原发性中枢神经系统淋巴瘤发病不同阶段影像学和病理学特点

目的回顾1例弥漫性大B细胞淋巴瘤不同发病阶段的临床、影像学和病理学特点,并分析其可能的发生机制。方法与结果女性患者,29岁,汉族。首次发病以视物模糊、视野缺损为主要临床表现;头部MRI显示右侧顶枕叶皮质下斑片状T1WI稍低信号、T2WI和FLAIR成像高信号,无明显占位效应,增强扫描病灶呈点状强化;激素冲击治疗后病灶逐渐消失。再次发病以头痛、呕吐和左侧肢体瘫痪为主要临床表现;头部MRI显示右侧额顶叶大片状T1WI低信号、T2WI和FLAIR成像高信号,占位效应明显,增强扫描病灶呈实性强化。于手术显微镜下全切除肿瘤。组织学形态观察,肿瘤细胞体积较大,胞质较丰富,胞核大小形态不一,核分裂象易见,片状坏死,间质小血管增生。免疫组织化学染色,肿瘤细胞胞膜CD20、胞核配对盒基因5和多发性骨髓瘤癌基因1呈阳性,少数肿瘤细胞胞膜CD10和CD30、胞核周期蛋白D1呈阳性,CD3、间变性淋巴瘤激酶和胶质纤维酸性蛋白呈阴性,Ki-67抗原标记指数为80%。EBER原位杂交检测EB病毒编码m RNA呈阴性。肿瘤组织呈明显噬血管特性,围绕并侵犯血管壁,最终病理诊断为弥漫性大B细胞淋巴瘤。结论原发性中枢神经系统淋巴瘤的影像学表现多样,呈实性占位性或浸润性病变,是肿瘤发病不同阶段的不同表现,与肿瘤嗜血管特性有关。     

中枢神经系统原发性间变性大细胞淋巴瘤

研究背景中枢神经系统原发性间变性大细胞淋巴瘤可发生于各年龄阶段,通常与免疫缺陷无关,临床及影像学检查易误诊为脑膜炎症性病变,尤其是结核性脑膜炎。而在病理诊断上,形态与中枢神经系统以外的间变性大细胞淋巴瘤相似,间变性淋巴瘤激酶1可呈阳性或阴性。由于易误诊为脑膜炎而于组织活检前应用糖皮质激素治疗,造成组织学观察呈现大片坏死,以及大量组织细胞增生和吞噬现象,故在取材不够全面时易误诊为脑梗死或恶性组织细胞增生性疾病等。本文结合1例12岁中枢神经系统原发性间变性大细胞淋巴瘤患儿的临床资料,通过相关文献回顾,总结该病发病特点和临床表现,以提高临床及病理医师对该病的认识。方法与结果 12岁男性患儿,临床表现为发热、头痛,伴右侧肢体麻木、无力。MRI检查右侧顶叶局部脑回肿胀及软脑膜异常强化,并累及右侧颞叶;左侧顶叶软脑膜异常强化。右侧颞顶叶病变组织活检肿瘤细胞体积较大且形态不规则,胞质丰富、嗜伊红,可见马蹄形和肾形核。免疫组织化学检测肿瘤细胞CD3、CD45RO、CD30、间变性淋巴瘤激酶1和上皮膜抗原表达阳性,CD20和CD79a表达阴性。结论间变性大细胞淋巴瘤是中枢神经系统的罕见病理亚型,临床及影像学极易误诊为脑膜炎症性病变。因此,对临床考虑为脑膜炎,但治疗效果差、病情反复的患者,应尽早进行脑组织活检或反复脑脊液细胞学检查,尤其是脑组织活检为明确诊断之重要手段。     

