第四脑室卵黄囊瘤1例报告并文献复习

目的 探讨颅内卵黄囊瘤的临床特点、影像学特征及治疗方法,提高对颅内卵黄囊瘤(YST)的认识。方法 回顾性分析中国医科大学附属盛京医院神经外科手术治疗的1例经病理证实的第四脑室卵黄囊瘤患儿的临床资料;并对相关文献进行复习。结果 患儿行开颅显微镜下手术全切除肿瘤,术中见肿瘤基底位于小脑蚓部,充满第四脑室,约3 cm×3 cm×4 cm大小,色暗红,供血丰富,质地较软;仔细分离肿瘤周边,逐渐深入,分块完整切除肿瘤。病理检查为卵黄囊瘤。术后患儿恢复良好,随访1个月未见复发,目前仍在随访中。结论 颅内第四脑室卵黄囊瘤临床非常罕见;其病因尚不明确,临床表现与肿瘤大小和部位有关,影像学检查有一定诊断价值,血清及脑脊液甲胎蛋白(alpha fetoprotein,AFP)浓度升高。目前治疗大多采用手术完整切除肿瘤,术后联合放化疗。因第四脑室卵黄囊瘤的恶性程度较高,故预后差。     

颅内非典型畸胎瘤样/横纹肌样瘤1例报告并文献复习

目的探讨颅内非典型畸胎瘤样/横纹肌样瘤(AT/RT)的临床特点,诊断及治疗,提高对其认识水平。方法回顾性分析1例经病理和免疫组化诊断为颅内非典型畸胎瘤样/横纹肌样瘤患者的临床资料,并进行相关文献复习。结果患者主要表现头痛、头晕伴恶心,渐进性加重;MRI示病灶位于右侧岛叶。显微镜下手术切除肿瘤,免疫组化检查证实为颅内非典型畸胎瘤样/横纹肌样瘤。术后患者行放化疗,存活期12个月。结论颅内非典型畸胎瘤样/横纹肌样瘤高度恶性,极具侵袭性,生长迅速,预后极差,临床和影像学表现缺乏特异性;病理组织学改变是确诊的主要依据;采用以手术为主的个体化综合治疗是关键。     

原发性颅内滑膜肉瘤1例报道及文献复习

目的研究原发性颅内滑膜肉瘤的临床表现、病理、治疗与预后的特点。方法回顾性分析1例原发性颅内滑膜肉瘤11岁患儿的临床资料、影像学表现、病理、治疗和预后。结果 CT检查示右颞枕大片混杂密度影,肿瘤大小为3.5 cm×3.0 cm×2.0 cm。接受右颞枕叶肿瘤根治性切除术,术后病理示镜下小圆核瘤细胞呈片巢状或围绕血管密集排列,细胞异型,可见核分裂相,部分区域见胶原纤维增生,部分区域黏液变性;免疫组化染色示CK(部分+)、Vim(部分+)、Syn(+)、EMA(-/+)、CK7(-)、CK19(+)、INI-1(+)、AFP(-)、Oct-4(-)、Des(-),GFAP(-),CD99(-),CgA(-),PLAP(-),NF(-)、CD117(-/+),MIB-1 10%,诊断为(右颞枕叶)间叶组织来源的恶性肿瘤——滑膜肉瘤。术后在当地医院行放射治疗,总剂量为50 Gy,共25次。术后11个月复查头颅MRI见肿瘤复发累及右侧小脑半球。结论原发性颅内滑膜肉瘤是一种非常罕见的恶性肿瘤,目前采用手术切除肿瘤结合术后放、化疗的方案,但预后不良。     

颅盖和颅底动脉瘤样骨囊肿

动脉瘤样骨囊肿好发于管状骨的干骺端,位于颅底者极少见。作者从文献中收集到44例,其中多数位于颅盖骨,累及颅底者仅3例。多数学者认为从病理上确定为本病并不难,根治性切除亦易,但位于颅底者诊断和治疗均较困难。本文报导颅盖及颅底动脉瘤样骨囊肿共8例。例一,2个月婴儿,出生24小时后发现右顶部有一包块隆起,迅速发展,入院时已达4cm直径。     

