经翼点入路显微手术切除小儿颅咽管瘤20例

目的　探讨提高手术根治颅咽管瘤的方法。方法　对 2 0例颅咽管瘤 ,均在全麻下行翼点入路 ,采用显微外科技术 ,根据肿瘤的位置 ,采用视交叉前间隙、颈内动脉和视神经间隙及颈内动脉外侧等间隙切除肿瘤。结果　全切除 16例、近全切除 3例、大部切除 1例。近期明显好转 14例、改善 4例、无变化及死亡各 1例。半年以上随访 18例 ,能正常生活者 16例 ,生活需人照顾 2例。结论　翼点入路手术能满足鞍区多种类型颅咽管瘤的显露 ,能提高肿瘤全切率及治愈率。     

56例儿童颅咽管瘤的显微手术治疗及随访

目的：探讨儿童颅咽管瘤显微手术方法,术后并发症的防治及复发的因素。方法：回顾性分析我院2000年1月至2010年12月儿童颅咽管瘤手术病例56例。分别采用经翼点入路28例（50%）、经额下入路14例（25%）、经前额纵裂入路6例（10.7%）,经胼胝体-透明隔间腔-穹隆间入路5例（8.9%）,皮层造瘘入路3例（5.3%）。结果：全切除＋次全切除38例（67.9%）、大部切除17例（30.4%）,围手术期死亡1例（1.7%）。术后并发症主要为尿崩症、血电解质紊乱及癫痫。结论：恰当地选择手术入路、术中娴熟的显微神经外科操作、术后积极防治并发症,是影响颅咽管瘤复发及预后的关键因素。     

经纵裂经终板入路切除颅咽管瘤：附14例临床分析

背景与目的：颅咽管瘤位置深在,毗邻重要的结构,手术全切肿瘤往往比较困难。本研究探讨经纵裂经终板入路切除颅咽管瘤的手术技巧。方法：回顾性分析了经纵裂经终板入路切除的14例颅咽管瘤的手术经过与疗效,重点讨论了手术入路技术、肿瘤切除技术和垂体柄的保护技术。结果：肿瘤全切除13例（92．8％）,次全切除1例,无手术死亡,术后视力症状（6例）均较术前好转,术后出现的尿量增多与低钠血症持续3—35天,给予垂体后叶素和常效尿崩停（弥凝）均可控制。结论：经纵裂经终板入路是切除颅咽管瘤的有效手术途径。     

56例儿童颅咽管瘤的显微手术治疗及随访

目的:探讨儿童颅咽管瘤显微手术方法,术后并发症的防治及复发的因素。方法:回顾性分析我院2000年1月至2010年12月儿童颅咽管瘤手术病例56例。分别采用经翼点入路28例(50%)、经额下入路14例(25%)、经前额纵裂入路6例(10.7%),经胼胝体-透明隔间腔-穹隆间入路5例(8.9%),皮层造瘘入路3例(5.3%)。结果:全切除+次全切除38例(67.9%)、大部切除17例(30.4%),围手术期死亡1例(1.7%)。术后并发症主要为尿崩症、血电解质紊乱及癫痫。结论:恰当地选择手术入路、术中娴熟的显微神经外科操作、术后积极防治并发症,是影响颅咽管瘤复发及预后的关键因素。     

46例颅咽管瘤显微外科治疗及预后影响因素分析

目的：探讨颅咽管瘤手术入路,影响全切除的因素及术后并发症的防治。方法：回顾性分析本院1995年1月至2005年1月颅咽管瘤手术病例46例。主要临床表现为头痛、视力视野障碍、下丘脑功能障碍、尿崩症。分别采用经翼点20例（43.5%）、经额下12例（26.1%）、经胼胝体-透明隔间腔-穹隆间入路3例（6.5%）,经前额纵裂入路5例（10.9%）,经额叶皮层造瘘-侧脑室室间孔入路2例（4.3%）、翼点联合侧脑室室间孔入路4例（8.6%）切除肿瘤。结果：全切除＋次全切除31例（67.4%）、大部切除15例（32.6%）,围手术期死亡1例（2.2%）。术后并发症主要为尿崩症、血电解质紊乱及癫痫。结论：选择恰当的手术入路、术者娴熟的显微神经外科技术及术中对下丘脑及重要穿支血管的保护、术后并发症积极防治是影响颅咽管瘤预后的关键因素。     

