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。     

原发性血小板增多症致颅内静脉窦血栓形成一例

正1病例资料患者,女性,24岁,主因"左眼视物模糊1个月"于2015年12月就诊本院神经内科。患者1个月前于劳累、情绪紧张后出现左眼视物模糊,伴左眼眶周痛,偶有恶心,视物模糊逐渐加重,无头痛、呕吐、一过性黑蒙、耳鸣、视物成双、四肢麻木无力等,外院诊断"视神经炎",并给予激素冲击治疗,上述症状一度曾有好转,但激素减量过程中左眼视物模糊再次加重。既往史:体健,否认外伤史和手术史,否认     

Primary Intracranial Leptomeningeal Melanomatosis

Primary intracranial malignant melanoma is a very rare and highly aggressive tumor with poor prognosis. A 66-year-old female patient presented a headache that had been slowly progressing for several months. A large benign pigmented skin lesion was found on her back. A brain MRI showed multiple linear signal changes with branching pattern and strong enhancement in the temporal lobe. The cytological and immunohiostochemical cerebrospinal fluid examination confirmed malignant melanoma. A biopsy confirmed that the pigmented skin lesion on the back and the conjunctiva were benign nevi. We report a case of primary intracranial malignant melanoma and review relevant literatures.     

Primary Carcinoid Tumor of the Skull Base: Case Report and Review of the Literature

We present an unusual case of primary intracranial carcinoid tumor of the skull base centered at the level of the foramen jugulare, which was proven with surgical biopsy and later with Somatostatin receptor nuclear medicine scan. We present the salient magnetic resonance imaging features of this rare tumor, describe their characteristic nuclear medicine findings, and briefly review the literature.     

Atypical Intramuscular Myxoma of the Lumbosacral Paraspinal Muscle: The First Case Report in Asian

Intramuscular myxoma (IM) is a benign neoplasm of mesenchymal origin. We report a rare case of IM which was located in the lumbosacral paraspinal muscles. A 62-year-old female patient presented with progressive low back pain for 2 months, and the radiologic findings showed a large mass (4.0×3.5×6.5 cm) in the right lumbosacral paraspinal area. Total resection of the tumor was performed and the symptom was nearly resolved after surgery. Although the immuno-histopathological analysis was consistent with IM, there were some different findings from typical pathological characteristics of IM in this case. Firstly, the symptomatic change of the mass took relatively short time (less than 3 months), and this change was accompanied by partial calcification inside the mass. Moreover, iatrogenic interruption of paravertebral muscle by the other previous operation might be the promoting factor of the fibrous dysplasia, which can explain the pathogenesis of IM. To our knowledge, this is the eighth case of the lumbar paraspinal myxoma reported in the literatures and the first case in Asian population.     

Rapid Development of Brain Abscess Caused by Streptococcus Pyogenes Following Penetrating Skull Injury via the Ethmoidal Sinus and Lamina Cribrosa

Objective

Streptococcus pyogenes is a beta-hemolytic bacterium that belongs to Lancefield serogroup A, also known as group A streptococci (GAS). There have been five reported case in terms of PubMed-based search but no reported case of brain abscess caused by Streptococcus pyogenes as a result of penetrating skull injury. We present a patient who suffered from penetrating skull injury that resulted in a brain abscess caused by Streptococcus pyogenes.

Methods

The patient was a 12-year-old boy who fell down from his bicycle while cycling and ran into a tree. A wooden stick penetrated his skin below the right lower eyelid and advanced to the cranium. He lost consciousness on the fifth day of the incident and his body temperature was measured as 40℃. While being admitted to our hospital, a cranial computed tomography revealed a frontal cystic mass with a perilesional hypodense zone of edema. There was no capsule formation around the lesion after intravenous contrast injection. Paranasal CT showed a bone defect located between the ethmoidal sinus and lamina cribrosa.

Results

Bifrontal craniotomy was performed. The abscess located at the left frontal lobe was drained and the bone defect was repaired.

Conclusion

Any penetrating lesion showing a connection between the lamina cribrosa and ethmoidal sinus may result in brain abscess caused by Streptococcus pyogenes. These patients should be treated urgently to repair the defect and drain the abscess with appropriate antibiotic therapy started due to the fulminant course of the brain abscess caused by this microorganism.     