颅底外科常用手术入路的临床应用

目的:探讨和分析颅底外科常用手术入路在临床应用的初步经验,以改善位于颅底部病变的治疗效果。方法:回顾分析7年间手术治疗的颅底病变175例,其中颅底肿瘤154例,颅内动脉瘤15例,其它病变6例。采用9种手术入路,包括扩大前颅底、额下经眶、眶颧、眶颧-颞极、岩骨联合、远外侧枕下、口-咽、颈-颌下和颞下窝入路。结果:颅内肿瘤154例中,全切除116例(75.3％),与入路有关并发症7例(4.5％),死亡2例(1.3％)。15例动脉瘤中,术后13例恢复良好。结论:选择恰当的入路具有扩大显露范围、减少脑组织牵拉、便于修复颅底等优点,可明显提高全切除率,减少术后并发症和降低手术死亡率。     

颅内海绵状血管瘤(附15例报告)

目的探讨颅内海绵状血管瘤的临床表现、影像学特征、诊断及治疗。方法对1993-2004年我院收治的15例海绵状血管瘤进行回顾性分析。结果15例均行手术治疗,病理确诊为海绵状血管瘤。其中位于脑叶内6例,病灶均全切除;位于脑干3 例,2例全切除,1例部分切除;位于海绵窦内3例,2例部分切除,1例仅取活检,行栓塞术;位于颅底3例,2例全切除,1例部分切除。术前头颅CT和MR增强扫描提示肿瘤呈均一性显著强化,边缘清晰。其中12例行MR检查,9例考虑为海绵状血管瘤;15例行CT检查,显示为占位性病变。手术无1例死亡,疗效较满意。结论海绵状血管瘤术前诊断主要靠影像学检查,但位于颅底和海绵窦常误诊为脑膜瘤;手术是主要的治疗方法,能否全切除与病变位置有直接关系。     

脑内深部病变术中彩色多普勒超声定位的临床研究(附42例分析)

目的探讨彩色多普勒超声定位技术在脑内深部病变手术中的应用价值。方法回顾性分析42例脑深部病变的临床资料。手术切除肿瘤前均行硬膜外或硬膜下彩超检查,以定位肿瘤及选择手术入路。手术切除肿瘤后再行彩超检查以评估手术效果。结果手术前后影像学资料对比显示42例病变均定位准确。其中显微镜下全切除20例,次全切除14例,部分切除8例。术后4例出现神经功能缺损加重,3例术前偏瘫短期内加重,其余病人神经功能保留良好。结论术中彩超有助于病变的术中定位和手术路径的选择,准确地切除病灶,最大程度减少手术的副损伤。     

婴幼儿颅内非典型畸胎样/横纹肌样瘤的诊治体会(附四例报告)

总结4例婴幼儿颅内非典型畸胎样/横纹肌样瘤的诊疗经验。4例患儿中,男3例,女1例;年龄为9~34个月。肿瘤均为颅内巨大占位,最大径>5 cm;其中位于小脑幕上左侧颞岛叶1例,小脑幕下3例。肿瘤全切除3例,活组织检查术1例,术后均经病理确诊。4例患儿的随访时间为3~12个月,术后化疗1例,该患者目前仍存活但肿瘤复发;余3例拒绝化疗,于术后3~4个月死亡。婴幼儿颅内非典型畸胎样/横纹肌样瘤是相对罕见的高度恶性肿瘤,总体预后很差,手术加化疗是主要的治疗方法。     

多部位神经纤维瘤病Ⅱ型1例报告

患者男性,24岁,头痛、头晕伴左耳听力下降半年于2006年7月5日第一次入院,实验室检查阴性,皮肤无咖啡斑,神经系统检查阴性,家族无类似疾病史,头颅MRI示：左中颅窝底占位。同月份全麻下行“左颞顶开颅肿瘤切除术”,术中见肿瘤呈灰白色,质地韧,边界清楚,血供一般,显微镜下尽可能完全切除肿瘤,行病理检查证实为神经纤维瘤。2011年7月18日患者左侧肢体麻木第二次入院,MRI示左中颅窝底海绵窦旁片状强化灶（图1）,胸椎椎管内多发占位,于同月份全麻下分别行“左颞顶开颅肿瘤切除术”、“胸3～4、胸11～腰1椎管内多发肿瘤切除术”,颅内肿瘤术中所见同前述,椎管内肿瘤肉眼呈灰白色,质韧,边界清楚,血供一般,大小不一,最大3 cm×2 cm×2cm,最小米粒到芝麻大小,散在分布,与脊神经联系密切者未强行切除,其余瘤体显微镜下完全切除,术后病理证实均符合神经纤维瘤。     

