儿童幕上室管膜瘤

１９８２～１９９３年收治１９例儿童幕上室管膜瘤。其中，男１２例，女７例，年龄９个月～１４岁。幕上室管膜瘤少见，大多位于脑实质内，病程比幕下室管膜瘤长。ＣＴ扫描显示较高的钙比率及囊变率。该病术后复发率高，本组为２１．１％（４例）。术后放疗可用效延缓或防止肿瘤复发，预防性脊髓放射并非常规。肿瘤复发是影响预后的主要因素。     

儿童颅内室管膜瘤临床特点及预后因素分析

目的探讨儿童颅内室管膜瘤的临床特点、诊疗分析及其预后影响因素。方法回顾性分析2012年1月至2020年12月首都医科大学附属北京儿童医院神经外科收治的32例儿童颅内室管膜瘤患儿的临床资料。男23例, 女9例;诊断中位年龄为4.5岁(0.7～10.0岁)。其中<3岁者12例(37.5%), ≥3岁者20例(62.5%)。肿瘤位于幕上14例, 幕下18例。患儿均行肿瘤切除术, 术后放疗18例, 术后化疗20例, 未行放疗及化疗4例。采用Kaplan-Meier法绘制颅内室管膜瘤患儿的生存曲线;采用Log-rank检验单一变量差异是否有统计学意义。结果 32例患儿中, 30例为全切除, 2例为未全切除。平均随访时间为24个月(1～62个月), 至随访截止存活26例, 死亡6例。1年生存率为88%, 3年为生存率为78%。单因素结果分析提示肿瘤部位(OR=1.5, 95%CI:1.082～2.079, P=0.040)对于术后生存时间差异有统计学意义, 是否进行化疗(OR=0.5, 95%CI:0.125～1.999, P=0.034)对于年龄<3岁患儿术后生存时间差异有统计学意义...     

儿童后颅窝肿瘤的诊治特点

目的对儿童后颅窝肿瘤的临床特征、病理类型、手术切除及术后并发症进行分析。方法回顾分析我院儿童(15岁以下)后颅窝肿瘤30例，术前头颅CT及MRI确定肿瘤位置分别采用咬骨窗开颅(21例)和骨瓣开颅(9例)显微镜下切除肿瘤。结果全切除肿瘤20例，近全切除肿瘤10例。无手术死亡。术后病理证实：星形细胞瘤9例，髓母细胞瘤14例，室管膜瘤4例，恶性室管膜瘤3例。结论MRI能清楚地显示肿瘤的大小、部位、范围和界限，根据肿瘤不同部位选择最佳的手术入路，显微镜下暴露肿瘤最直视的部位分离肿瘤，可安全地全切除或近全切除肿瘤。     

儿童脑室管膜瘤15例诊治分析

目的 评价肿瘤放疗对儿童脑室管膜瘤的治疗效果和预后因素。方法 2000年至2005年,15例儿童脑室管膜瘤接受术后放疗,男10例,女5例;幕上5例,幕下10例;手术完全切除8例,不完全切除7例;全颅全脊髓放疗5例,全颅加局部放疗4例,局部放疗6例。结果 5年生存率为66.7%,死亡5例,局部复发3例,脑脊膜转移2例,5例死亡患儿肿瘤皆位于幕下。年龄、肿瘤位置、病理、是否行全颅全脊髓放疗、性别、手术切除情况对生存率无影响（P均 〉 0.05）。结论 肿瘤位于幕下似乎比幕上预后差,幕下脑室管膜瘤是否进行全颅全脊髓全中枢系统的放疗,应根据预后因素来确定。     

显微外科手术治疗第四脑室肿瘤42例

目的探讨显微外科手术治疗第四脑室肿瘤患儿的疗效。方法第四脑室肿瘤患儿42例,均在显微镜下采用后颅窝枕下正中入路切除肿瘤。肿瘤全切除28例,次全切除14例。结果成活40例,死亡2例。术后缄默症6例,脑积水15例,11例行侧脑室-腹腔分流术。随访30例,生活、学习良好;2例室管膜瘤术后1年肿瘤原位复发,行γ刀治疗;4例术后脑积水放弃治疗,自动出院后0.5年死亡。结论及时诊断、适当的手术方法、精准的显微外科手术、积极处理术后脑积水是提高第四脑室肿瘤疗效的关键。     