腺样型胶质母细胞瘤

目的回顾1例腺样型胶质母细胞瘤患者的诊断与治疗经过,总结此类肿瘤的组织病理学特征及诊断与鉴别诊断要点。方法与结果男性患者,63岁,临床表现为口角左偏10余天。头部MRI增强扫描提示左侧额颞叶占位性病变,考虑转移瘤可能性大。18F-脱氧葡萄糖(18F-FDG)PET显像未见恶性肿瘤征象。行神经导航联合术中超声引导下左侧额颞叶占位性病变切除术,于手术显微镜下全切除病变。组织学形态观察,肿瘤细胞呈片状或巢状多中心生长,部分肿瘤区域黏液丰富;肿瘤细胞呈条索状、筛状、腺腔样或乳头状排列;肿瘤细胞胞质较少,胞核大小较一致、呈圆形或卵圆形、核深染,偶见明显核仁;可见肾小球样血管内皮细胞增生。免疫组织化学染色,肿瘤细胞胞质弥漫性表达胶质纤维酸性蛋白、波形蛋白和同源性磷酸酶-张力蛋白,胞核表达少突胶质细胞转录因子2和P53,胞质和胞核表达S-100蛋白,胞膜表达表皮生长因子受体,不表达细胞角蛋白、上皮膜抗原、癌胚抗原、甲状腺转录因子-1、CD31、CD34、CAM5.2和异柠檬酸脱氢酶1,Ki-67抗原标记指数约为76.80%。最终病理诊断为腺样型胶质母细胞瘤。术后12 d因呼吸功能和循环功能衰竭死亡。结论腺样型胶质母细胞瘤临床极为罕见,明确诊断依靠特异性组织形态学特征和免疫组织化学染色。应注意与转移性腺癌相鉴别。     

以海绵窦综合征为首发症状的间变性大细胞淋巴瘤

目的回顾分析1例以海绵窦综合征为首发症状的间变性大细胞淋巴瘤患者的临床病理学特点。方法与结果女性患者,35岁。头面部疼痛1个月、视物成双7天、右上睑下垂4天;头部MRI显示右侧海绵窦区异常信号影,考虑垂体腺瘤或海绵窦综合征可能。激素治疗无效后改行手术部分切除病灶。组织学形态可见肿瘤细胞呈弥漫浸润性生长、异型性明显,胞质丰富、粉染,胞核呈圆形、卵圆形、不规则形,部分胞核呈肾形。免疫组织化学染色肿瘤细胞胞膜表达CD30和CD56,胞膜和胞质表达上皮膜抗原,不表达间变性淋巴瘤激酶,Ki-67抗原标记指数达80%。术后一般情况较差,无法耐受放射治疗和药物化疗,术后3周死亡。结论临床表现为海绵窦综合征的患者应警惕间变性大细胞淋巴瘤的可能,必要时行组织病理学检查,诊断过程中需注意与未分化型非角化性鼻咽癌、生殖细胞瘤、浆母细胞性淋巴瘤、恶性黑色素瘤和经典型霍奇金淋巴瘤等相鉴别。     

原发性眶内卵黄囊瘤:病例报告并文献分析

目的 探讨临床少见原发于眶内卵黄囊瘤的病理学特征.并讨论颅内生殖细胞肿瘤的病理学鉴别要点.方法 分析l例原发于左侧眶尖卵黄囊瘤患儿的临床表现.HE和免疫组织化学染色观察颅内卵黄囊瘤的临床病理学特点及免疫表型.结果 患者男性.2岁.表现为左眼上睑下垂并眼球突出.MRI显示颅底肿瘤侵犯左侧后组筛窦、眼眶及翼窝并蝶骨翼板,左侧上颌窦后壁、筛骨及眼眶尖部骨质破坏,与硬膜分界尚清,未突破硬膜向脑实质浸润.术中可见肿瘤位于眶尖区之眶内外,累及左侧海绵窦区,位于硬膜外尚未侵入硬膜下区,无包膜,破坏眶尖区骨质,长人蝶窦和筛窦.肿瘤组织由许多相互交通的间隙形成疏松的网状结构,衬覆胞质透亮的圆形或多边形细胞,核深染、不规则且核仁突出,核分裂象多见;可见Schiller-Duval(S-D)小体结构.为单个圆形或长形乳头状结构,含有单个血管的纤维血管轴心,被覆柱状细胞,乳头占据的间隙衬覆立方、扁平或"鞋钉"样细胞;肿瘤细胞内或细胞间可见嗜伊红且PAS阳性透明小体.肿瘤细胞弥漫表达细胞角蛋白,灶性表达甲胎蛋白、CD99和CD117,但不表达CD30、CD45、胎盘碱性磷酸酶、突触素、嗜铬素A和CD34.连续组织切片HE染色未发现合并其他类型生殖细胞肿瘤.病理诊断左侧眶尖原发性卵黄囊瘤(WHOⅣ级).结论 颅内卵黄囊瘤好发于青少年,多生长于中线结构,具有特殊的组织学构象和免疫组织化学表型,预后不良,诊断时应与其他生殖细胞肿瘤相鉴别,并注意是否同时合并其他生殖细胞肿瘤成分.     