颅骨硬化性上皮样纤维肉瘤1例报告并文献复习

目的 探讨硬化性上皮样纤维肉瘤(SEF)的临床特点、影像学表现、病理诊断及治疗方法。方法 回顾分析1例颅骨SEF患者的临床资料,并复习相关文献。结果 本例男性患者,40岁,临床表现为头部持续增大包块;头颅CT检查示,左侧顶骨近椭圆形溶骨性病变。行手术切除,术中见肿瘤位于左侧顶枕部,固定于颅骨深面,边界尚清,质韧,向下生长压迫脑组织,相邻硬脑膜有增厚,手术全切病灶和受累硬脑膜。术后病理检查诊断为硬化性上皮样纤维肉瘤。术后随访15个月未见病灶复发。结论 颅骨硬化性上皮样纤维肉瘤较为罕见,易转移、复发,影像学检查误诊率高,病理检查具有重要诊断作用,手术全切病灶,未行放化疗,未见复发迹象。     

A case of calvarial aneurysmal bone cyst: transcranial contrast sonographic examination with pulse inversion harmonic imaging method]

A 31-year-old female came to our hospital complaining of left frontal bulging with pain on 10 August 2000. The head x-p showed a radiolucent lesion and bulging at the same calvarial site. CT scan and MRI showed fluid-fluid levels, diploic cyst, deformity and hypertrophic calvarial change. There was a partial hypervascular part of cyst adjacent to the left frontal base by selective left external carotid angiography. Harmonic image is a contrast specific imaging modality that uses the nonlinear properties of contrast agents by transmitting at the fundamental frequency and receiving at multiples of these frequencies. Pulse inversion harmonic image(PIHI) using pulse inversion to eliminate and strengthen the harmonic frequency is more effective than conventional harmonic imaging. Transcranial sonographic examination showed hyper- and hypoechoic appearances in the cyst around abnormal hyperechoic appearances of the calvarial site. The transcranial contrast sonographic images with PIHI (hereinafter TCIpi) demonstrated an enhanced intracystic lower stratum and nearby the diploic part. That modality facilitates better visualization than the harmonic imaging method, enabling differentiation of vascular from avascular areas. As contrast agents are microbubbles, those are restricted in the vascular canal space. So TCIpi findings proved to be a blood circulation of the tumor, cyst and neighboring tissue. Enhanced areas changed by every minute and disappeared gradually. The findings were probably based on the vascular component of the tumor. We obtained images similar to those with CT and MRI. The patient underwent on 21 August. The tumor was removed along with the surrounding skull component. Fresh and old blood were mixed in the cyst. The clinical and pathological diagnosis was aneurysmal bone cyst. The postoperative course was uneventful and she was discharged on 29 August. Aneurysmal bone cyst is a rare calvarial tumor. An diagnostic finding is fluid-fluid levels that appear in approximately 30% of aneurysmal bone cysts. However, this is not a specific finding and has also been reported to occur in osteosarcoma, malignant fibrous histiocytoma, fibrous dysplasia, synovial sarcoma, hemangioma and simple bone cyst. Therefore, diagnosis of aneurysmal bone cyst is based on a combination of the various imaging applications, clinical and pathological findings. Gometz reported that sonographic examination was superior to any diagnostic imaging studies for aneurysmal bone cyst. Furthermore, the perfusion examination like TCIpi can directly observe blood circulation channels in tissue, so the specific enhancement changes of aneurysmal bone cyst could be observed. TCIpi is a useful method for diagnosis of aneurysmal bone cyst.     

Aneurysmal bone cyst concomitant with fibrous dysplasia in the frontal bone

Secondary aneurysmal bone cyst in fibrous dysplasia is exceedingly rare, especially in the skull and particularly in the frontal bone. We present a case of aneurysmal bone cyst concomitant with fibrous dysplasia in the frontal bone in a 15-year-old male patient presenting with headache and euphoria with an uncharacteristic imaging appearance and treated successfully by total resection.     

