Kadish C期嗅神经母细胞瘤的综合治疗

目的 探讨Kadish C期嗅神经母细胞瘤合理的治疗方法.方法 回顾性分析6例Kadish C期嗅神经母细胞瘤的临床资料.均采取以手术为主,结合放疗、化疗或同步放化疗等措施的综合治疗.其中3例为初治病例,3例为复发病例.6例术中送冰冻病理,切缘均阴性.结果 5例整块全切除的患者随访20个月以上,无复发;1例分块全切除的患者,术后11个月死于肿瘤复发.并发症包括3例气颅,2例精神症状,1例脑脊液鼻漏.结论 Kadish C期嗅神经母细胞瘤治疗宜选择以手术为主的多学科综合治疗.手术可采用经额下入路,无瘤原则和颅底重建非常重要.     

颅内嗅神经母细胞瘤

目的探讨颅内嗅神经母细胞瘤的诊断及治疗。方法对我科收治的1例颅内嗅神经母细胞瘤患者的临床资料进行分析并进行相关文献复习。结果患者头颅磁共振示前颅底巨大占位病变,分叶状,有囊变,T1WI稍低信号,T2WI稍高信号,增强扫描明显强化,鼻内镜检查未见鼻腔肿物。行双额入路前颅底占位切除术,术中见肿物基底位于大脑镰与筛板连接处。病理结果证实为嗅神经母细胞瘤。结论嗅神经母细胞瘤是一种少见肿瘤,发生于鼻腔内或鼻腔向颅内侵犯,单独发生在颅内者罕见;嗅神经母细胞瘤具有远处转移的特点,手术切除+术后放疗为其首选治疗方案。     

侵袭颅内嗅神经母细胞瘤

目的 探讨侵袭颅内嗅神经母细胞瘤临床特点及治疗方法。方法 对我院神经外科从2001年7月．2005年8月收治的5例侵袭颅内嗅神经母细胞瘤病人的临床表现、影像学特点、病理学特点，手术治疗进行分析，1例行内镜下经鼻活检，4例手术治疗，分别选择经额入路和经额扩展入路，应用不同方法进行颅底重建。全部病人术后行放疗，1例辅助化疗。术后6-45个月进行随访。结果 4例侵袭颅内肿瘤全部切除，术后鼻塞全部改善，3例术前视力下降者术后视力均有一定程度的改善，术后均未发生脑脊液漏，2例术后出现腰骶段椎管内转移，分别为术后6-8个月。其中1例手术，死亡1例。结论 侵袭颅内的嗅神经母细胞瘤治疗仍然需要多手段进行干预，对侵袭到颅内肿瘤要争取全切除，术后应辅以放疗。     

显微镜开颅联合内镜经鼻入路手术切除颅内侵袭性嗅神经母细胞瘤疗效分析

目的 总结颅内侵袭性嗅神经母细胞瘤的临床特点、手术策略和治疗效果.方法 纳入2005年1月至2020年12月在首都医科大学附属北京同仁医院诊断与治疗的24例颅内侵袭性嗅神经母细胞瘤患者,均行双侧扩大经基底入路联合内镜经鼻入路手术+颅底重建术.结果 24例患者肿瘤全切除22例(91.67％)、近全切除2例(8.33％),...     

内镜经鼻颅内外联合入路切除前中颅底沟通瘤

目的 探讨内镜绛鼻颅内外联合入路切除前中颅底沟通瘤的手术方法.方法 对13例患者在扩大经额下入路显微切除肿瘤颅腔内部分的基础上,采用经鼻内镜下切除肿瘤颅外部分并重建颅底缺损.结果 手术全切除11例,次全切除2例,无手术死亡和新神经功能废损、脑脊液漏、脑膜腩膨出及颅内感染等并发症发生.术后病理爪神经鞘瘤4例,鼻咽纤维血管瘤3例,脑膜瘤2例,垂体瘤、内翻性乳头状瘤、鳞状细胞痛和嗅神经母细胞瘤符1例.结论 采用内镜经鼻颅内外联合入路切除前中颅底沟通瘤创伤小且深部视野良好,能安全且完全切除良性肿瘤.     