脊髓内梭形细胞横纹肌肉瘤:一例报告并文献复习

目的 探讨梭形细胞横纹肌肉瘤的临床表现及病理学特征.方法 回顾分析1例脊髓内梭形细胞横纹肌肉瘤患者的临床表现、组织形态学及免疫组织化学特征,并复爿相关文献.结果 男性患者,50岁.临床主要表现为颈肩部疼痛伴左侧上肢麻木、无力,左侧下肢无力,排尿困难.颈椎MRI检查C5～6脊髓内异常信号伴脊髓卒洞.手术中可见肿瘤位于颈椎C5～6脊髓,椭圆形,呈灰白、灰褐色,大小约3.00 cm×1.50 cm×1.50 cm,质地坚韧,血液供应丰富,似有包膜,与周围神经组织粘连紧密,蛛网膜与硬脊膜粘连.组织形态学观察肿瘤主要由形态一致的梭形细胞组成,呈"漩涡状"紧密排列;肿瘤细胞边界清楚,细胞质少,呈嗜酸性;细胞核深染,呈长卵圆形,偏位,可见小的核仁和核分裂象;肿瘤细胞之间有数量不等的胶原纤维.免疫组织化学染色肿瘤细胞胞质中结蛋白、波形蛋白、肌特异性肌动蛋白表达阳性;胞核中肌浆蛋白表达阳性,Ki-67抗原标记指数约50%;部分肿瘤细胞胞膜和(或)胞质中CD57表达阳性,而平滑肌肌动蛋白、上皮膜抗原、神经元特异性烯醇化酶、胶质纤维酸性蛋白、广谱细胞角蛋白、钙视网膜蛋白和S-100蛋白表达阴性.患者手术后末接受其他辅助治疗,6个月后死亡.结论 梭形细胞横纹肌肉瘤为胚胎型横纹肌肉瘤的一种亚型,主要山紧密排列成漩涡状或长束状、形态一致的梭形细胞组成.多见于儿童和青少年,预后良好;发生于成人者,则预后不良.     

弥漫性星形细胞瘤,IDH-突变

正2016年世界卫生组织(WHO)中枢神经系统肿瘤分类将"弥漫性星形细胞瘤,IDH-突变"定义为伴异柠檬酸脱氢酶1/2(IDH1/2)基因突变的弥漫浸润性星形细胞瘤。其典型特征为中度细胞多形性,分化较好,生长缓慢,ATRX和TP53基因突变支持诊断。该肿瘤好发于青年,可发生于中枢神经系统任何部位,常累及额叶。组织学形态观察,肿瘤组织由分化良好的纤维性星形胶质细胞组成,间质疏松,呈微囊样,肿瘤细胞密度轻至中等,胞质不明显,胞核呈"雪茄"状或不规则深染(图1)。免疫组织化学染色,肿瘤细胞胞质弥漫性表达胶质纤维酸性蛋白(GFAP),但表达强度不一,胞质强阳性表达、胞核弱阳性表达抗     