A huge occipital osteoblastoma accompanied with aneurysmal bone cyst in the posterior cranial fossa

Osteoblastoma is an infrequent benign tumor and skull involvement is extremely rare. The occipital bone is much less frequently involved. We report an unusual case of a huge occipital osteoblastoma with aneurysmal bone cyst in the posterior cranial fossa of a 20-year-old young man. MRI scan and CT three-dimensional reconstruction revealed that the tumor (approximately 8.5 cm x 6 cm x 5 cm) occupied nearly half volume of the posterior cranial fossa. The cerebellum, the forth ventricle and brain stem were compressed remarkably and hydrocephalus was noted. The tumor mass was multiloculated cystic and highly vascular. Excessive bleeding occurred during operation and total removal was achieved. The patient was symptom-free and MRI scan revealed no evidence of recrudescence after a follow-up period of 18 months. Pathological examination revealed that the lesion was a benign osteoblastoma with secondary aneurysmal bone cyst (ABC). ABC constitutes a highly vascular lesion while complete surgical resection is often impeded by excessive intraoperative bleeding. Preoperative embolization may reduce the risk. Despite the benign nature of osteoblastoma, local recurrence after incomplete resection is not rare and malignant transformation during recurrence may occur even after adequate surgery. Our case confirms the findings of previous investigators that if osteoblastoma is removed completely, no recurrence is expected even without adjuvant irradiation and chemotherapy.     

颅前窝内外沟通性肿瘤切除术后的颅底重建

目的总结手术切除颅前窝内外沟通性肿瘤后颅底重建的方法。方法回顾性分析21例颅底肿瘤切除术后的颅底重建经验。对颅底小缺损（〈1.0cm×1.5cm）,行颞肌筋膜修补7例;对颅骨大缺损（≥1.0cm×1.5cm）,采用“四步法”行颅底重建：第一步取自体脂肪填塞肿瘤切除后的残腔;第二步用带血管蒂骨膜瓣覆盖颅底骨缺损,边缘严密缝合于周围硬脑膜;第三步用自体骨或钛板等颅骨修补材料修补骨缺损;第四步取自体筋膜修补硬脑膜;本组14例。结果颅底缺损修补均获得满意效果。术后脑脊液漏1例,经腰穿置管引流后痊愈。术后3个月出现局部感染1例,经抗炎治疗后缓解。无局部脑膨出、脑膜炎等并发症发生。结论“四步法”重建颅底简单易行,能有效预防脑脊液漏、局部脑膨出和脑膜炎等并发症的发生。     

Aneurysmal bone cyst of the orbit : a case report with literature review

Aneurysmal bone cyst (ABC) is benign vascular lesion destructing the cortical bone by the expansion of the vascular channel in the diploic space that usually involve long bone and spine. Orbital ABC is rare and the clinical symptoms deteriorate rapidly after initial slow-progression period for a few months. A 12-year-old female patient visited ophthalmologist due to proptosis and upward gaze limitation of the right eye, and orbital mass was noted in the upper part of right eye on orbital MRI. Five months later, exophthalmos was worsened rapidly with other features of ophthalmoplegia. Orbital mass was enlarged on MRI with intracranial extension. Surgery was done through frontal craniotomy and intracranial portion of the tumor was removed. Destructed orbital roof and mass in the orbit was also removed, and surrounding bone which was suspected to have lesion was resected as much as possible. Histopathological diagnosis was aneurysmal bone cyst. Postoperative course was satisfactory and the patient's eye symptoms improved. Authors report a rare case of orbital ABC with review of the literature. Exact diagnosis by imaging studies is important and it is recommended to perform surgical resection before rapid-progressing period and to resect the mass completely to prevent recurrence.     