小儿囊性颅内肿瘤同位素内放射治疗:附18例报告

小儿脑肿瘤是常见的危及儿童健康的肿瘤,临床上治疗以手术切除为主,但对于部分以囊性为主、位于垂体-下丘脑要害部位肿瘤手术难以达到全切,术后易导致垂体功能低下和内分泌紊乱[1,2],以及对于脑肿瘤术后复发难以承受第2次大手术者,以往常采用放疗、化疗,总的疗效不甚满意更带来严图15岁男孩子,右额叶室管膜瘤3级图2术后9月,肿瘤切除部位囊性复发图3内放疗后囊肿消失重并发症,针对上述患儿的治疗,我科于2000年1月至2005年4月采用同位素胶体32P注入瘤腔行微创性的姑息放射治疗,取得满意近期疗效,现报告如下。临床资料一、一般资料18例患儿,年…     

肾母细胞瘤手术与肿瘤局部复发的关系

目的 探讨肾母细胞瘤手术后肿瘤局部复发与手术的关系。方法 1994年1月-1999年12月经手术病理证实肾母细胞瘤35例，年龄11个月-9岁。术后按照国际肾母细胞瘤研究组织（MWTS）Ⅲ-V期放化疗方案治疗并随访。结果 肿瘤复发7例（I期1例，Ⅱ期2例，Ⅲ期4例），复发发生在术后2个月-3年。术中肿瘤破溃6例中4例局部复发；未作淋巴结活检2例局部复发；间变型肾母细胞瘤3例复发。结论 术中肿瘤破溃则复发率高，对巨大肾母细胞瘤术中应仔细操作，避免破溃。必须作腹主动脉旁及肾门淋巴细胞结病理检查，明确临床分期，针对性性放化疗，是提高患儿存活率的重要手段。     

儿童马蹄肾合并肾母细胞瘤的诊治分析

目的探讨儿童马蹄肾合并肾母细胞瘤合理的诊治方法。方法回顾性分析2007～2018年我院收治7例马蹄肾合并肾母细胞瘤患儿的临床资料。其中男3例,女4例,就诊年龄1岁至8岁8个月,平均4岁6个月。肿瘤位于左侧4例,右侧2例,峡部1例。主要临床表现:腹痛4例,无症状腹部包块1例,偶然超声检查发现肿物1例,外院手术并放化疗后肿瘤复发1例。4例(其中2例因瘤体破裂,1例因瘤体巨大且合并肺部转移,1例因瘤体巨大)术前化疗后行患侧肾脏切除及峡部肿瘤切除术,术后辅以放疗+化疗;2例肿瘤位于肾脏一极行肿瘤剜除术,术后辅以化疗;外院术后及放化疗后肿瘤复发1例于我院行复发肿瘤切除术+盆腔转移灶切除。结果 5例获随访(含2例肾肿瘤剜除术)6个月至11年,平均44个月,均未发现肿瘤复发,行肿瘤剜除者肾脏功能良好。2例失访,包括外院术后及放化疗后肿瘤复发1例,术前肺转移1例。结论马蹄肾合并肾母细胞瘤较少见,根据肿瘤位置及大小选择个体化治疗方式,如肿瘤位于肾脏一极可行保留肾脏的肿瘤剜除术,术后按病理分型分期辅以化疗和/或放疗。     

儿童双侧肾母细胞瘤手术后复发的再治疗

目的总结双侧肾母细胞瘤手术治疗后复发的临床特点及治疗经验, 以提高双侧肾母细胞瘤的总体治疗水平。方法回顾性分析首都医科大学附属北京儿童医院泌尿外科2008年1月至2020年12月收治的10例双侧肾母细胞瘤复发患儿临床资料, 包括患儿初次手术情况、复发时间及部位、后续治疗方法及随访情况等。结果 10例中男7例, 女3例, 发病年龄10～69个月, 平均27.3个月。初次手术的20侧中, 18侧采取保留肾单位的肿瘤剜除术;1侧因肿瘤位于肾门处, 保留肾脏困难而行瘤肾根治性切除术;1侧因肾内占位为多发小瘤灶而未行手术。术后病理均为预后良好型, 未见间变型。复发时间为术后2～36个月。1例为双侧复发, 同时有腹膜后淋巴结转移, 未再行手术, 以化疗为主, 后转移至纵隔, 最终死亡。一侧复发的9例患儿中, 1例予单纯化疗9个月, 肿瘤消失;8例予再次手术, 仍采用保留肾单位的肿瘤剜除术;5例无再次复发;3例二次复发患儿中, 1例术后再次出现原位复发合并肺转移, 再次行瘤肾切除术及肺叶楔形切除术, 无瘤生存至今, 其余2例因多处转移而最终死亡。术后均予化疗, 化疗药物包括长春新碱、阿霉素、环磷酰胺...     