经扩大的前颅底入路切除鼻、蝶、斜坡肿瘤

目的:颅底中线部位侵袭鼻、蝶、斜坡的肿瘤进行根治性切除时,遗留的颅底缺损修复十分困难。改良扩大的前颅底入路方法可以克服这些困难。方法:扩大的前颅底入路是在双额瓣开颅的基础上,再做一个双侧眶顶和额窦的游离骨瓣,以扩大显露。我科自1993年10月至1995年3月采用该方法对10例鼻、蝶、斜坡肿瘤进行根治性切除,取得良好效果。其中男5例,女5例,平均年龄50(34～63)岁。组织学诊断为脊索瘤4例,嗅神经母细胞瘤2例,垂体瘤1例,脑膜瘤1例,巨细胞瘤1例,鳞癌1例。肿瘤侵入副鼻窦8例、斜坡6例、眶内1例、颅内鞍上或前、中颅窝7例。结果:获肿瘤全切除者7例,近全切除者3例,无手术死亡和严重手术并发症,颅底缺损的修复也无困难。结论:我们采用扩大的前颅底入路的初步手术结果是令人满意的,该入路具有以下优点:①显露良好,不必过高抬起额叶底面即可增加术者在矢状面上的视角30°。②可显露和直视下切除的肿瘤范围广。③颅底重建可靠,基本上可避免脑脊液漏和颅内感染等并发症。     

9例嗅神经母细胞瘤治疗临床分析

目的探讨嗅神经母细胞瘤手术治疗的疗效及放射治疗的意义。方法回顾性分析2001-01—2013-12 9例嗅神经母细胞瘤经手术治疗患者的临床资料,根据Kadish分期,A期1例,B期7例,C期1例,全部患者随访12~84个月。结果经鼻内窥镜进路手术切除A期、B期5例,鼻侧切开进路手术切除B期3例,鼻侧切开加经颅进路手术切除C期1例,术后1例未作放疗,5个月后复发,8例术后加放疗肿瘤无复发。结论对于嗅神经母细胞瘤A期和B期的患者,经鼻内窥镜手术进路和鼻侧切开手术进路的治疗效果相当,鼻内窥镜手术具有术中视野清晰、局部组织损伤轻微、术后手术创面恢复较快等优势,是治疗鼻腔鼻窦嗅神经母细胞瘤的最有效手术方法,术后配合放疗,疗效更显著。     

前颅底嗜酸性肉芽肿1例

1临床资料患者女,5岁。因头痛、左眼视力下降1个月,鼻腔流血10d入院。查体:左眼视力0.6,右眼视力1.0,鼻腔内有血痂CT见前颅底、蝶窦、筛窦略高密度占位性病变,左眶内侧壁颅底骨质破坏。MRI见前颅底、蝶窦、筛窦略长T1、略长T2占位性病变,左眶内侧壁、颅底骨质破坏。嗜酸性粒细胞5%,血沉稍有增快。诊断:前颅底内外沟通瘤,考虑为嗅神经母细胞瘤或嗜酸性肉芽肿的可能性较大。手术取前方颅底入路,硬膜外牵起额叶,可见颅底灰黄色肿瘤。硬膜切一小口,见肿瘤未突破硬膜,严密缝合硬膜。切除颅底肿瘤,肿瘤质地较脆,血运中等。沿肿瘤生长方向将左眶…     

经鼻旁——改良翼点入路切除嗅神经母细胞瘤

目的 通过对本组手术方法和入路的探讨,旨在提高嗅神经母细胞瘤的手术疗效。方法 4例嗅神经母细胞瘤患者均先由耳鼻喉科经鼻旁切口入路切除鼻腔及副鼻窦内肿瘤,接着由神经外科医生经改良翼点入路行开颅肿瘤切除及颅底重建。结果 本组4例无手术死亡,其中肿瘤全切除3例,次全切除1例(副鼻窦内残存)。随访3年,3例存活,1例术后死于急性脑梗塞。结论 神经外科、耳鼻喉科联合经鼻旁—改良冀点入路切除嗅神经母细胞瘤是首选治疗手段,术后辅以放疗有预防复发作用。     

手术切除从颅底侵犯海绵窦的肿瘤

目的探讨手术切除侵犯海绵窦的颅底肿瘤的指征及手术要点。方法自1998年11月至2002年5月，中日友好医院神经外科与中国医学科学院肿瘤医院头颈外科合作，连续切除侵犯海绵窦的颅底肿瘤32例，其中鼻咽纤维血管瘤7例，脊索瘤5例，鼻咽癌和鼻咽囊腺癌5例，副鼻窦癌5例，神经鞘瘤3例，嗅神经母细胞瘤1例，颞下翼腭窝低分化癌2例，颞下翼腭窝肉瘤3例，恶性纤维组织细胞瘤1例。23例曾经1次或多次手术切除后复发。对临床资料进行回顾性总结。结果根据肿瘤主体的部位分别选用经上颌骨入路、颞下耳前入路、或额眶入路。全部病例术中显露满意，肿瘤均得到肉眼切除，受累的颅神经一并切除，无手术死亡，术后无偏瘫等严重并发症。、术后辅以放射治疗。随访3—50个月，平均19个月，失访3例，4例术后3—6个月死亡，4例带瘤生仔，21例健在。结论由下向上侵犯海绵窦的颅底肿瘤可以手术切除，近期效果满意。对颅底正常和病理性解剖结构的熟练掌握以及多学科医生的密切协作是手术取得成功的关键。     

