儿童颅内未成熟畸胎瘤的诊断及治疗(附36例报告)

目的 探讨儿童颅内未成熟畸胎瘤的诊断及治疗.方法 2002年1月至2010年12月北京天坛医院神经外科收治40例颅内未成熟畸胎瘤患者,随访36例.对其临床症状、体征、血液中肿瘤标志物及影像学检查的结果等进行分析判断,明确未成熟畸胎瘤诊断后,根据肿瘤体积及患者的临床症状采取不同的治疗方式.结果 36例均开颅手术,病理明确诊断为未成熟畸胎瘤.26例肿瘤直径＞3 cm的患者根据临床表现及影像特点分为A、B二组.A组10例,临床症状较轻,影像表现周围浸润不明显,先行手术切除肿瘤,再行放化疗.B组16例,临床症状较重,先行化疗,待瘤体缩小,再手术,术后再化疗放疗.另10例瘤体＜3 cm的患者均先手术,然后化疗、放疗.结论 颅内未成熟畸胎瘤应先化疗,待瘤体缩小并周围浸润局限后再行手术治疗,术后再化疗、放疗可明显延长患者的生存时间.     

原发颅内恶性畸胎瘤一例

目的探讨原发颅内恶性畸胎瘤的临床特点及诊断治疗方案,提高对该疾病的认识。方法报告1例颅内三脑室恶性畸胎瘤病例临床资料,结合文献进行分析。结果患者行手术切除,病理为恶性畸胎瘤,术后行全中枢照射+瘤床推量放疗,放疗后全身化疗4个周期。治疗后随访56个月,未出现肿瘤复发及脊髓内或远处转移。结论颅内恶性畸胎瘤为罕见颅内肿瘤,肿瘤切除术后辅以放疗、化疗是安全、有效的治疗措施之一。     

儿童颅内未成熟畸胎瘤(附30例报告)

目的 探讨儿童颅内未成熟畸胎瘤的临床特征、治疗策略和预后.方法 回顾性分析30例经手术及病理证实的未成熟畸胎瘤患儿的临床资料,26例获得随访.结果 19例术前行血和(或)脑脊液肿瘤标记物(AFP,B-HCG)检测,12例阳性.初次手术肿瘤全切15例,近全切7例,大部切除6例,部分切除2例.随访患儿中,23例行辅助治疗.患儿半年、1年及2年的生存率分别为80%、69%、60%,患儿的存活时间与肿瘤的切除程度筹异有统计学意义(P<0.05).结论 肿瘤标记物的检测有助于诊断.手术是首选的治疗措施,应尽量做到全切.术后应辅以放、化疗.     

延髓成熟畸胎瘤1例并文献复习

目的 探讨延髓畸胎瘤临床特征及诊疗经验。方法 回顾性分析首都医科大学宣武医院2021年1月收治的1例延髓畸胎瘤患者的临床资料,总结其临床表现、影像学特征和治疗方法,并对相关文献进行总结。结果 该患者延髓占位,经枕下后正中入路手术治疗后,病理诊断为成熟型畸胎瘤。术后未出现严重并发症及其他神经系统异常。术后核磁共振成像(MRI)示肿瘤全切,症状消失。结论 计算机断层扫描、MRI及肿瘤标志物联合病理可确诊延髓畸胎瘤,首选手术切除。     

颅内海绵状血管瘤43例临床分析

一、临床资料１一般资料：男１９例,女２４例,年龄５～６３岁。单纯头痛１３例,癫痫１７例,出血偏瘫４例,颅神经障碍６例,头晕９例。额颞叶２７例,中颅窝及鞍旁９例,基底节３例,小脑半球２例,脑干和侧脑室各１例。２影像检查：ＣＴ检查３５例,平扫半球小片状混杂密度,边界清,无占位及水肿,增强示均匀强化的１５例诊为ＣＡ,８例中颅窝鞍旁边界清晰的高密度占位病变,均匀强化诊为脑膜瘤;４例片状低密度,轻微强化,略有占位表现的诊为胶质瘤,２例诊断为脑梗塞,５例出血者诊断为血肿,正常１例。ＭＲ检查３４例,２５例…     