儿童脊髓内肿瘤的显微外科治疗

目的总结儿童髓内肿瘤的病理特点、显微手术和治疗效果，并进行探讨。方法81例儿童脊髓内肿瘤均经手术切除和病理证实，对手术治疗的效果进行回顾性分析。结果儿童髓内肿瘤以星形细胞瘤24例（29．6％）、室管膜瘤13例（16．0％）和表皮样囊肿11例（13．6％）为多见，不同的病理类型的肿瘤，切除率不同。放疗用于高度恶性的肿瘤。椎板复位术可以有效地减少脊柱畸形的发生率。结论大多数的髓内肿瘤组织学上为低级别星形细胞瘤（Ⅰ～Ⅱ级）、室管膜瘤和胚胎残余组织肿瘤，积极手术切除能够获得满意的疗效。     

Astrocytoma in Childhood: Survival and Performance

Seventy-nine children (35 female, 44 male) with proven or presumed astrocytoma were treated from 1967 to 1987. The tumors were supratentorial located in 24 children, cerebellar in 21 children, and pontine in 34 children.

If possible, a radical tumor resection (4%), a subtotal tumor resection (51%), or a biopsy (8%) was performed. The predominant pathological Kernohan grading for the supratentorial, cerebellar, and pontine located tumors were grades II, II, and IV respectively (Table 1). Histology was unknown in 15 out of 34 pontine tumors and in 1 out of 24 supratentorial tumors.

Low-graded tumors (46%) were irradiated with a local field (1.8/45-50 Gy) and children with high-graded tumors (34%) received a total brain irradiation (1.8/40 Gy) followed by a boost irradiation (10 Gy) in 5 or 6 fractions.

Overall 1-, 5-, and 10-year survivals of children with supratentorial, cerebellar, and pontine located tumors were 96%-91%-46%, 95%-95%-95%, and 35%-20%-20% respectively. For all tumor locations, 77% of deaths occurred within 2 years of treatment. The performance status of both children with supratentorial and cerebellar astrocytoma showed an increase during the first year of treatment and then stabilized on a rather high level (mean performance after 5 years of 60% and 70% respectively). Children with pontine tumors showed a steep decrease in performance status during the first year of treatment and then stabilized on a low level (mean performance after 5 years of 15%). In our study, children with supratentorial astrocytoma showed improvement in both survival and performance status after irradiation following surgical removal of the tumor. The children with cerebellar astrocytoma showed a relatively high survival and performance status after a combined treatment of surgery and radiotherapy. Neither survival nor performance status seemed to be improved by irradiation whatever the surgical treatment of pontine tumors.     

Brain tumors in children under 3 years of age. Recent experience (1987-1997) in 39 patients.

Brain tumors in children under 3 years of age differ in clinical presentation and pathological behavior from those in older patients. In this study, we reviewed data from 39 children (24 males and 15 females) under 3 years of age who were treated for intracranial brain tumors since the introduction of magnetic resonance imaging. The purpose was to assess correlations between clinicoradiological and treatment-related factors, and their impact on prognosis. The following factors were analyzed: sex, age, duration of symptoms, intracranial hypertension on admission, tumor location, surgical removal and histology. Associations between these factors and correlations with prognosis were determined using bivariate analyses (chi(2) test) and Kaplan-Meier survival curves. Collins' concept of a period of risk recurrence was tested. Mean follow-up was 41 months (range 0-136). In March 1998, 20 children were still alive (51.2%) with a mean survival time of 65 months (range 2-136). The incidence of supratentorial tumors was significantly higher in children less than 1 year old (p = 0.027). Lateral tumors were 9/10 (90%) supratentorial versus only 7/26 (27%) midline tumors (p = 0.001). Outcome (dead or alive) was significantly better (p = 0.037) for low-grade astrocytomas (9/12 = 75% survival) when compared to ependymomas (2/6 = 33%) and primitive neuroectodermal tumors (3/12 = 25%). Total tumor removal was achieved in 20 cases and was associated with a better outcome (65 vs. 33% survival; p = 0.049). Survival analysis confirmed a worse prognosis for children with ependymomas and primitive neuroectodermal tumors (p = 0.011) and revealed a worse survival for children with intracranial hypertension on admission (p = 0.047). Total tumor removal was associated with a longer survival, although not significantly (p = 0. 077). Finally, we found no exceptions to Collins' law.     

