小儿复发性骶尾部畸胎瘤临床研究

目的 探讨骶尾部畸胎瘤(SCT)复发原因及降低SCT复发率的合理治疗方案。方法 回顾性分析12例复发性骶尾部畸胎瘤(RSCT)疗效。结果 12例RSCT，其中良性7例，恶性5例，施行骶尾路手术切除9例，腹骶尾联合切除3例。5例行化疗。随访1～20年，存活9例，恶性SCT复发转移于术后10月内死亡3例。结论 良性SCT复发主要原因是肿瘤和尾骨残留。恶性复发为瘤组织局部侵蚀，以及淋巴、血道转移。化疗可降低复发和死亡率。     

小儿恶性肿瘤介入治疗进展

常用的肿瘤介入治疗包括经导管的动脉化疗栓塞(TACE)和经导管的动脉灌注化疗(TAIC或IAC)。TACE是将化疗药与栓塞剂注入肿瘤供血动脉,适用于肝、肾肿瘤。TAIC用于其他部位的肿瘤,只注入化疗药物而不用栓塞剂,进行局部的动脉大剂量冲击化疗,而避免邻近脏器受到栓塞。介入治疗所用的化疗药物有卡铂或顺铂,多柔比星或表柔比星,5-氟尿嘧啶,长春新碱或长春地辛等。栓塞剂有碘油、明胶海绵颗粒、微球颗粒等。根据肿瘤的状况,介入治疗可间隔4周重复1～3次,直至估计手术能完整切除肿瘤。国内TACE应用最多的儿童肿瘤为肝母细胞瘤和晚期肾母细胞瘤。TAIC被用于盆腔、卵巢、阴道、膀胱、骶尾部、腹腔、大网膜、肠系膜根部、臀部、上臂等部位恶性肿瘤的治疗。介入治疗与静脉用药全身化疗的交替应用具有更好的治疗作用。     

选择性动脉灌注化疗治疗小儿卵巢恶性生殖细胞瘤

近年来，随着介入放射学的发展，选择性动脉灌注化疗已逐渐在临床应用，此法与全身化疗的区别在于前者经位于肿瘤供血动脉内的导管，向肿瘤组织灌注化疗药物，由于局部血药浓度高，对肿瘤的杀伤作用远远超过后者，不良反应也较后者轻。我院自1997年4月～2001年2月，应用该技术治疗2例全身静脉化疗无效的复发性小儿卵巢恶性生殖细胞瘤，取得一定的疗效，现报告如下。     

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.     

腔镜技术在小儿肿瘤切除手术中的应用及技巧探讨

目的探讨腔镜切除小儿肿瘤的可行性、临床效果、操作技巧与应用前景等,总结临床经验。方法回顾性收集2009年1月至2016年12月经胸腔镜、腹腔镜治疗的221例肿瘤患儿作为研究对象,分析治疗效果。结果 221例肿瘤患儿中胸腔镜切除肺及纵膈肿瘤53例,腹腔镜切除腹腔、盆腔及腹膜后肿瘤168例。其中2例腹膜后肿瘤中转开腹手术; 5例回盲部肿瘤,33例卵巢囊肿及19例卵巢畸胎瘤腹腔镜探查后经脐部拖出切除;其余手术均在腔镜下完成。术中无副损伤、无输血,术后Trocar口感染9例,瘢痕疙瘩5例;术后住院时间平均(5±1. 6) d。术后随访发现2例肺囊肿出现复发,行2次手术切除后治愈; 1例肠系膜淋巴管瘤出现黏连性肠梗阻,保守治疗后好转; 1例膀胱横纹肌肉瘤术后半年死亡; 1例腹膜后未成熟畸胎瘤化疗后死亡;其余恶性肿瘤患儿生存良好,恶性肿瘤生存率为80%(8/10)。结论腔镜切除小儿肿瘤就安全可行的;囊性肿瘤抽吸减压后经脐部拖出操作便捷、创伤小,囊实性肿瘤可以减压后拖出切除,也可全腔镜下切除;小的实性肿瘤不论良性、恶性均可腔镜更好切除;瘤体较大人实性肿瘤要适时中转开放手术。     