含成熟畸胎瘤的松果体区肿瘤病理分析及诊治

目的探讨如何全面诊断和恰当治疗包含成熟畸胎瘤的松果体区肿瘤。方法对14例含成熟畸胎瘤的松果体区肿瘤的临床资料、诊治过程、病理构成进行总结分析。结果单纯成熟畸胎瘤9例,成熟畸胎瘤+生殖细胞瘤4例,成熟畸胎瘤+绒毛膜癌1例。8例手术前曾接受过不同形式的放疗。术后均恢复良好,但2例在鞍上出现新的肿瘤,考虑为种植转移。结论含成熟畸胎瘤的松果体区肿瘤诊断及鉴别诊断困难,手术是首选治疗方法,术前放疗应慎用,术后处理应规范。     

松果体区成熟畸胎瘤的临床特点及治疗分析

采用经枕部小脑幕入路手术治疗9例松果体区成熟畸胎瘤患者,无一例围手术期死亡,术后均经病理检查明确诊断。术后4例发生手术相关性并发症(双眼上视困难、视野缺损、癫发作)。术后共随访3个月至7年,平均3.70年。松果体区成熟畸胎瘤多见于男性儿童,以颅内高压和共济失调为主要临床表现,神经外科手术治疗可获得满意疗效。     

小儿骶尾部巨大成熟型畸胎瘤的手术治疗（附1例报告）

我院1998年3月收治1例小儿骶尾部巨大畸胎瘤,经手术治疗后痊愈出院,结合文献,对小儿骶尾部巨大畸胎瘤的治疗探讨如下:1 临床资料 患儿女,8个月,出生时骶尾部即有10cm×8cm×5cm大小的肿块,当时未处理,现肿块逐渐长大至19crn×12cm×8cm,小儿发育正常,智力正常,饮食、睡眠、大小便均正常。查体见肿块实质感,局部皮肤血管增生。X片示肿块内有钙化影,密度不均。穿刺肿瘤呈实性,无囊液。在全麻下行骶尾部巨大肿瘤全切术,术中见肿瘤包膜完整,界限清楚,蒂与尾骨尖相连,予以末节尾骨切除,肿瘤完整切除,重890克。病理检查:骶尾部成熟畸胎瘤,由分化成熟的纤维脂肪、肌肉、血管、软骨、淋巴、消化道粘膜及脑组织混杂而成。伤口愈合良好,痊愈出院。2 讨论 小儿骶尾部成熟良性巨大畸胎瘤并不多见,为先天性,对小儿的生长发育无影响。但随着年龄的增长而增大,会影     

化、放疗联合治疗儿童颅内生殖细胞瘤34例临床随诊观察

目的：观察颅内生殖细胞瘤经过化疗并辅以中低剂量放疗后的疗效及对儿童生长发育的影响。方法：自1993年12月至2001年12月共对63例颅内生殖细胞瘤的儿童进行静脉化疗并辅以中低剂量放疗，随访到34例，其中肿瘤位于松果体区20你，松果体区并鞍区6例，鞍区4例，松果体区并脑室内3例，底节区1例。使用药物为长春新碱、甲氨蝶呤、平阳霉素及顺铂。用药后每周化验2次血常规，1次血生化，以观察周围血象变化及心、肝、肾功能情况，并进行针对性治疗。4周后如血常规及血生化的各项指标均正常，再行第二疗程化疗。2个疗程结束后1个月，在肿瘤病灶局部补充中低剂量（25-35Gy）的放疗。每个疗程间进行CT或MRI检查，观察肿瘤消退的情况。：34例患者随访最短1年，最长7年。1例为手术加全剂量放疗后2年肿瘤复发，此次化疗进行2个疗程，但化疗后未再补充放疗，4年后肿瘤复发，家属放弃治疗，患者死亡。1例化、放疗后5年肿瘤复发，再行2疗程化疗，疗效较首次治疗差，其余32例（94％）化、放疗后1-7年，复查MRI显示肿瘤无复发，患者生存质量良好，儿童生长发育及智力均未受影响。结论：静脉化疗辅以中低剂量放疗应为治疗儿童颅内生殖细胞瘤的最佳方案。     