儿童期颅咽管瘤的诊断与经蝶显微手术治疗

 目的　提高儿童期颅咽管瘤的诊断水平与手术治疗效果。方法　回顾性总结 2 5例颅咽管瘤的诊断措施 ,手术技巧和治疗结果。本组均经 CT或 MRI扫描确诊。手术采取经蝶入路行肿瘤切除术。结果　 1 3例肿瘤获全切除 ;8例次全切除 ;余 4例为部分切除 ,术后无死亡。2 0例获长期随访 (平均 3.6年 ) ,其中 1 7例 (85.0 % )恢复良好 ,3例 (1 5.0 % )肿瘤复发 ,需行再次手术治疗。结论　对儿童期颅咽管瘤采用 CT或 MRI进行诊断并选择合适病例经蝶入路显微手术切除 ,是一种安全、有效的方法。     

纵裂入路显微外科切除鞍区肿瘤的研究

目的 探讨分析纵裂人路切除鞍区大型或巨大型不同肿瘤的疗效.方法 对36例呈浸润性生长的、大或巨大型的垂体腺瘤、脑膜瘤、颅咽管瘤、胶质瘤、皮样囊肿等不同性质的鞍区肿瘤采用经纵裂入路的显微手术进行治疗.结果 在36例中,依据术中显微镜下所见及术后CT或MRI复查结果.29例全切,5例次全切,2例大部切除.无明显术后并发症,无死亡病例.术前视力、视野损害者26例,24例术后当天开始好转,无变化或加重1例.其中有内分泌症状者,术前闭经、泌乳14例,术后泌乳停止13例,1例较术前减少.18例功能性垂体腺瘤患者中接受随访12例,血激素水平恢复正常10例,较术前明显下降2例.2例肿瘤复发,均行放射治疗.证明手术治疗效果良好.结论 纵裂入路是鞍区大型浸润性生长肿瘤手术治疗的重要方式.     

经蝶及双额底入路治疗垂体腺瘤的手术选择

背景与目的：垂体腺瘤手术分为经蝶和经颅入路两种,常根据不同的情况选择合适的手术入路。这与肿瘤的全切除程度和术后疗效密切相关。本研究探讨经单鼻孔-蝶窦及双额底入路治疗垂体腺瘤的手术适应证、显微外科技术及疗效。方法：回顾性分析47例垂体腺瘤手术患者,其中经单鼻孔-蝶窦手术30例。经双侧额底入路手术17例,分析手术经过、治疗效果和随访情况。结果：肿瘤总体全切率89-36％（43／47）,其中经单鼻孔-蝶窦入路组86．67％（26／30）,经双额底人路组94．12％（16／17）。肿瘤未获全切者术后辅以放射治疗。随访6,24个月。4例患者肿瘤复发．接受经双额底入路手术后恢复良好。结论：经单鼻孔-蝶窦入路手术治疗垂体腺瘤创伤小、恢复快,适用于肿瘤大部分位于鞍内或向蝶窦生长的患者。经双额底入路肿瘤全切率高．便于保护垂体柄,适用于肿瘤主要向鞍上及鞍旁生长的大型或巨大型肿瘤．     

经翼点入路显微手术切除鞍区肿瘤66例临床研究

目的探讨经翼点入路显微手术切除鞍区肿瘤的方法及临床效果。方法对2000年1月至2012年2月收治的66例经翼点入路显微手术切除鞍区肿瘤患者进行回顾性分析总结。结果66例患者,肿瘤全切55例,次全切8例,大部切除3例。术后病理检查,垂体瘤38例,颅咽管瘤16例,脑膜瘤12例;术后32例发生尿崩,18例出现低钠血症,3例高钠血症,无手术死亡病例。54例患者随访6个月至5年,术前视力下降45例,术后视力恢复好转38例,无变化5例,恶化2例。肿瘤残留11例,术后行1刀放疗6例,肿瘤复发3例,1例鞍区并发眶内脑膜瘤。结论在经翼点入路行鞍区肿瘤显微手术中,熟练掌握规范的手术入路和局部显微解剖以及良好的显微外科技术是提高肿瘤全切率和手术疗效的保证。     