儿童骶尾部畸胎瘤手术治疗

目的 总结儿童畸胎瘤的手术措施,分析手术并发症的常见原因,以提高肿瘤切除率,减少手术并发症.方法 对2002年2月-2011年5月本院收治的12例儿童骶尾部畸胎瘤的临床资料进行回顾性分析.男4例,女8例;年龄7个月～11岁,平均 6.67 岁.按Altman分类:Ⅰ型(显露型)5例,Ⅱ型(内外混合型)5例,Ⅲ型(哑铃状型)1例,Ⅳ型(隐匿型)1例.术前空腹血清AFP正常7例(＜20 μg·L-1),5例有不同程度的增高,其中2例 >1 210 μg·L-1.所有患儿均行手术切除肿瘤及尾骨,Ⅰ、Ⅱ、Ⅲ型患儿中9例采用经骶尾部倒"V"形切口切除术,2例因瘤体较大采取横梭形切口切除术,1例Ⅳ型患儿采用经腹骶联合切口切除术.恶性肿瘤患儿术后行联合化疗.结果 本组瘤体完整切除10例,术中瘤体包膜破溃切除2例.病理检查证实良性7例,恶性5例.12例患儿术后全部进行随访,随访时间6个月～ 5 a.近期并发症有切口感染3例,直肠损伤1例;远期并发症小便失禁1例.术后复发2例,死亡1例.结论 骶尾部畸胎瘤须尽早手术治疗.术中切除尾骨、避免肿瘤破溃残留是预防复发的关键.盆底修复,肛尾韧带重建及消灭死腔是减少术后并发症的关键.     

399例儿童畸胎瘤的单中心临床诊疗分析

目的回顾性分析小儿畸胎瘤的治疗及预后特点。方法收集重庆医科大学附属儿童医院1994年10月至2015年6月收治的畸胎瘤患儿,共399例,按照性别、年龄、部位、病理类型、血清AFP水平、治疗及预后等因素进行回顾性分析。结果①成熟型畸胎瘤301例;未成熟畸胎瘤17例,其中组织学Ⅰ级9例,Ⅱ级5例,Ⅲ级3例;恶性畸胎瘤81例,按COG/CCG肿瘤分期标准,Ⅰ期5例,Ⅱ期14例,Ⅲ期44例,Ⅳ期18例。②成熟型畸胎瘤及未成熟型畸胎瘤共318例一期接受手术治疗,其中未成熟畸胎瘤Ⅲ级及部分Ⅱ级病例术后辅以化疗;81例恶性肿瘤患儿中,Ⅰ、Ⅱ期均手术完全或完整切除肿瘤,部分Ⅱ期病例术后辅以化疗;40例接受术前新辅助化疗、手术治疗、术后化疗的个体化综合治疗;另外22例中断治疗并放弃;③术后随访1~10年,3例成熟型畸胎瘤患儿术后良性复发,再次予以完整切除后,截止随访时间,301例均无瘤存活;1例未成熟畸胎瘤Ⅲ级术后恶性复发,放弃治疗后死亡;恶性畸胎瘤完成治疗的患儿仅1例恶性复发后死亡,未完成治疗患儿中10例死亡,其余患儿各有不同程度的病情进展。结论在儿童畸胎瘤治疗中,一期完整切除肿瘤,避免肿瘤破溃是预防复发及恶变的关键,未成熟畸胎瘤依据AFP、影像学、肿瘤组织分级、病理免疫组化、术中情况等选择是否化疗;对于恶性畸胎瘤,彻底手术切除的同时结合化疗,术后密切随访,可获得较高的生存率。     