Mediastinal T-cell lymphoma in a boy 7 years after treatment of supratentorial primitive neuroectodermal tumor

Medulloblastoma/primitive neuroectodermal tumor (PNET) is one of the most common central nervous system tumors in children and requires aggressive multimodality therapy. The authors describe a patient who developed mediastinal T-cell lymphoma 7 years after treatment of supratentorial PNET. Despite a good tumor response, the patient died during induction therapy because of invasive pulmonary aspergillosis. The authors conclude from this case that patients treated for medulloblastoma/PNET should be followed carefully for years. In addition, patients with second malignancies should be considered as a high-risk population for opportunistic infections.     

Collins' law revisited: can we reliably predict the time to recurrence in common pediatric tumors?

Collins' law states that the period of risk for tumor recurrence is the age of the child at diagnosis plus 9 months. The purpose of this study is to validate this rule through a retrospective review of common pediatric tumors seen at 1 institution. Inclusion criteria for this study included an age at diagnosis of < 16 years old, minimum follow-up time of the Collins risk period (CRP) if child did not relapse and treatment with curative intent. The records of 424 children seen and treated for neuroblastoma (n = 98), Wilms tumor (n = 86), rhabdomyosarcoma (n = 82), medulloblastoma (n = 59), Ewing sarcoma (n = 43), ependymoma (n = 25), supratentorial PNET (n = 22), and synovial sarcoma (n = 9) from 1960 to 2001 were reviewed. CRP was calculated using the age of child at initial diagnosis plus 9 months. The median follow-up time was 164 months (range, 11-484 months), while the median follow-up/CRP ratio was 4.89 (range, 1.0-48.0). A total of 183 of 424 (43.2%) patients relapsed, with 180 (98.4%) relapses occurring during the CRP. Relapses beyond the CRP were seen in 3 young children (ages 7 months, 24 months, and 2 weeks at initial diagnosis) with a diagnosis of Wilms tumor (n = 2) and supratentorial PNET (n = 1) at 1, 3, and 26 months post-CRP. Collins' law is a useful and simple way of predicting risk period for relapse in the tumor types studied.     

小儿非骨化性纤维瘤的临床特点及诊治

目的：探讨小儿非骨化性纤维瘤的临床特点及治疗方法。方法：回顾性分析我院收治的小儿非骨化性纤维瘤20例。其中男12例，女8例，年龄1．5～14岁，平均8．5岁。病灶均在下肢长管状骨，其中股骨远端10例，股骨颈1例，胫骨近端7例，远端1例，腓骨1例。全部病例均经手术治疗，术式包括单纯病灶切除、病灶切除加自体髂骨或加异体骨移植术。结果：全部病例均经病理证实为非骨化性纤维瘤。术后随访1～5年，平均3．2年，无一例复发。结论：非骨化性纤维瘤的病因和发病机制不清。根据其临床特点，典型的X线片表现，诊断并不困难。但应与骨纤维异样增殖症、骨囊肿及骨巨细胞瘤相鉴别。因本病可引起疼痛及病理性骨折，确诊后应早期手术治疗。术式以病灶切除植骨术为主。     