21例儿童颅咽管瘤的显微手术治疗

目的总结儿童颅咽管瘤的临床特点及显微手术术后并发症的防治方法。方法回顾性分析21例儿童颅咽管瘤的临床表现、手术方法和术后并发症。结果肿瘤全切除13例（61．9％）,次全切除4例（19．1％）,大部分切除4例（19．1％）。术后尿崩症16例（76．1％）,其中2例为长期性尿崩;7例（33．3％）出现下丘脑损伤症状;意识障碍10例（47．6％）;垂体功能低下15例（71．4％）;癫痫2例（9．5％）。全组无死亡病例。随访32．5个月,3例（14．2％）复发。结论显微手术切除肿瘤是儿童颅咽管瘤的有效治疗措施,术前影像学的详细检查,合适手术入路的选择,术后并发症的及时防治有助于患儿预后的改善。     

Pediatric sarcomas and related tumors of the head and neck

Sarcomas of the head and neck region are a rare group of tumors in children and present challenges with regard to evaluation and treatment. Rhabdomyosarcomas are the most common sarcomas of the head and neck in children. Presence of metastases and complete surgical resectability continue to be the most relevant clinical prognostic factors in patients with sarcomas. However, many patients present with unresectable tumors; these require radiation therapy, which is associated with concerns about immediate and long-term side effects. New technologies, including proton beam therapy (PBT), appear very promising in terms of reducing acute and long-term toxic effects. A multi-disciplinary approach is required for best long-term outcomes in children with head and neck sarcomas.     

Hepatocellular carcinoma and liver tumors in South African children: a case for increased prevalence

BACKGROUND: The high regional incidence of hepatocellular carcinoma (HCC) in South Africa also may be present in children of the region, although the link to hepatitis B (HBV) appears less clear. The objective of this study was to assess the incidence and probable causes of HCC in South African children. METHODS: Data were obtained from seven participating pediatric oncology units and from the tumor registry to review hepatic tumors in children in South Africa. RESULTS: One hundred ninety-four children (ages 0-14 years) presented with malignant primary hepatic tumors (1988-2003). One hundred twelve tumors (57%) were hepatoblastoma (HB), 68 tumors (35%) were hepatocellular carcinoma (HCC) (including 9 patients with the fibrolamellar variant, 6 of which occurred in black children), 10 tumors (5%) were sarcoma of the liver, and 4 tumors were lymphoma. The ratio of HB to HCC (1.67) was markedly lower compared with other reports, suggesting a greater prevalence of HCC. Correlation with population statistics indicated an incidence of 1.066 malignant liver tumors per year per 10(6) children age < 14 years (HB, 0.61 per 10(6) children; HCC, 0.39 per 10(6)). Two-thirds of patients with HCC were positive for HBV surface antigen (HBsAg), and HCC occurred mostly in black African patients (93%). The mean age of onset was 1.47 years for HB and 10.48 years for HCC. A preponderance of males (3.5:1.0) was noted in the HBsAg-positive group that was not reflected elsewhere. Serum alpha-fetoprotein (AFP) levels were raised both in patients with HB (100%; most AFP levels were very high) and in patients with HCC (69%), although 15% of patients with HCC had low or normal AFP levels. CONCLUSIONS: It appeared from the current results that HCC is more prevalent among children in South Africa compared with the children in more developed countries, although their rates were lower that the rates noted in adults. A collaborative approach will be required to improve their diagnosis and management.     

Treatment of children with malignant brain tumors]

The current treatment of children with malignant brain tumors is reviewed. Multidisciplinary treatment is essential for malignant gliomas or glioblastoma. The prognosis of children with glioblastoma remains dismal and a new treatment approach needs to be established. Recent aggressive multi-drug chemotherapy in combination with radiotherapy and surgery have markedly improved the treatment of children with medulloblastoma and primary intracranial malignant germ cell tumors. The number of long-term survivors and even cure rates have been increasing.     