颅内血管网织细胞瘤的临床特点及治疗

目的 探讨颅内血管网织细胞瘤的临床特点及治疗方法。方法 对７２例颅内血管网织细胞瘤的临床资料进行回顾性分析,并结俣献对其临床、病结合献对其临床、病理及影像学特点等问题进行了讨论。结果 ７２例颅内血管网织细胞瘤占同期颅内肿瘤的１．５７％,６４例位于幕于,占８９％,囊性肿瘤５２例,占７２％。７２例均手术治疗,５８例均行手术治疗,５８例治愈,９例好转,５例死亡。结论 本病临床特点为男性多于妇性,多发     

替莫唑胺应用于恶性脑胶质瘤的研究进展

脑胶质瘤是临床最常见的肿瘤之一,约占颅内肿瘤发病率的40％～50％,由于胶质瘤呈恶性浸润性生长,且多生长在脑重要结构,如基底节、中央沟区、丘脑、脑干等部位,不仅手术难以全切,而且术后易复发。长期以来,单纯手术治疗恶性脑胶质瘤的5年存活率〈25％。但术后合理的放疗、化疗和其他综合治疗可以控制肿瘤生长和延缓复发,使一些病人病情得到较好的改善。化疗作为颅内恶性胶质瘤综合治疗手段之一,已为广大临床医生所接受。替莫唑胺作为一种新型的烷化剂,对恶性脑胶质瘤辅助治疗效果良好。本文就替莫唑胺应用于恶性脑胶质瘤的发展近况作一综述。     

恶性胶质瘤术后放化疗效果及影响因素分析

目的分析恶性胶质瘤术后放、化疗的效果与影响因素。方法以我院收治的60例恶性胶质瘤患者为研究对象,所有患者接受手术治疗,并于术后进行放、化疗,分析影响放疗与化疗效果的各项因素。结果 7例完全缓解,36例部分缓解,总缓解率为71.67%(43/60);随访发现复发23例(38.33%),死亡27例(45.00%),存活33例(55.00%)。结论恶性胶质瘤手术疗效受术前癫痫发作、肿瘤病理分级、肿瘤全切因素影响,于术后采用放疗结合替莫唑胺进行化疗,效果良好,值得临床推广。     

Surgical treatment of huge congenital extracranial immature teratoma: a case report

Congenital cranial teratomas are usually characterized by complete loss of the normal intracranial architecture. In the majority of reports, the tumors are associated with stillbirth, perinatal death, or significant morbidity after surgical resection. The few reported attempts at total or subtotal tumor resection have had poor outcomes, although there are rare reports of prolonged survival up to 3.5 years following resection of smaller tumors. Neonatal teratomas are rarely located in the scalp. In the literature, there were only a few patients who underwent surgery during the neonatal period with a good outcome; however, all such patients survived. In this paper, we present a neonatal case of huge congenital extracranial immature teratoma on the scalp extending to the orbita, ears, and brain. Examination of the patient revealed a large craniofacial mass and head circumference that was bigger than normal; there were no other neurological deficits. Computed tomography (CT) and magnetic resonance imaging (MRI) revealed a multiloculated, heterogeneous cystic mass that was larger than the patient’s head, displacing and distorting anatomical structures. MRI showed mass with calcifications, soft tissue, fat, and fluid components. There was deformity and remodeling of the adjacent calvaria. A total surgical excision was performed and histopathological examination showed immature teratoma. The patient’s early postoperative course was uneventful. Postoperative CT and MRI were normal. To date, the patient has survived for 6 months without neurological deficit. We conclude that acceptable functional outcomes in the context of massive congenital craniofacial teratomas can be achieved by early radical resection.     