不可切除型肝母细胞瘤的术前介入治疗临床研究

目的探讨术前介入性动脉栓塞化疗（TACE）在不可切除型肝母细胞瘤治疗中的可行性。方法分析对8例经检查诊断为不可切除型肝母细胞瘤行TACE的治疗结果。结果8例患儿TACE后均有不同程度呕吐、发热、一过性肝功能损害，1例有骨髓抑制，无心、肾功能损害。有6例复查B超检查，肿瘤体积较前均有明显缩小，缩小比例为22．5％～57．5％；5例手术完整切除，均已无瘤存活1～4年，3例复诊检查发现有肺转移或多发转移，家属放弃进一步治疗后死亡。结论TACE具有化疗药物剂量小、全身副作用少、化疗效果好的优点，可作为一种术前治疗方法应用于不可切除型肝母细胞瘤。     

新生儿腹部实体肿瘤

目的通过总结我院收治的25例新生儿腹部实体肿瘤的临床资料，探讨新生儿腹部实体肿瘤的诊治经验。方法回顾分析1980年1月-2003年12月治疗25例新生儿腹部实体肿瘤，男15例，女10例，年龄从出生后3～30d，出生前经B超诊断胎儿腹部占位性病变9例，占36．0％，恶性实体瘤13例，占52．0％。肝母细胞瘤7例，肝错构瘤2例，神经母细胞瘤5例，先天性中胚层肾瘤2例，腹膜后良性畸胎瘤4例，胃恶性畸胎瘤1例，卵巢囊肿2例，肾上腺错构瘤1例，肾上腺腺瘤1例。肿瘤完整切除20例，肿瘤部分切除1例，仅行肿瘤活检4例。结果肝母细胞瘤存活2例，死于化疗毒副反应1例，失访4例，肝错构瘤生存2例，神经母细胞瘤生存4例，死于化疗毒副反应1例。除1例胃恶性畸胎瘤术后失访外，其余病例均长期生存。结论新生儿腹部实体肿瘤罕见，但通过产前B超可早期发现．出生后小儿外科医师应早期介入，根据病种采取合理检查及治疗手段，包括早期手术，术后合理、规范的化疗，可提高患儿的救治率。     

儿童输尿管肿瘤的诊治分析

目的 总结儿童输尿管肿瘤的病理类型、临床表现、诊断、治疗和预后,提高对该病的认识.方法 回顾性分析2011年1月至2015年10月我院收治的5例输尿管肿瘤患儿的临床资料,结合文献总结儿童输尿管肿瘤的病理类型、临床表现、诊断、治疗及预后.5例患儿均为男性,输尿管炎性肌纤维母细胞瘤3例,2例以腹痛就诊,分别为6岁和10岁,肿瘤位于输尿管下段,均行输尿管下段肿物切除+输尿管端端(输尿管膀胱)吻合,1例以尿痛并血尿就诊,年龄1岁9月龄,肿物来源于下段输尿管,经输尿管膀胱连接部长入膀胱,继发膀胱输尿管积水,经输尿管和膀胱联合切除肿物+输尿管膀胱再植术.结果 术后随访5~48个月,未见肿瘤复发及转移.恶性横纹肌样瘤1例6岁,以左下腹痛2周就诊,输尿管肿瘤边界不清与腰大肌肉和后腹膜粘连,切除肿物行输尿管端端吻合,术后ICE方案化疗一疗程后局部复发,放弃治疗,术后233d死亡.尤文/原始神经外胚层瘤1例12岁,以右侧腰痛10d就诊,肿瘤位于输尿管髂血管水平,行输尿管肿瘤切除术+输尿管端端吻合术,术后予CAV+IE化疗12个月,随访56个月,未见转移及复发.结论 输尿管肿瘤在儿童发病率极低,文献曾报道的病理类型包括炎性肌纤维母细胞瘤、恶性横纹肌样瘤、尤文/原始神经外胚层瘤、横纹肌肉瘤,术前无特异方法诊断,手术完整切除肿瘤并重建输尿管是治疗的主要方法,确诊需要结合病理检查,根据病理类型决定是否化疗,治疗后均需要长期随访.     