翼点入路切除鞍区肿瘤的体会(附119例报告)

背景与目的：鞍区肿瘤较为常见,且近年来采用翼点入路手术逐渐增多,本文结合我们的临床经验,探讨翼点入路切除靶区肿瘤的方法。方法：本组119例鞍区肿瘤,术前根据CT、MRI及脑血管造影,确定肿瘤与周围的关系,经翼点入路手术,采用显微外科技术,经4个不同的间隙切除肿瘤。结果：72例全切,36例近全切,11例大部切除。近期明显好转85例,改善24例,无变化8例,死亡2你,半年以上随访92你,能正常工作（或学习）61例,能做轻工作等15例,生活自理9例,生活不能自理6例,死于心脏病1例。结论：采用翼点入路多能满足靶区不同类型鞍区肿瘤的显露,能提高肿瘤全切率及治愈率。     

Neurofibromatosis and childhood tumors

Although tumors are a known complication of von Recklinghausen's neurofibromatosis (NF), they often develop after the second decade and their characteristics in the pediatric population are unclear. To define the frequency and distribution of tumor types in children with NF, the authors reviewed the experience at Children's Hospital of Pittsburgh between 1953 and 1984. During that time, 121 children younger than 18 years with documented NF were seen. Three patients (2.5%) developed locally extensive, unresectable sarcomas, two of which were clearly neural in origin. Two other children died from complications of highly cellular but not histologically malignant neurofibromas. Seventeen patients (14%) had brain tumors, of which 3 (2.5%) were malignant astrocytomas and 9 were optic gliomas. Two children had acute myelogenous leukemia. Although it has been said that patients with NF are particularly susceptible to the oncogenic effects of radiation, 12 children were treated with a median of 5000 rad to their tumor bed. Follow-up after radiation in that small cohort of patients ranged from 6 months to 12 years (mean, 6 years). Eleven of 12 patients experienced adequate local control. During the follow-up period, none developed a second tumor outside or within the radiation port that could be related to radiation. The authors conclude that clinically significant tumors in children with NF, not all of which are histologically malignant, are not rare. Although more prospective information is needed on response to and sequelae from therapy, the data in this report suggest no contraindication to aggressive therapy.     

Application of three-dimensional computer reconstruction from computerized tomography to intracranial tumors in children

A three-dimensional surface reconstruction algorithm was devised from contour information based upon anatomic structures identified by computed tomographic (CT) scanning. The triangular 'tiling' method from which the algorithm was derived accurately reconstructs the surfaces between contours of both supra- and infra-tentorial anatomic sites. Volume and surface area of the lateral ventricles and tumor site were calculated from the surface reconstruction algorithm in four children with intracranial mass lesions. Reconstruction of three-dimensional surfaces from CT head scans could be utilized to plan the surgical approach to supra-tentorial and posterior fossa lesions. The incorporation of this technique may greatly assist neurosurgeons and neuro-oncologists in planning multi-modality therapy and also for localization of small and relatively inaccessible lesions which may not be adequately assessed by conventional methods. Furthermore, the efficacy of radiation therapy and/or chemotherapy in brain tumors can accurately be assessed.     

Outcomes of children with intermediate-risk neuroblastoma after treatment stratified by MYCN status and tumor cell ploidy.