前中颅底沟通瘤的解剖学分类及手术治疗

目的 探讨前中颅底沟通瘤的临床分类方法 及手术治疗,提高临床治疗水平. 方法 根据肿瘤主体位置和生长方向将29例前中颅底沟通瘤患者划分为额鼻眶区(16例)、中颅窝一侧颅底区(8例)、颅底中央区-中间颅底区(4例)及岩骨颈静脉孔区(1例)4类,据此并结合病理资料等分别选择扩大经额下人路(13例)、眶上-翼点入路(9例)、额颞眶颧入路(3例)、额颞人路(3例)及岩骨切除入路(1例)进行肿瘤切除和颅底缺损重建,其中采用经鼻内镜等颅内外联合入路11例.结果 肿瘤全切除24例.次全切除5例,无手术死亡发生;术后早期出现动眼神经麻痹2例,余未有新的神经功能缺损及脑脊液漏、颅内感染、脑膜脑膨出等严重并发症发生. 结论 该分类方法 具有界限清楚、部位和范围明确的优点,有利于选择合理手术人路进行肿瘤切除和颅底缺损修复及临床手术治疗效果的提高.     

颅面联合手术切除前颅底沟通肿瘤的临床疗效分析

目的研究颅面联合手术切除前颅底沟通肿瘤的疗效,为改进手术操作提供依据。方法回顾性分析16例采用颅面联合手术切除并颅底重建的前颅底沟通肿瘤患者的临床资料,对术后早期相关并发症、相关影像学表现、病理结果、术中情况等进行分析总结。结果本组16例患者中肿瘤全切除9例,次全切除4例,部分切除3例。有5例患者术后出现一种或数种相关并发症,其中术后短期颅内积气4例、脑脊液漏3例、短暂性精神异常2例、颅内感染2例、切口感染2例、复视1例、尿崩症1例。以上所有术后并发症,予以对症处理后均有好转。患者的手术远期效果好,无严重术后并发症或后遗症发生。结论颅面联合手术入路方案是颅底区沟通性肿瘤外科治疗的良好手术方式,该术式可有效降低患者术后相关并发症的发生率,改善预后。     

Giant cranionasal schwannoma

Giant schwannomas concomitantly involving the intracranial and nasal cavities are rare. We report a 22-year-old male patient with a giant schwannoma involving the frontal skull base and extending into the nasal cavity. The patient had a 2-year history of nasal obstruction that was originally misdiagnosed as nasal polyps. A CT scan and an MRI revealed a large cranionasal tumor. Surgery was performed using a coronal incision with a bilateral frontal skull base extradural approach. The tumor originated from the anterior skull base and the dura and nasal mucosa were intact. Histopathology was consistent with a schwannoma. Schwannoma should be listed as part of the differential diagnosis of a cranionasal tumor and the surgical approach should be carefully selected to achieve total resection.     

Intranasal myiasis: A rare complication of transnasal skull base surgery

A 57-year-old woman with a history of atypical intracranial meningioma had undergone multiple craniotomies and endoscopic skull base procedures over several years. She presented most recently with nasal discharge consisting of intranasal larvae. Isolated organisms from the nasal cavity and maxillary sinus were identified as blow fly larvae (Calliphoridae family). The patient was treated with transnasal debridement and antibiotic therapy. The organisms were successfully eradicated and she is free from further signs of infection. Intranasal myiasis is an unusual complication of anterior skull base surgery.     

The role of the superior turbinate flap in skull base reconstruction

We describe surgical treatment of a hemangiopericytoma in the anterior skull base and nasal cavity. The patient underwent a novel method of anterior skull base reconstruction, using the superior turbinate and cerebral falx, which was successful. The patient did not develop a cerebrospinal fluid leak or intracranial infection.     

