例,卵巢囊肿6例。恶性肿瘤改变占10．45％,包括恶性畸胎瘤5例,内胚窦瘤1例,胚胎性癌1例。发病年龄均在10～14岁。卵巢肿瘤蒂部扭转17例,占25．37％。成熟性畸胎瘤经手术治疗痊愈,预后良好,7例恶性肿瘤经手术切除加化疗,5例术后无复发,复查AFP正常,2例失访。结论小儿卵巢肿瘤以成熟性畸胎瘤多见,常见并发症为瘤蒂扭转。随年龄增长,恶性程度增高。治疗后预后良好。     

小儿肾上腺皮质肿瘤10例报告

目的 探讨小儿肾上腺皮质肿瘤的诊断、治疗及影响预后的因素。方法 回顾性分析1982～2002年间我院收治的10例病例,男4例,女6例,年龄6个月～12岁,平均(4.0±3.5)岁,皮质腺瘤7例,皮质癌3例,均有内分泌功能紊乱,评价治疗结果。结果 肾上腺皮质腺瘤7例,5例手术完整切除肿瘤,4例随访6个月～17年,均健在。肾上腺皮质癌3例,2例手术加化疗,分别存活6个月及1年;1例仅作活检,存活3个月。结论 肾上腺皮质腺瘤通过完整切除肿瘤,围手术期及术后激素监控替代治疗,可获治愈。肾上腺皮质癌需手术加化疗综合治疗,预后仍不佳。由于术前尚无可靠指标可区别肿瘤的良恶性,早期诊断和完整切除肿瘤至关重要。     

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.     

小儿先天性中胚叶肾瘤

目的：探讨小儿先天性中胚叶肾瘤（ＣＭＮ）的临床特点、诊断及治疗。方法：近２０年间收治ＣＭＮ７例，男４例，女３例，年龄７个月～１０岁。术前均可扪及一腹部肿块，其中１例伴肉眼血尿，病变位于左侧４例，右侧３例。全部病例均接受了手术治疗，２例证实有肾门旁肿瘤转移，病理检查属平滑肌瘤型５例，细胞型２例，因术前及术中疑诊为Ｗｉｌｍ＇ｓ瘤而接受了不同疗程化疗。结果：除１例２个月龄患儿术后死于窒息外，余６例术后均随访，１例术中肿瘤破裂者术后８个月复发，化疗无效后再次手术已无瘤生存９年，余５例无瘤生存至今，最短５年，最长已１５年。结论：ＣＭＮ是一罕见的肾肿瘤，虽好发于新生儿及婴儿早期，但也可见于年长儿，其临床表现和病理改变虽有诸多特点，但有时仍难与Ｗｉｌｍ＇ｓ瘤区别，手术切除是治疗本病最有效的方法     

Endodermal sinus tumour of the omentum in a child

Endodermal sinus tumour usually arises in a gonad; extragonadal endodermal sinus tumours are rare. We report a 3-year-old boy with an endodermal sinus tumour arising in the greater omentum, which may be the second reported case in the English literature. He presented with a solid mass in the upper abdomen and a markedly raised serum -fetoprotein (AFP) level. Following percutaneous needle biopsy and omentectomy, histological examination revealed classic morphological features of an endodermal sinus tumour. This rare diagnosis is possible from the imaging features in association with a raised serum level of AFP.     

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.     

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.     

14例肝母细胞瘤患儿的临床疗效观察

目的：通过对儿童肝母细胞瘤患儿的临床治疗结果的回顾总结,对ICE化疗方案的有效性和安全性进行评估。方法：自2000年6月至2008年6月,14例初发患儿入选,男7例,女7例,中位年龄：1.33岁（范围0.25～8.25岁）。临床分期：Ⅰ期6例,Ⅱ期1例,Ⅲ期5例,Ⅳ期2例。诊断时血甲胎蛋白（AFP）水平显著升高13例,1例AFP正常。采用多科室协作模式进行治疗,其中一期手术8例,3例进行了二期手术。化疗方案采用ICE方案,14例患儿共接受了73个疗程化疗,其中术前化疗25个疗程。结果：14例患儿治疗后有效12例（85.7%）,其中完全缓解10例（71.4%）,部分缓解2例,2例无效。随访至2008年7月31日,疾病处于长期完全缓解者9例（64.3%）,中位随访时间为35个月（范围：16～96个月）。5年总生存率（OS）为：（70.71±12.37）%,5年无事件生存率（EFS）为：（64.29±12.81）%。1例患儿复发,2例失访。结论：ICE化疗方案联合手术治疗能有效并且安全地治疗儿童肝母细胞瘤,Ⅳ期患儿的治疗有待于进一步研究。［中国当代儿科杂志,2009,11（8）：659-662］     

TREATMENT OF ALPHA-FETOPROTEIN SECRETING HEPATOBLASTOMA BY RESPONSE OF SERUM ALPHA-FETOPROTEIN LEVELS: A New Concept

Hepatoblastoma, thecommonest primary malignant livertumorin infants and children, is usually associated with elevated serum alpha-fetoprotein (AFP) levels. The authors sought to determine if AFP levels can be used to modify treatment, thereby avoiding the wait for formal imaging studies and prolonged suboptimal treatment and limiting the use of effective but toxic chemotherapy. From April 1984 to December 1997, 8 children were diagnosed with AFP-secreting hepatoblastoma. Serum AFP levels were measured weekly. If AFP levels failed to improve, or increased on at least 2 successive examinations, the chemotherapy protocol was changed. When an excellent response was achieved, less toxic chemotherapy was substituted. Six patients (75%) were disease-free for at least 2 years, some with high-risk or metastatic disease. Two patients died. Six of the 7 nonmetastatic patients (86%) remain disease-free (only one had a resectable tumor). Chemotherapy changes resulted in reduced AFP levels in 7 patients. This study supports the use of AFP monitoring to modify treatment in hepatoblastoma responding to therapy with less toxic drugs and the use of nonstandard therapy when suboptimal responses are obtained.     

TREATMENT OF ALPHA-FETOPROTEIN SECRETING HEPATOBLASTOMA BY RESPONSE OF SERUM ALPHA-FETOPROTEIN LEVELS: A New Concept

Hepatoblastoma, thecommonest primary malignant livertumorin infants and children, is usually associated with elevated serum alpha-fetoprotein (AFP) levels. The authors sought to determine if AFP levels can be used to modify treatment, thereby avoiding the wait for formal imaging studies and prolonged suboptimal treatment and limiting the use of effective but toxic chemotherapy. From April 1984 to December 1997, 8 children were diagnosed with AFP-secreting hepatoblastoma. Serum AFP levels were measured weekly. If AFP levels failed to improve, or increased on at least 2 successive examinations, the chemotherapy protocol was changed. When an excellent response was achieved, less toxic chemotherapy was substituted. Six patients (75%) were disease-free for at least 2 years, some with high-risk or metastatic disease. Two patients died. Six of the 7 nonmetastatic patients (86%) remain disease-free (only one had a resectable tumor). Chemotherapy changes resulted in reduced AFP levels in 7 patients. This study supports the use of AFP monitoring to modify treatment in hepatoblastoma responding to therapy with less toxic drugs and the use of nonstandard therapy when suboptimal responses are obtained.     

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.     

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.