海绵窦显微外科相关三角解剖学研究

目的为海绵窦显微外科手术提供解剖学参数。方法在６～２０倍手术显微镜放大下,对２０例成人头颅进行了海绵窦１２个三角解剖学测量。结果测量了上壁４个三角,外侧壁６个三角,后壁２个三角,通过这些三角所显示海绵窦内结构及腔隙的范围。结论应用这些三角解剖学测量可指导与海绵窦相关的手术,安全切开海绵窦上壁、侧壁、后壁,不损伤重要神经和血管     

Intracranial Undifferentiated Sarcoma Arising from a Low-Grade Glioma: A Case Report and Literature Review

Undifferentiated sarcomas are rarely identified in the intracranial region. A 23-year-old man was admitted with a chief complaint of headache. Initial magnetic resonance images showed signs of low-grade glioma in the frontal lobe. Stereotactic biopsy was performed, and a diagnosis of diffuse astrocytoma was confirmed. Three months later, the patient presented with a high-grade tumor as seen on imaging studies. He underwent total resection of the tumor and histopathological tests identified an undifferentiated sarcoma. The patient died eight months later due to massive tumor bleeding. To the best of our knowledge, this is the first report of undifferentiated sarcoma arising from low-grade glioma without any chemotherapy or radiotherapy.     

Chondroblastoma of the apex portion of petrousal bone

Background Skull chondroblastoma is extremely rare. We described in this study the first case of petrousal bone apex chondroblastoma. Methods The tumor occurred in a 12-year-old girl and was revealed by left hypoacusis, vertigo, and walking instability. Computerized tomography scan depicted well-demarcated and osteolytic lesion of the left petrousal bone just before the internal acoustic meatus. The tumor was operated on by a subtemporal approach with a subtotal resection. Lesion recurred and was operated a second time by a combined approach subtemporal and translabyrinthine. This treatment was completed by radiotherapy. We used a combination of photon therapy and proton therapy. Outcome was good and free of recurrence 36 months after the second operation. Conclusion Proton therapy can be particularly useful for skull base tumors frequently radioresistant and near very important structures. This case is the first one of a successful treatment by proton therapy for chondroblastoma recurrence.     

Skull base chondrosarcoma presenting with parenchymal hemorrhage: A case report

Chondrosarcoma (CS) is a locally invasive, slow growing malignant neoplasm. Intracranial chondrosarcoma are extradural osseous lesions and intradural location with intratumoral hemorrhage is very rare. We report a rare case of 65-year-old male presenting with a small mass in left posterior skull base and later, suddenly deteriorating due to large cerebellar hemorrhage. A brief review of literature of such cases is also presented.     

颅底脑膜瘤的治疗进展

瘤基底与前、中、后颅凹底附着的脑膜瘤统称为颅底脑膜瘤,约占脑膜瘤的35%～50%.颅底解剖结构复杂,颅底脑膜瘤常包裹脑神经、基底动脉、颈动脉及其分支,甚至侵袭脑干.     

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.     

Spontaneous Epidural Hematoma from Skull Base Metastasis of Hepatocellular Carcinoma

We report a case of an acute spontaneous epidural hematoma (EDH) due to skull base metastasis in a 46-year-old male patient with hepatocellular carcinoma (HCC). The patient presented with the acute onset of severe headache followed by unconsciousness, and computed tomography showed a large EDH in the right temporal and parietal lobes with midline shift. Emergency evacuation of the EDH was performed, and the hemorrhage was determined to be secondary to skull base metastasis of HCC.     

前入路的斜坡侧壁显微解剖研究与临床应用

目的 为前方经斜坡入路提供斜坡侧壁重要结构的显微解剖参数和显露方法。方法 对20具成人头颅标本，仿Janecka的标准面部移位入路切口(左侧)行两侧斜坡侧壁结构显微解剖观察和测量，并初步探讨颞肌瓣转移重建斜坡硬膜的可行性。结果 上斜坡侧壁主要结构有视神经管隆起和颈内动脉隆起；中斜坡侧壁重要结构有颈内动脉岩骨段、岩下窦和外展神经经过的Dorello管，循翼管向后方可安全确认颈内动脉岩骨段；下斜坡侧壁首要结构为椎动脉和后组颅神经。颞肌瓣转移可有助于斜坡硬膜重建。结论 掌握斜坡侧壁的显微解剖和重要结构的显露方法，有助于经斜坡入路的侧方扩展。颞肌瓣转移重建斜坡硬膜具有可行性。     