显微手术治疗前颅底沟通肿瘤（附23例报告）

目的探讨前颅底沟通肿瘤的临床特征、手术治疗方法及效果。方法回顾性分析23例前颅底沟通肿瘤病人的临床表现、病理分型、采用前颅底入路显微手术。结果肿瘤全切除19例,次全切除2例,大部分切除2例,术后所有病人症状、体征明显改善。结论采用前颅底入路显微手术治疗可提高前颅底沟通肿瘤的切除率。     

Anterior cranial base glioneuronal heterotopia

Background Cranial base glioneuronal heterotopia is a nest or linear array of glioneuronal tissue within the basal meninges. It is thought to arise from aberrant migration of embryonic neuroepithelial tissues into the subarachnoid space. It frequently mimics tumors and may extend through basal skull bones into extracranial soft tissues.Case report We describe a case of intracranial anterior skull base leptomeningeal glioneuronal heterotopia in a newborn female who was diagnosed in the prenatal period by ultrasound examination to have an intracranial space-occupying lesion at 35 weeks of gestation. There was no evidence of increased intracranial pressure at birth. Magnetic resonance imaging showed a large, predominantly solid, minimally enhancing tumor in the anterior cranial fossa extending into the suprasellar region as well as into the right and left middle fossae. The gross tumor was totally resected through bifrontal craniotomy.Conclusions Although uncommon, diagnosis of intracranial extracerebral glioneuronal heterotopia should be strongly ascertained while evaluating intracranial space-occupying lesions during the prenatal period and infancy. It needs to be distinguished histologically from teratoma and primary central nervous system tumors. Prenatal screening for early diagnosis of the lesion is vital. The location of the lesion and its relationship to the surrounding structures should lead to an accurate diagnosis in the majority of cases. Complete resection is curative and should be the goal. Long-term prognosis is excellent in view of the benign nature and extraaxial origin of the lesion.     

前颅底-筛窦入路切除斜坡区硬膜外肿瘤

目的探讨前颅底-筛窦入路切除斜坡区硬膜外肿瘤的手术方法和技巧。方法对我院1999年11月至2006年7月间收治的10例斜坡区硬膜外脊索瘤病人均采用前颅底-筛窦入路显微手术治疗。结果10例肿瘤均达到镜下基本全切，无手术死亡病例，其中1例术后第7天出现脑脊液漏，重新手术修补成功。术后均无颅内感染及长期脑脊液漏等严重并发症发生，且均恢复良好。结论经前颅底-筛窦入路显微手术切除斜坡区硬膜外肿瘤具有可行性和优越性，对合适的病例可以做到彻底切除肿瘤，取得满意的效果。     

内镜经鼻前颅底肿瘤的外科治疗

目的总结内镜经鼻入路切除前颅底肿瘤的经验。方法回顾性分析2003年11月～2006年5月18例肿瘤侵犯前颅底的临床资料．其中14例单独采用内镜经鼻手术入路．4例采用颅鼻联合入路进行了治疗。病理类型包括：脑膜瘤2例，脊索瘤1例，视神经鞘瘤1例．骨纤维异常增殖症1例．鳞状细胞癌3例，嗅神经母细胞瘤3例，腺样囊性癌1例，恶性骨巨细胞瘤1例，脊索肉瘤1例．神经内分泌小细胞癌1例，透明细胞癌1例，甲状腺癌颅底转移1例，腮腺癌颅底转移1例。结果经术中镜下及术后影像学检查证实17例肿瘤被全部切除．1例大部分切除。2例术后出现脑脊液鼻漏，经保守治疗后痊愈。无颅内出血、感染及死亡病例。结论内镜经鼻入路能够充分显露和切除前颅底肿瘤．且大多数病例无需处理硬脑膜及进行颅底重建。     

Evaluation of outcome and QOL in patients with craniofacial resection for malignant tumors involving the anterior skull base

The purpose of this study was to retrospectively evaluate the effectiveness of anterior craniofacial resection in the treatment of nasal and paranasal malignant tumors involving anterior skull base. Between 1992 and 1998, 13 patients with nasal or paranasal malignant tumors underwent this surgical procedure. The site and time of metastasis or recurrence, and survival outcome were retrospectively surveyed. Current status of long-surviving patients and their subjective assessment of the surgical treatment were also evaluated through questionnaires. Median follow-up period was 52 months. Nine patients (69%) were alive with no evidence of disease. Of these patients, eight had survived for more than three years. Recurrence or metastasis occurred in four patients (31%). The mean time interval between surgery and recurrence or metastasis was 11 months. According to the results of questionnaires to long-surviving patients, 89% patients had some complaints. In particular, complaints of unsightly appearance were manifested by all these patients. When the patients themselves evaluated their current conditions resulting from this surgical treatment, 63% were dissatisfied. These results suggest that this surgical treatment is valid for selected patients in regard to survival outcome. When the effectiveness of this treatment is evaluated, however, psychological and functional issues should not be taken lightly.