颅内黑色素细胞瘤的诊疗

目的探讨颅内黑色素瘤的诊治方法。方法回顾性分析7例颅内黑色素瘤病人的临床资料,分别采用额颞、颞枕、顶枕、远外侧或后正中入路行肿瘤切除术。结果肿瘤全切除5例,大部分切除2例。病理证实均为黑色素瘤。本组无手术死亡病例,术后均无新的神经功能缺失。术后行放化疗3例,放疗2例。随访3~18个月,存活3例,死亡3例,均死于术后复发或转移。失访1例。结论颅内黑色素瘤发病率低,误诊率较高,预后差。治疗以手术切除为主,辅以放疗或伽玛刀治疗。     

中枢神经系统原发性黑色素细胞肿瘤临床病理学特征

目的总结中枢神经系统原发性黑色素细胞肿瘤(简称黑色素细胞肿瘤)的临床、影像学和病理学特征,提高对疾病的诊断与鉴别诊断能力。方法与结果纳入2012年1月至2019年10月在广东三九脑科医院诊断与治疗的7例黑色素细胞肿瘤患者,5例手术切除,2例行组织活检术;MRI表现为T1WI高信号,T2WI低信号,FLAIR成像高信号;组织学形态由卵圆形或多边形黑色素细胞构成,胞核偏位,核仁嗜酸性,胞质内含数量不等的色素细胞,细胞异型性不等;免疫组化染色,Melan-A、S-100蛋白呈阳性,HMB-45呈阴性、散在阳性或强阳性。最终诊断为黑色素细胞瘤4例,黑色素瘤1例,弥漫性黑色素细胞增生症1例,黑色素瘤病1例。3例黑色素细胞瘤术后辅以放疗,其中2例分别随访56和116个月状态良好,1例失访;1例未行放疗的黑色素细胞瘤患者总生存期为20个月;1例黑色素瘤患者术后辅以放化疗,总生存期为15个月;1例弥漫性黑色素细胞增生症患者总生存期为13个月;1例黑色素瘤病患者经放化疗后7个月状态良好。结论中枢神经系统原发性黑色素细胞肿瘤罕见,黑色素细胞瘤常见于中老年人,黑色素细胞增生症好发于儿童。肿瘤细胞恒定表达Melan-A和S-100蛋白,而HMB-45表达模式有助于鉴别肿瘤良恶性。肿瘤预后尚不清楚,黑色素细胞瘤预后最好,放疗可获益。     

中枢神经系统恶性黑色素瘤的诊断与治疗

目的 探讨中枢神经系统恶性黑色素瘤的临床表现、影像学特点及诊治要点.方法 分析17例中枢神经系统恶性黑色素瘤的临床表现、影像学特点、诊治方法和疗效.结果 17例中13例位于幕上,幕下小脑半球3例,颈髓1例.其临床表现及体征无特异性,GT和MRI无法明确诊断.手术全切除12例,近全切除5例.病理检查均证实为恶性黑色素瘤.随访14例,均死于术后肿瘤复发,最短于术后1个月死亡,最长于术后14个月死亡,术后中位生存期6.5个月.结论 中枢神经系统恶性黑色素瘤发病率低,误诊率高,预后差,以手术切除为主,可辅以化疗、放疗等综合性治疗.     

黑色素性神经鞘瘤2例报道

色素性神经鞘瘤起源于Schwann细胞,是一种起源于神经嵴且具有双向分化的原始细胞肿瘤,属于良性惰性肿瘤,肿瘤可以产生黑色素临床容易误诊为恶性黑色素瘤。临床治疗上主要以手术切除为主,残留肿瘤需要放疗,并需要术后长期的随访。本文分析总结2例中枢神经系统色素性神经鞘瘤,对该疾病流行病学、病理学、治疗方法进行分析。     

中枢神经系统黑色素细胞肿瘤的临床和病理学特征分析

目的探讨中枢神经系统黑色素细胞肿瘤的临床和病理学特征。方法回顾性分析2018年6月至2021年11月首都医科大学附属北京地坛医院神经外科收治的6例中枢神经系统黑色素细胞肿瘤患者的临床资料。收集患者的一般资料、既往病史、临床表现、影像学资料及术后病理学结果等。采用门诊、电话及影像学随访, 随访内容包括术后辅助治疗情况、症状改善情况及有无肿瘤复发、转移等。结果临床表现为头痛4例、下肢疼痛及麻木1例、面部疼痛1例、记忆力下降1例。影像学检查显示6例均为单发肿瘤, 肿瘤位于L2椎体水平椎管内1例, 颅内5例。肿瘤达全切除4例, 次全切除1例, 部分切除1例。病理学诊断为脑膜和脊膜黑色素细胞瘤各1例(影像学分型为黑色素型), 原发性黑色素瘤2例(影像学分型为非黑色素型), 转移性黑色素瘤2例(影像学分型为血肿型)。HE染色结果显示, 6例肿瘤组织均可见不同含量的黑色素颗粒。免疫组织化学染色结果显示, 抗黑色素瘤特异性单抗(HMB-45)(+)6例, S-100(+)4例, 黑色素细胞抗原(Melan-A)(+)6例, 波形蛋白(VIM)(+)占比为3/3, 上皮膜抗原(-)占比为3/3。2例黑色...     