Growth hormone therapy and the risk of tumor recurrence after brain tumor treatment in children

To assess the effect of human growth hormone (hGH) therapy and other factors on tumor recurrence after treatment of pediatric brain tumors (BTs), we retrospectively analyzed data from 108 craniopharyngioma, medulloblastoma, and ependymoma patients. Risk factors were identified using multifactorial univariate regression analysis. Recurrences occurred in 41 and second malignant neoplasms in 4 patients. There were significant correlations for completeness of tumor removal and recurrence-free survival (RFS). 13/44 hGH-treated and 28/59 non-hGH-treated children relapsed. This difference was found only for medulloblastomas and accounted for by higher rates of incomplete tumor removal in non-hGH patients. Craniopharyngioma recurrence correlated only with RFS. Malignant BT recurrence correlated with completeness of tumor removal, chemotherapy, and RFS. 4 children developed SMNs, 3/4 after hGH therapy. Our regression model yielded accurate within-sample prediction of recurrence for 90% of the study population. We conclude that hGH therapy after treatment of pediatric BTs does not increase tumor recurrence risk.     

Medulloblastome, primitiv neuroektodermale Tumoren und Ependymome

Medulloblastoma, primitive neuroectodermal tumor (PNET), and ependymoma are the most frequent malignant brain tumors of childhood. Since the 1980s, multimodal therapy optimization trials of the German Society of Pediatric Oncology and Hematology (GPOH) have led to improved survival rates. Within the current primary treatment trial HIT 2000, modern strategies for radiotherapy and chemotherapy are evaluated according to histology, staging, and age after maximal safe tumor resection. Young children with desmoplastic medulloblastoma receive chemotherapy alone, and the aim for young children with classic medulloblastoma and PNET is for chemotherapy to delay radiotherapy. Intensified treatment concepts are evaluated in metastatic disease. In ependymoma, the extent of resection and local radiotherapy are most important. Prolonging survival while preserving a good quality of life is the aim of the trial HIT-REZ 2005 for children with relapsed medulloblastoma, PNET, or ependymoma; it includes the evaluation of new drugs and treatment modalities (temozolomide, intraventricular etoposide). Central tumor banking and improved treatment stratification by prognostic molecular markers are planned for future trials.     

Primary cerebellar histiocytic sarcoma in a 17-month-old girl

The authors report on a case of histiocytic sarcoma (HS) in a pediatric patient presenting with a solitary tumor in the cerebellum, with the aim of providing insight into primary HS in the CNS, which is especially rare. A 17-month-old Japanese girl presented with a 2-week history of progressive gait disturbance. Brain MRI revealed a 4.7 × 4.3 × 4.3-cm well-demarcated solitary mass in the right hemisphere of the cerebellum, initially suggestive of medulloblastoma, ependymoma, or anaplastic astrocytoma. On intraoperative inspection the cerebellar tumor showed intensive dural attachment and was subtotally removed. Histological and immunohistochemical findings were consistent with HS. The patient subsequently received chemotherapy, and her preoperative neurological symptoms improved. Primary HS in the CNS usually demonstrates an aggressive clinical course and is currently considered to have a poor prognosis. The possibility of this rare tumor should be included in the differential diagnosis of localized cerebellar tumors in the pediatric age group.     