上颌骨翻转入路切除颅底沟通肿瘤的应用解剖

目的 探讨经上颌骨翻转入路切除颅内外沟通肿瘤可行性及其显微解部学关系.方法 (1)应用显微外科解剖技术,对经甲醛固定、动静脉血管内分别灌注红、蓝乳胶的10具(20侧)尸头标本,模拟手术入路逐层解剖,观察手术径路中颢下窝、翼腭窝、蝶筛区、海绵窦等区域的显露情况及重要解剖结构之间的关系.(2)经该手术入路切除颅底沟通肿瘤6例,回顾分析其临床资料.结果 翼突、中鼻甲、上颌神经是经上颌骨翻转手术入路的三个重要解剖标志,此入路对前、中颅底、斜坡区显露满意,有充足的手术操作空间,从颅外暴露颅底,对脑组织的牵拉损伤小.临床应用中,6例肿瘤均得到全切,术后反应小,恢复快.结论 上颌骨翻转入路切除颅内外沟通肿瘤手术可行,熟悉此入路的显微解剖学关系,对开展此手术入路切除颅底沟通肿瘤具有指导意义.     

Outcomes and patterns of care in adult skull base chondrosarcomas from the SEER database

This study aims to demonstrate survival rates and treatment patterns among patients with chondrosarcomas of the skull base using a large population database. Patients with skull base chondrosarcomas between 1973 and 2009 were identified from the USA Surveillance, Epidemiology, and End Results (SEER) database. Kaplan–Meier survival analysis was used to examine the effect of surgery and radiation on overall survival. We identified 226 patients with skull base chondrosarcomas. Median follow-up was 5.4 years. Median overall survival was 22 years, and 10 year survival was 68.2%. Most patients underwent surgery (92.5%). Few received radiation after diagnosis (38.1%). Ten year survival for all patients treated with surgery was significantly increased compared to those without surgery (69.3% versus 53.9%, p = 0.02). There was a significant difference in survival amongst treatment groups (p = 0.02), with median overall survival not yet reached for patients who received surgery and radiation (median follow-up 5.3 years), compared to 22 years for non-irradiated surgical patients. Surgery predicted better overall survival by univariate analysis (hazard ratio [HR] 0.420, p = 0.03). Female sex (HR 0.470, p = 0.011), younger age at diagnosis (HR 1.046, p < 0.0001), and later year of diagnosis (HR 0.949, p = 0.0006) were prognostic of improved survival in a multivariate model. In subgroup analysis of patients with documented tumor size, smaller tumor size (HR 1.054, p = 0.0003) and younger age (HR 1.021, p = 0.0067) predicted improved survival. This population based study further reaffirms the role of surgery as an effective treatment for skull base chondrosarcoma as previously reported in small case series. Adjuvant radiation may also confer survival benefit. Optimal treatment strategy has yet to be defined in the literature.     

颅底中央区脑膜瘤的显微外科治疗

目的 探讨颅底中央区脑膜瘤治疗策略和手术的一些原则问题。方法 回顾分析我科自1990年至2002年收治的生长于颅底中央区的脑膜瘤103例。结果 病变切除达到Simpson1、2级病例所占百分比为:鞍结节及鞍隔脑膜瘤94％(16/17),前床突及蝶骨嵴内侧脑膜瘤78％(28/36),岩斜区及蝶岩斜区脑膜瘤68％(26/38),海绵窦脑膜瘤58％(7/12)。死亡率约4％,术后早期严重井发症发生率16％。结论 对于颅底中央区难治性脑膜瘤,采取积极的外科手术态度和小心谨慎的操作,可使2/3或更多的病例获得全切除,而其中2/3以上病人获得良好生存。     

Skull Base Invasion of Adenoid Cystic Carcinoma of the Lacrimal Gland : A Case Report

Although adenoid cystic carcinoma (ACC) of the lacrimal gland is a rarely encountered orbital tumor, it invades intracranially more frequently than carcinomas of other glands in the head and neck. A 52-year-old man underwent orbital exenteration and resection of intracranially extended tumor via a fronto-orbito-zygomatic approach in combination with a transfacial approach. Histopathologically, the tumor showed perineural, vascular, and lymphatic invasion. Additionally, he received radiotherapy (60 Gy) and adjuvant systemic cisplatin and 5-fluorouracil chemotherapy due to residual tumor in the orbit and systemic metastases (lung, ribs, and spines). He was free of progression and recurrence at 6 months after treatment. The authors report a case of skull base invasion by an ACC of the lacrimal gland to remind neurosurgeons planning intervention that this disease shows a tendency to invade intracranially.