中枢神经系统原发性黑色素肿瘤的临床特征及病理特征分析

目的探讨中枢神经系统原发性黑色素肿瘤的临床表现、病理特征及手术效果。方法回顾性分析2003年3月至2017年2月手术治疗的3例中枢神经系统原发性黑色素性肿瘤的临床资料,1例位于颞叶,1例位于小脑,1例位于右侧桥小脑角区。结果 2例全切,1例次全切。术后随访1~3个月,3例一般情况良好。结论中枢神经系统原发性黑色素肿瘤确诊依赖病理检查,临床分型对判断预后有重要意义。     

脑膜黑色素瘤病五例临床、脑脊液及影像学特征

目的探讨脑膜黑色素瘤病的临床、脑脊液以及影像学特征。方法回顾性分析5例经病理诊断的脑膜黑色素瘤病患者的临床表现、影像学特征及腰穿检查结果。结果5例患者临床上以颅内压增高、脑膜刺激征、脑神经损害以及脊神经根性疼痛为主要表现。1例有左眼视乳头上方视网膜隆起伴色素沉着，4例皮肤有先天性色痣，其中2例为先天性巨大色痣。5例腰穿压力均≥180mm H2O（1mmH2O=0．0098kPa），3例为血性或淡黄色脑脊液，4例蛋白增高，3例葡萄糖降低。2例头颅MRI增强检查显示软脑膜异常强化。病理学检查：3例腰穿可见恶性黑色素瘤细胞；1例腋窝淋巴结活体组织检查可见恶性黑色素细胞浸润；1例尸体解剖可见脑底部软脑膜和蛛网膜下腔呈弥散性黑色素瘤浸润。2例诊断为转移性脑膜黑色素瘤病，3例可能为原发性脑膜黑色素瘤病。结论脑膜黑色素瘤病临床上表现为脑脊膜、神经根受累，常合并存在皮肤黑色素痣，腰穿检查常提示蛛网膜下腔出血，腰穿检查脑脊液发现黑色素瘤细胞能够确定诊断。     

手术治疗颈静脉孔区脑膜瘤(附8例报告)

目的 探讨颈静脉孔区脑膜瘤手术治疗方法及预后.方法 我科1999至2006年共手术治疗颈静脉孔区肿瘤115例,发现8例脑膜瘤.本文分析其临床、病理及影像学特征与预后的关系.结果 4例肿瘤达到显微外科手术伞切除.8例颈静脉孔区脑膜瘤3例为恶性.平均随访80.8个月,4例内皮型脑膜瘤仍然存活.2例(1例乳头型,1例间变型)患者术后短期死亡.2例(1例微囊型,1例间变型)患者分别存活3年和6年,最后死于病情恶化.结论 良性颈静脉孔区脑膜瘤应尽量追求手术全切除,其术后各种神经功能废损的发生率较此医其他良性肿瘸高.而且此区的脑膜瘤多为恶性.     

手术治疗颅内黑色素瘤的临床疗效观察

颅内黑色素瘤较为罕见。本文回顾性分析9例颅内黑色素瘤患者的临床资料。8例行手术切除肿瘤,其中全切除5例,次全切除2例,部分切除1例;1例仅行活组织检查术。术后病理学证实6例为转移性颅内黑色素瘤,3例为原发性颅内黑色素瘤。无因手术死亡和发生严重并发症者。术后获随访7例,6例术后3~18个月死亡,1例原发性颅内黑色素细胞瘤患者术后36个月死亡,平均生存期为6.8个月。     

中枢神经系统脑脊膜黑色素细胞瘤三例及文献复习

目的 探讨中枢神经系统脑脊膜黑色素细胞瘤(PMM)临床及病理学特征.方法 结合文献复习,回顾性分析3例中枢神经系统原发的脑脊膜黑色素细胞瘤.结果 2例肿瘤位于椎管内,1例位于小脑脑桥角.肿瘤外观呈黑色,部分肿瘤包膜完整.光镜下见瘤细胞由上皮样细胞和梭形细胞混合而成,细胞无明显异型,核仁明显.大部分瘤细胞质内含有大量黑色素颗粒.例1、例3肿瘤核分裂像无或偶见(0～1个/10 HPF),例2核分裂像较多(2～3个/10 HPF).免疫表型HMB45、MelanA和S-100蛋白呈阳性表达.例1、例2肿瘤完全切除,例3部分切除,随访均无复发.结论 原发性脑脊膜黑色素细胞瘤是一种罕见的交界性或低度恶性肿瘤,具有一定的病理学特征,可局部复发或发生恶变,需要与原发或转移的恶性黑色素瘤、黑色素性脑膜瘤及黑色素性神经鞘瘤进行鉴别.     