Sacrococcygeal teratoma

This retrospective study details our experience regarding 72 patients with sacrococcygeal teratoma treated over a period of 17 years. The sex incidence was nearly equal, but there was a high proportion of Altmann type IV tumors. A preliminary colostomy before combined abdominosacral excision of large type III and IV lesions reduced morbidity. Sixty-six percent of the patients presented beyond the neonatal period; 14 had been treated elsewhere for bowel/urinary obstruction. Imaging studies included radiography, abdominal ultrasound, computed tomography, and magnetic resonance imaging (after 1995). In 60 patients the tumor was excised via the sacral route, 11 had a preliminary colostomy, and 1 had a vesicostomy. Eight children (5 with malignant lesions) required abdominosacral excision. After 1990, serial estimation of serum alpha-fetoprotein (AFP) was used to monitor tumor recurrence. There were 34 male and 38 female patients (age range 3 days-12 years); 47 had benign tumors, of which 42 were excised through the sacral route. Three patients underwent a preliminary colostomy and abdominosacral excision of the tumor with subsequent colostomy closure. There were 4 deaths in this group; no recurrence was seen in the surviving children with benign tumors. Twenty-five patients had malignant teratomas. In 18 of these the tumor was excised via the sacral route and 5 underwent abdominal-sacral excision. Eight had a preliminary colostomy and chemotherapy followed by excision of the residual tumor and colostomy closure. None of the initial 14 patients with malignant lesions survived beyond 2 years. Of the latter 11 (who received cisplatinum-based chemotherapy), 10 were alive 1 year after surgery. One patient is currently on preoperative chemotherapy and another developed recurrence of the tumor. The overall follow-up ranged from 3 months to 8 years; there has been no complaint of functional neurological deficit in any of the patients. As intrapelvic tumors tend to have a delayed diagnosis, this can be avoided by performing a rectal examination. There should be no recurrence after excision of a benign teratoma. Cisplatinum-based chemotherapy has improved the survival of patients with malignant tumors.     

Pattern of recurrence in children with midline posterior fossa malignant neoplasms

BACKGROUND: Surveillance imaging of the brain and spinal neuraxis in patients with posterior fossa malignant tumors is commonly performed, with the assumption that early detection of tumor recurrence will improve outcome. However, the benefit of this imaging has not been proven. PURPOSE: To evaluate the usefulness of spinal surveillance imaging in children with nonmetastatic (at diagnosis, M0) posterior fossa ependymoma and medulloblastoma. MATERIALS AND METHODS: This retrospective study included 65 children (3 months to 16 years, mean 5.7 years) treated between 1985 and 1997 for ependymoma (22) and medulloblastoma (43). Medical records were reviewed for pathology and treatment data. Serial imaging of the head and spine was reviewed for evidence of tumor recurrence. RESULTS: Twenty-four patients (37 %) had tumor recurrence, including 13 with ependymoma and 11 with medulloblastoma. Of the 17/24 recurrent patients initially diagnosed as M0 (6 medulloblastoma and 11 ependymoma), 13 (76 %) had a cranial recurrence only, and 4 (24 %) presented with concomitant cranial and spinal recurrence. No M0 patient presented solely with spinal metastases at recurrence. CONCLUSION: This study suggests that spinal surveillance imaging in patients with posterior fossa ependymoma or medulloblastoma initially staged as M0 may not be useful, as these patients initially recur intracranially. Thus, until an intracranial recurrence is detected, these patients may be spared the time, expense and sedation risk necessary for spinal imaging.     