易误诊的化生型脑膜瘤

目的 化生型脑膜瘤临床少见,对其临床表现和组织病理学特征进行探讨,并复习相关文献,避免误诊.方法与结果 女性患者,17岁,彝族.临床表现为突发性头痛、呕吐、发热伴四肢抽搐.头部CT显示右侧额顶叶不规则低密度影,边界欠清晰,其内含类钙化高密度影,大小约2 cm×1.80 cm×1.70 cm;MRI可见右侧额顶叶大小约2.40 cm×2.10 cm×2 cm的肿瘤影,周围脑组织大片状水肿.术中于右侧额顶叶脑实质内见一占位性病变,一侧紧邻软脑膜,未侵及硬脑膜和颅骨,形状不规则,边界较清晰,表面血供丰富,呈黄色、质地较硬,大小约3 cm×2.50 cm×2 cm.光学显微镜观察肿瘤组织广泛骨化和钙化,肿瘤细胞呈片状和小巢状分布在网状排列的小梁状骨组织间;肿瘤细胞表达上皮膜抗原、孕激素受体、波形蛋白和Bcl-2,Ki-67抗原标记指数约为6％.结论 化生型脑膜瘤的化生成分多种多样,其影像学也存在多样性,通过组织形态学和免疫组织化学可明确诊断,并与其化生成分相似的肿瘤进行鉴别,以免误诊.     

Giant epidermoid cyst of the skull with extra and intracranial extension. A case report

Intradiploic epidermoid cyst of the skull is a rare clinical entity that can exceptionally grow to a large size with intracranial extension. The authors report the case of a 38-year-old man with a giant epidermoid cyst of the parietal bone with extra and intracranial extension, presenting with focal neurological symptoms. The diagnosis was suggested at imaging (skull radiographs, CT and MRI), and confirmed at histology. Complete removal of the cyst and its capsule was performed followed by cranioplasty. Postoperatively, the patient was discharged free of symptoms. CT scan provides good evaluation of the bony lesion and may suggest intracranial extension. MRI is superior for evaluation of cerebral compression. The pathogenesis, clinical presentation, diagnostic evaluation and therapeutic management of these rare lesions are reviewed.     

带蒂帽状腱膜下层骨膜瓣修复前颅底缺损的临床研究

目的 探讨带蒂帽状腱膜下层骨膜瓣在前颅底缺损修复中的治疗作用.方法 额发际内冠状切开头皮和帽状腱膜,紧贴帽状腱膜深面锐性分离,按缺损分型和范围设计瓣膜大小,将帽状腱膜下疏松结缔组织层和颅骨外膜合为一层从颅骨表面剥离,制成带蒂帽状腱膜下层骨膜瓣,用于修补15例外伤及26例肿瘤术后前颅底缺损患者.结果 本组患者缺损面积2.0 cm × 1.5 cm～6.5 cm ×4.0 cm,其中6例缺损＞4.0 cm×3.0 cm患者使用钛网板修复颅底骨缺损.所有外伤患者术后与颅底缺损有关的症状消失,两组均无颅内感染、搏动性突眼、额纹消失、上睑肌无力及额部头皮麻木和坏死等并发症发生,无围手术期死亡.术后两组各有1例发生脑脊液鼻漏,均经腰椎穿刺置管引流7 d内消失.结论 带蒂帽状腱膜下层骨膜瓣制备简单,对外伤或肿瘤术后2.0 cm×1.5 cm～4.0 cm×3.0 cm的前颅底缺损修补效果良好,是一种修补可靠、取材方便的前颅底缺损修补材料.     

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。     

Ollier disease

Background  Ollier disease is a rare, nonfamilial disorder characterized by multiple enchondromatosis with an asymmetric distribution and areas of dysplastic cartilage. Clinical manifestations usually start with local pain, bone swelling, and palpable bony masses, often associated with bone deformity. Intracranial enchondromas arise from the skull base because of its cartilage embryological derivation. Headache and cranial nerve palsy are the most prominent clinical findings. The only effective treatment is represented by surgery that allows to resect the tumors and to treat their complications, such as pathological fractures, growth defect, and neurological symptoms. Method  We report on a 12-year-old boy affected with Ollier disease and operated on for a skull base enchondroma. The tumor was partially removed through a right pterional approach to the cavernous sinus. Serial postoperative magnetic resonance imaging showed an increasing residual tumor and the patient underwent radiation therapy. Conclusion  Surgery remains the main therapy for intracranial enchondromas. Radiotherapy is required in case of sarcomatous evolution or when a gross total tumor resection is not feasible, as in the present case.