Large primary meningeal melanoma in an adult patient with neurocutaneous melanosis

Neurocutaneous melanosis is often associated with melanoma of the central nervous system. Due to the inconspicuously looking cutaneous melanosis additional examinations are often not performed. We describe a patient with cutaneous melanosis who presented with neurological symptoms due to a large primary meningeal melanoma. The diagnosis of neurocutaneous melanosis was made. This case is an illustration of melanoma development in the central nervous system in a patient with cutaneous melanosis. This phenomenon should be kept in mind when observing patients with such skin lesions, even if they are of minor extent.     

Central nervous system involvement in Turkish children with primary hemophagocytic lymphohistiocytosis

This report mainly presents the clinical and laboratory findings in a group of 15 primary hemophagocytic lymphohistiocytosis patients with central nervous system involvement (group 1) and compares some of the findings with those of 13 hemophagocytic lymphohistiocytosis patients without central nervous system involvement (group 2). Statistical analysis showed that age and sodium level at diagnosis were significantly higher while alanine aminotransferase and bilirubin levels were significantly lower in group 1 than group 2 (P < .05). There were no statistically significant differences between the 2 groups in the other clinical, laboratory, and overall survival parameters. Three patients in group 1 initially had central nervous system involvement in the absence of systemic findings, which led to the initial misdiagnosis of these patients as central nervous system disorders other than hemophagocytic lymphohistiocytosis.     

Primary amelanotic meningeal melanomatosis

Primary melanocytic lesions of the central nervous system are rare. The spread of melanoma cells primarily into the meningeal layers is referred to as meningeal melanomatosis. A few case reports about either intracranial or intraspinal primary meningeal melanoma were published in the past. The amelanotic variation of primary melanoma in the central nervous system is a very rare event and so far no cases of primary amelanotic meningeal melanomatosis have been described in the current literature. We present a case with combined multiple intracranial and intraspinal primary malignant amelanotic melanomas.     

Malignant melanoma and the central nervous system. A guide for classification based on the clinical findings.

Twenty-seven cases diagnosed as having malignant melanoma affecting the central nervous system have been studied. In 20 patients the tumours represented secondary spread from elsewhere, but there were six who had a primary melanoma either of the spinal cord, leptomeninges, or brain. Confusion exists in the literature about how to differentiate primary from secondary tumours but this suggests several clinical factors which may indicate that the lesion is probably a primary one. This method of categorizing the cases is supported by the differences in duration of symptoms and survival times for each group and a simple classification can therefore be proposed.     

系统性红斑狼疮合并中枢神经系统感染34例临床分析

目的研究分析系统性红斑狼疮(SLE)合并中枢神经系统感染的临床表现。方法回顾性分析2001年1月至2011年12月石家庄平安医院34例SLE合并中枢神经系统感染患者临床资料,提出临床特征及其相关危险因素。结果 34例SLE合并中枢神经系统感染,发生率为1.44%;其中结核性脑膜炎25例(73.53%),新型隐球菌脑膜炎7例(20.59%),病毒性脑膜炎2例(5.88%)。给予内科常规治疗,25例结核性脑膜炎痊愈21例,死亡4例;7例隐球菌脑膜炎痊愈3例,死亡4例。病毒性脑膜炎痊愈1例,死亡1例。本文SLE合并中枢神经系统感染34例中总共死亡9例(26.47%)。全部患者在诊断前均经过糖皮质激素治疗,28例患者应用较大剂量激素治疗,18例患者联合免疫抑制剂治疗。SLE合并中枢神经系统感染的临床特点主要为高热、头痛及意识障碍;狼疮活动度评分(SLEDAI)平均9±3分。脑脊液检查发现糖和氯化物降低明显。结论①系统性红斑狼疮合并中枢神经系统感染以结核性脑膜炎最常见;②肾上腺皮质激素、免疫抑制剂的应用是SLE合并感染的危险因素;③SLE合并中枢神经系统感染与狼疮活动度无关,而神经精神狼疮多发生于狼疮高度活动期,SLEDAI评分对二者鉴别诊断有意义;④狼疮合并中枢神经系统感染以高热、头痛及颅内压增高为主要表现,反复腰穿脑脊液检查对SLE合并中枢神经系统感染的诊断和判断预后有重要价值。     