Ovarian germ cell tumors in children: a clinical study of 66 patients

BACKGROUND: Ovarian germ cell tumors are rare in childhood. The aim of this study is to review clinical presentation, management, and outcome in a two-center series of girls with ovarian germ cell tumor. PROCEDURE: The records of 66 patients (median age 9 years) with histologically proven ovarian germ cell tumor (either benign or malignant), treated over a 44-year-span, were reviewed. RESULTS: Pain and an abdominal mass were the most frequent symptoms. The tumors were right-sided in 35, left-sided in 28, and bilateral in 3. Most patients (52) were stage I, 4 were stage II, 6 stage III, and 1, with liver metastases, stage IV. Sixteen patients had an emergency operation for tumor torsion. Unilateral salpingo-oophorectomy was the most frequently performed procedure (n = 46), and ovarian-sparing tumorectomy was performed in 9 patients (one bilaterally). Histologically, teratomas were found most frequently (mature: 45, immature: 9), followed by mixed tumors (n = 7), yolk sac tumors (n = 3), dysgerminoma (n = 2), gonadoblastoma (n = 2), and embryonal carcinoma (n = 1). Surgical removal of the tumor with or without the ovary and/or adnex was the sole treatment in 55 patients, chemotherapy was administered in 10 and radiotherapy + chemotherapy in one. Intra-operative spillage of tumoral fluid occurred in six; this did not influence outcome in five. Recurrence was observed in three patients. Two patients, with malignant disease, died. The 64 survivors are now between 8 months and 44 years after treatment. CONCLUSIONS: With a recurrence rate of 4.5% and a mortality rate of 3%, this series confirms the excellent prognosis for girls with ovarian germ cell tumor (GCT).     

Permanent low-activity (125)I seed placement for the treatment of pediatric brain tumors: preliminary experience

Although external beam radiation therapy is effective in the treatment of many pediatric brain neoplasms its use in this patient population has been associated with the development of significant cognitive and endocrine dysfunction and is severely limited as an option in previously irradiated patients. Therefore, we have adopted a strategy for management of residual microscopic disease by implantation of low-activity (125)I seeds in the tumor bed at the time of surgery. Six patients aged 2-14 years with recurrent tumors including two supratentorial primitive neuroectodermal tumors (n = 2), one medulloblastoma, one malignant ependymoma (n = 1), glioblastoma (n = 1) and one pleomorphic xanthoastrocytoma were implanted at the time of reoperation. A total of 11-126 seeds were implanted resulting in total doses of 16-21.8 Gy (after theoretical infinite time) at a depth of 5 mm from the implanted resection bed. Five patients had prior external beam radiation while the other patient (2 years old at initial diagnosis) progressed after surgery and chemotherapy. Two patients had lasting local tumor control. One patient is alive at 390 weeks of follow-up and another who died of distant failure at 366 weeks had no recurrence on MRI at 333 weeks' follow-up. Only 2 patients had first local failures. These results suggest that the use of permanent low-activity (125)I seeds as an adjunct to surgery can provide good local tumor control and is a suitable treatment option for pediatric patients.     

Second surgery for recurrent pilocytic astrocytoma in children.

Pilocytic astrocytoma (PA) is the most common childhood brain tumor. In cases where the tumor progresses or recurs following primary surgical resection, the appropriate treatment is unclear. Options include chemotherapy, radiation therapy, surgical resection or a combination thereof. To analyze the utility of further surgery, we performed a retrospective, single-institution review of pediatric patients with recurrent PAs from 1990 to 1999 who were treated with a second surgical resection. Patients were excluded if they received adjuvant chemotherapy or radiation therapy. Twenty cases were identified. Tumor locations included: cerebral hemisphere (3), cerebellum (7), optic pathway/hypothalamus (5), thalamus (1) and brainstem (4). The indication for 4 surgeries included an enlarging tumor-associated cyst. At second surgery, 10 of 20 patients had a gross total resection (GTR), 2 a near total resection (NTR), and the remaining 8 patients had a subtotal resection (STR). No patients have died. Two of 10 tumors after GTR, 0 of 2 tumors after NTR, and 7 of 8 tumors after STR had second recurrence/progression at a mean of 15 months (range 4-33 months) following second surgery. The remaining 11 patients are recurrence/progression-free at a mean of 40.7 months (range 19-119 months). Surgery for tumors or midline structures rarely resulted in a GTR (1 of 10 cases). Surgery for tumors located in the cerebral hemispheres or cerebellum resulted in GTR or NTR in all cases and can result in long periods of progression-free survival without further adjuvant treatment.