PURPOSE: The goal of Pediatric Oncology Group 9243 was to improve outcomes for children with intermediate-risk neuroblastoma (NB). PATIENTS AND METHODS: Patients were assigned to treatments on the basis of age, tumor MYCN status, and tumor cell ploidy. Children in the less intensive arm A received cyclophosphamide/doxorubicin and surgery. Patients not in complete remission postoperatively were treated with cisplatin/etoposide, cyclophosphamide/doxorubicin, and additional surgery. Patients with less favorable features were assigned to arm B, which consisted of carboplatin, etoposide, ifosfamide, and surgery. Survival rates were determined using an intent-to-treat approach. RESULTS: For arm-A patients, the 6-year event-free survival (EFS) was 86% with an SE of 3%. For arm-B patients, the 6-year EFS was 46% with an SE of 7%. MYCN status was the only statistically significant prognostic variable. Among patients whose tumors were MYCN nonamplified, a trend toward improved EFS was seen in children with hyperdiploid versus diploid tumors. However, many of these children responded well to salvage therapy, and overall survival rates did not differ on the basis of ploidy. Six-year EFS rates for arm B were patients with MYCN nonamplified, hyperdiploid tumors, 86% with an SE of 3%; patients with MYCN nonamplified, diploid tumors, 74% with an SE of 10%; patients with MYCN-amplified, hyperdiploid tumors, 46% with an SE of 15%; and patients with MYCN-amplified, diploid tumors, 22% with an SE of 10%. CONCLUSION: Outcomes for patients with MYCN-nonamplified, hyperdiploid tumors were excellent. Therapy reductions for these patients merit study. A trend toward less favorable outcomes for patients with MYCN-nonamplified, diploid tumors was observed; more children may need to be evaluated before therapy is reduced for this subgroup. For patients with MYCN-amplified tumors, new strategies are needed.     

Management of adults with Wilms’ tumor: recommendations based on international consensus

Since Wilms’ tumor occurs rarely in adults, there are no standard treatments available. Most adult patients will be diagnosed unexpectedly following nephrectomy for presumed renal cell carcinoma. Outcome for adults is inferior compared with children, although better results are reported when treated within pediatric trials. Multiple factors, including the unfamiliarity of adult oncologists and pathologists with Wilms’ tumors, lack of standardized treatment and consequent delays in initiating the appropriate risk-adapted therapy, may contribute to the poor outcome. A standardized approach for the management of adult Wilms’ tumors is proposed with the aim to limit treatment delay after surgery and encourage a uniform approach for this rare disease and thereby improve survival. These recommendations are based on discussions held with representatives of the renal tumor committees of the Society of Paediatric Oncology and Children’s Oncology Group, and have been updated with a review of more recently published institutional and trial experience of adults treated on pediatric protocols. They provide a critical evaluation of the current evidence for the management of adult Wilms’ tumors and propose details of how current pediatric therapeutic guidelines could be adapted for use in adults.     

Management of adults with Wilms' tumor: recommendations based on international consensus

Since Wilms' tumor occurs rarely in adults, there are no standard treatments available. Most adult patients will be diagnosed unexpectedly following nephrectomy for presumed renal cell carcinoma. Outcome for adults is inferior compared with children, although better results are reported when treated within pediatric trials. Multiple factors, including the unfamiliarity of adult oncologists and pathologists with Wilms' tumors, lack of standardized treatment and consequent delays in initiating the appropriate risk-adapted therapy, may contribute to the poor outcome. A standardized approach for the management of adult Wilms' tumors is proposed with the aim to limit treatment delay after surgery and encourage a uniform approach for this rare disease and thereby improve survival. These recommendations are based on discussions held with representatives of the renal tumor committees of the Society of Paediatric Oncology and Children's Oncology Group, and have been updated with a review of more recently published institutional and trial experience of adults treated on pediatric protocols. They provide a critical evaluation of the current evidence for the management of adult Wilms' tumors and propose details of how current pediatric therapeutic guidelines could be adapted for use in adults.     

Soft tissue sarcomas in infancy and childhood

Thirty-one soft tissue sarcomas in children were studied. The majority of these tumors occurred between the ages of 6 and 10; there were 18 boys and 13 girls. Three main histologic types were encountered, 14 children had fibrosarcoma, 13 rhabdomyosarcoma, 2 had solitary malignant schwannoma, and in 2 the histogenetic origin could not be determined. Head and neck region was found to be the most common anatomical site, and 16 of 31 patients had their primary tumors in this region. In the rhabdomyosarcoma group of the 11 eligible patients, 6 lived free of disease for 5 years; in the fibrosarcoma group of the 12 eligible patients, 6 lived for 5 years or more. Both children with solitary malignant schwannoma are living. It is proposed that rhabdomyosarcoma in children be treated by combining irradiation, chemotherapy, and surgery while fibrosarcoma should be treated by excision alone. Furthermore, it is also proposed that, when the histogenetic type of a tumor can not be determined, these children be treated as having rhabdomyosarcoma.