海绵窦畸胎瘤的诊断与手术治疗

目的探讨海绵窦畸胎瘤的临床诊断和治疗方法。方法回顾性分析3例海绵窦畸胎瘤的临床资料。均采用翼点硬膜下人路显微神经外科手术切除肿瘤，其中2例为海绵窦内畸胎瘤，l例为海绵窦外侧壁畸胎瘤。结果肿瘤均全切除，术后病理检查示均为成熟性畸胎瘤，无手术死亡。l例术前自发破裂的海绵窦内畸胎瘤术后加行放疗。随访6个月～3年肿瘤均无复发。结论海绵窦肿瘤术前影像学评估非常重要，对于良性海绵窦畸胎瘤，积极的显微手术切除一般可获得满意的治疗效果。     

三维适形放疗联合替莫唑胺化疗对术后脑胶质瘤的疗效观察

目的分析比较三维适形放疗联合替莫唑胺化疗和单纯放疗对脑胶质瘤部分切除术后患者的疗效和安全性。方法选取自2003年10月至2006年6月42例脑胶质瘤部分切除术后患者，随机分为2组：单纯放疗组（19例）仅行三维适形放疗，分次局部照射，2．0Gy／次，1次／d，5d／周，共持续6周，总剂量60Gy；联合治疗组（23例）于三维适形放疗同时联合替莫唑胺化疗6周＋辅助化疗6疗程。影像学动态观察患者肿瘤体积的变化及Karnofsky评分评价神经功能状态。结果放疗结束24周后，联合治疗组在影像学所示肿瘤实体的缩小和神经功能状态（日常生活能力）的改善方面均优于单纯放疗组（P〈0．05）。结论放疗联合替莫唑胺化疗可提高脑胶质瘤部分切除术后肿瘤缓解率和患者生活质量。     

Well‐formed cerebellum and brainstem‐like structures in a mature ovarian teratoma: Neuropathological observations

In the surgical case of a mature cystic teratoma of the ovary that arose in a 16‐year‐old girl, a large amount of well‐differentiated and highly organized cerebellar tissue was found. Three layers of the cerebellar cortex were well formed, and synaptophysin‐positive “glomeruli” were found in the granule cell layer. Some Purkinje cells exhibited focal expansion and a dysmorphic appearance of the dendrites. Adjacent to the cerebellar tissue, a large space lined by the ependymal layer and a club‐shaped CNS tissue mass resembling the brainstem were found, and structures reminiscent of the midbrain tectum and pontine nuclei were distinguished within this mass. The CNS tissue was surrounded by the leptomeninges and a skull‐like, bony shell. Structures consistent with the supra‐tentorial CNS tissue were not found. This case represents an example of infra‐tentorial CNS tissue that was well‐differentiated and organized to an exceptionally high degree in an ovarian mature teratoma. Various degenerative changes have been documented in CNS tissue in ovarian teratomas, but the dendritic abnormalities of Purkinje cells seen in the present case are novel findings.     

Intracranial teratoma in children: The role of chromosome 21 trisomy

Teratomas are very rare intracranial tumors and cytogenetic information on this group remains rare. We report a case of a mature teratoma with abnormal +21 trisomy in tumor karyotype ocurring in a non‐Down syndrome (DS) infant. Additionally, the evidence for the contribution of chromosome 21 trisomy in this neoplasia are briefly reviewed. The 6‐month‐old male baby presented with a posterior fossa tumor. Histological evaluation of tumor specimen showed a mature teratoma composed of fully differentiated ectodermal, mesodermal and endodermal components. Although somatic karyotyping of the index case was normal, composite tumor karyotype depicted 47, XY, +21[6]/46,XY[6]. Besides previous reports of children with DS and intracranial teratomas, this is the first report to describe the occurrence of an isolated chromosome 21 trisomy within the tumor of a non‐DS child. The participation of chromosome 21 in this rare pediatric tumor, either somatic or restricted to tumor specimen, may deserve special interest and further investigation.