脑脊液中检测DLL1对结核性脑膜炎的诊断意义

目的探索脑脊液(CSF)中Delta-like-1(DLL1)的检测在结核性脑膜炎诊断中的临床价值。方法选择诊断明确的中枢神经系统感染性疾病患者50例,分为结核性脑膜炎(结脑组)30例,病毒性脑(膜)炎(病脑组)20例,以及正常对照组20例,颅内转移瘤(肿瘤组)8例。采用酶联免疫吸附试验定量测定患者CSF中DLL1的含量。结果各组CSF中DLL1的含量,结脑组显著高于其他组别,差异均有统计学意义(P<0.01),其他组别之间相比差异均无统计学意义(P>0.05)。各组CSF中DLL1的含量与CSF蛋白、细胞数、葡萄糖、氯化物及颅内压均无相关性。结论 DLL1检测作为一种新的指标,在结核性脑膜炎的诊断中可能有重要临床价值。     

Primary melanocytic tumors of the central nervous system: a neuroradiological and clinicopathological study of five cases and brief review of literature

Primary melanocytic tumors of the central nervous system (CNS) are uncommon lesions. These lesions arise from the melanocytes located within leptomeninges and include diffuse melanocytosis and meningeal melanomatosis (seen in neurocutaneous melanosis), melanocytoma, and malignant melanoma. To study, the clinical course, neuroradiological features, morphology and immunohistochemistry of primary melanocytic tumor of CNS. Demographic, clinical and surgico-pathologic findings of five patients with melanocytic tumors seen between 1996 and 2003 were studied. In this study, five cases of primary melanocytic tumors have been reported: four cases of malignant melanoma and one case of melanocytoma. Three of the 5 cases were intracranial and 2 were spinal. The mean age in the present study was 26 years. Presenting features varied according to the location. Primary melanocytic tumor of CNS are rare. Whenever possible, complete surgical excision is the best treatment.     

Corpus callosum anomalies: birth prevalence and clinical spectrum in Hungary

Data regarding the epidemiology of callosal anomalies are contradictory. We performed a population-based retrospective survey to study the birth prevalence and clinical features of agenesis/hypoplasia of the corpus callosum and accompanying central nervous system and somatic abnormalities in southeastern Hungary between July 1, 1992 and June 30, 2006. Among 185,486 live births, 38 patients (26 boys and 12 girls) manifested agenesis/hypoplasia of the corpus callosum, corresponding to a prevalence of 2.05 per 10,000 live births (95% confidence interval, 1.4-2.7). Callosal anomalies were isolated in 18 patients, and were associated with other central nervous system malformations in five children. Both central nervous system and noncentral nervous system abnormalities were evident in seven patients, whereas callosal dysgenesis was accompanied only by somatic anomalies in eight children. Five of 18 patients with isolated agenesis/hypoplasia of the corpus callosum remained asymptomatic. Developmental delay, intellectual disability, or epilepsy occurred in all patients, except one, when callosal anomalies were combined with other brain or somatic abnormalities. Five patients with multiplex malformations died. Callosal anomalies form a clinically significant and relatively frequent group of central nervous system malformations.     

Second malignant neoplasms following the treatment of brain tumors in children

We investigated retrospectively 992 children with central nervous system tumors who were treated at our center between 1970 and 2004. All of the patients were treated by surgery, chemotherapy, and/or radiotherapy. Six patients developed second malignant neoplasms, and their clinical and histopathologic characteristics are reviewed in this article. The second malignant neoplasms were diagnosed as non-Hodgkin lymphoma, myelodysplastic syndrome, basal cell carcinoma, malignant melanoma, Kaposi sarcoma, and high-grade neuroectodermal tumor. The initial diagnoses were ependymoblastoma in one, medulloblastoma in three, and low-grade astrocytoma in two patients. The median latency time was 3.03 years (range 0.39-22.93 years). The outcome varied according to the histopathologic type of the second tumor. The patients who developed non-Hodgkin lymphoma and myelodysplastic syndrome died of progressive disease. The patients with second skin neoplasms are alive as of the time of this writing. The patient with Kaposi sarcoma developed one of the rare reported second malignant neoplasms following a primary brain tumor in childhood. A wide spectrum of second malignant neoplasms was detected after treatment of primary brain tumors with surgery, radiotherapy, and chemotherapy. Long-term follow-up is therefore necessary for the child who has survived a primary central nervous system tumor.     

仅累及中枢神经系统血管炎相关分析

目的初步探讨仅累及中枢神经系统血管炎的临床特点、检查及治疗方法。方法对诊治的11例仅累及神经系统血管炎的患者进行相关分析。结果患者多年轻化,临床表现多样化,磁共振表现无特征性,免疫系统相关检查阴性,大多对激素治疗有效。结论年轻患者,反复脑血管病,而无脑血管病危险因素,即使免疫相关检查正常,也要高度警惕仅累及中枢神经系统血管炎可能,及早完善DSA,尽早激素干预治疗。