手术治疗31例小儿颅咽管瘤临床分析

目的：为了提高对儿童颅咽管瘤的诊断率以及减少术后并发症的发生,该研究探讨小儿颅咽管瘤的诊断方法、手术方式及术后并发症的防治。方法：回顾性分析31例小儿颅咽管瘤的临床表现、手术方法和术后并发症治疗的经验。结果：31例患儿主要临床表现为：头痛,视力下降,生长发育迟缓等。CT和MRI可确诊颅咽管瘤。31例颅咽管瘤中,肿瘤全切除 19例（61.3%）,次全切除5例（16.1%）,大部分切除7例（22.6%）。术后出现尿崩症者19例（61.3%）,3例为长期性尿崩。6例（19.4 %）出现下丘脑损伤症状。无死亡病例。随访32.5个月, 5例（16.1%）患儿复发。结论：小儿颅咽管瘤的诊断主要依据临床表现、CT和MRI检查,该病以开颅手术治疗为首选。肿瘤切除的程度应考虑降低肿瘤的复发和减少并发症的发生为原则。［中国当代儿科杂志,2009,11（8）：663-665］     

Management of localized thoracic neuroblastoma

Thirteen children with localized (Evans stage I or II) thoracic primary neuroblastoma were divided into two groups according to the type of therapy administered, in order to compare the therapeutic efficacy and morbidity of excisional surgery followed by either irradiation alone or irradiation plus chemotherapy (group A) with similar surgery alone (group B). Group A consisted of 6 children (mean age 1 year, 2 months). Complete surgical excision was accomplished in 2 patients, while 4 had microscopic residual. All 6 patients are free of disease at 26--76 months (mean 47 months), including 2 who had recurrent tumor and received additional therapy. Two have developed congestive heart failure and one severe scoliosis secondary to irradiation. Of the 7 children in group B (mean age 2 years, 2 months), 3 had microscopic residual tumor and 2 had adjacent lymph node involvement. After 12--47 months (mean 23 months), no recurrence or surgery-related morbidity has been observed. From these limited data it appears that surgery alone may provide adequate therapy for localized thoracic neuroblastoma and obviate the morbidity associated with multimodal therapy.     

Childhood tumors of the brain: demographic pattern over a ten-year period in the Kashmir Valley

Brain tumors in children represent the second most frequent tumors in this age group after hematologic malignancies. We highlight the demographic pattern after retrospective analysis of brain tumors in children from geographically and ethnically distinct Kashmir Valley managed in our center between 2000 and 2009. We had a total of 248 pediatric patients with brain tumors. The parameters analyzed were age, gender, location of tumors and histopathological subtypes as well as WHO grade of tumor. We also did a comparison between the frequencies of common varieties of tumor in the first and second 5-year periods. We found that 111 tumors (44.75%) were supratentorial, and 137 (55.25%) were infratentorial. The male-to-female ratio was 1.4:1. The proportions of low-grade and high-grade tumors were 60 and 40%, respectively. The most common tumor in our series was astrocytoma. The most common tumors in the supratentorial and infratentorial compartments were craniopharyngioma and medulloblastoma, respectively. Our experience reflects a different demographic profile of pediatric brain tumors as compared with other regions of the world.     

Supratentorial embryonal tumors in children under 5 years of age: an SFOP study of treatment with postoperative chemotherapy alone

BACKGROUND: To determine the effectiveness of multiagent chemotherapy as sole post-operative treatment of supratentorial central nervous system (CNS) embryonal tumors in young children. PROCEDURE: The data of 25 children under 5 years of age diagnosed with supratentorial embryonal tumors (17 primitive neuroectodermal tumors, four pinealoblastomas, and four medulloepitheliomas) treated exclusively by postoperative chemotherapy (CT) between 1990 and 1997 were reviewed. RESULTS: Fifteen tumors were hemispheric and 10 were deeply seated. Four children presented with disseminated leptomeningeal disease. Total resection was performed in nine patients, subtotal in 9, partial in 3, and a diagnostic biopsy only in 2. Two children did not undergo surgery. Twenty-four children relapsed with a median time of 5.5 months. The median overall survival was 12 months, and the 2-, and 5- year survivals were 30 and 14%, respectively. The 2- year disease-free survival was 4%. There was a significantly worse prognosis in patients undergoing incomplete resection and in the group with deeply situated tumors. Four relapses were treated by second surgery followed by high-dose chemotherapy and radiotherapy. Two of them remain in CR2, and all these children are free of late sequelae. CONCLUSIONS: CT alone failed to maintain disease-free survival in most of the children, although, disease progression was delayed to some extent. Children under 5 years with supratentorial embryonal tumors should undergo total surgical resection if possible.