原发性中枢神经系统淋巴瘤影像学及病理学特征临床研究

目的探讨原发性中枢神经系统淋巴瘤的临床表现、影像学和病理学特征。方法回顾分析6例经术后病理学检查证实的原发性中枢神经系统淋巴瘤患者的临床资料,分析其临床表现、影像学和组织病理学特征。结果原发性中枢神经系统淋巴瘤临床表现多样,以头痛、头晕多见。6例患者中肿瘤单发5例、多发1例,共7个病灶,病灶位于大脑半球4个、小脑1个、侧脑室1个、丘脑1个,其中2个病灶累及胼胝体。病灶多呈类圆形或不规则形,边界较清晰,肿瘤周围水肿和占位效应相对较轻。CT显示肿瘤呈高密度,T1WI呈等或略低信号、T2WI呈等或稍高信号、增强扫描肿瘤呈均匀强化。所有患者均行手术治疗。光学显微镜观察,肿瘤细胞围绕血管周围分布,形成"袖套"样浸润;免疫组织化学表型分析,肿瘤均来源于B淋巴细胞。结论原发性中枢神经系统淋巴瘤影像学和病理学表现具有一定特异性,主要病理学类型为弥漫性大B细胞淋巴瘤,组织学形态和免疫组织化学表型分析是明确诊断的"金标准"。     

伴印戒细胞样细胞分化的中枢神经系统胶质母细胞瘤:病例报告及文献复习

目的 探讨中枢神经系统胶质母细胞瘤的临床表现及病理学特征.方法 回顾分析1例伴印戒细胞样细胞和神经节细胞分化的中枢神经系统胶质母细胞瘤患者的临床表现、组织病理学和免疫表型特点,采用免疫组织化学染色方法诊断与鉴别诊断,并复习相关文献.结果 男性患者,29岁.临床主要表现为头晕、头痛、记忆力减退并渐进性加重,伴双眼视力下降.MRI检查右侧额叶近中线灰白质区占位性病变伴出血.手术中可见肿瘤瘤体主要位于右侧额叶,小部分沿胼胝体浸润至左侧额叶,大小约为4.50 cm×5.00 cm×5.00 cm,与正常脑组织边界不清;肿瘤呈实性、灰红色、质地柔软、血液供应丰富.光学显微镜观察肿瘤主要由胶质分化的细胞组成;肿瘤细胞排列呈实性片状,散在单核或多核瘤巨细胞,可见周围有肿瘤细胞呈假"栅栏"样排列的小灶性坏死及呈肾小球样的间质血管增生;肿瘤细胞具有明显的异型性、细胞核深染以及核分裂象;部分肿瘤细胞呈印戒细胞样细胞和神经节细胞分化特征.免疫组织化学染色肿瘤细胞胞质胶质纤维酸性蛋白、神经微丝蛋白、巢蛋白和蛋白基因产物9.5表达阳性,部分肿瘤细胞胞质嗜铬素A和突触素表达阳性,肿瘤细胞胞核P53蛋白表达阳性,Ki-67抗原标记指数(MIB-1)为20%～30%.结论 中枢神经系统胶质母细胞瘤是恶性程度最高的星形细胞肿瘤,也是中枢神经系统好发的肿瘤,但伴印戒细胞样细胞和神经节细胞分化的胶质母细胞瘤临床少见,好发于成年人,预后不良.     

伴上皮样分化的中枢神经系统胶质肉瘤:病例报告及文献复习

目的 探讨中枢神经系统胶质肉瘤的临床表现及病理学特征.方法 回顾分析1例伴上皮样分化的中枢神经系统胶质肉瘤患者的临床表现、组织病理学以及免疫表型特点,并复习相关文献.结果 临床主要表现为头痛,渐进性加重伴意识障碍;头部CT检查显示左侧颞顶枕叶占位性病变并有出血灶.手术中可见肿瘤位于左侧颞顶枕叶,呈囊实性,大小约6 cm×6 cm×5 cm,呈侵袭性生长,顶部侵及硬脑膜,底部侵及横窦;肿瘤实性区域呈黄白色、质地柔软、血液供应丰富.伴有囊性变,囊内可见出血并形成血肿.光学显微镜观察肿瘤由高级别胶质瘤和纤维肉瘤两种分化成分组成.由胶质分化来源的肿瘤细胞排列成实性片状或乳头状,或被纤维肉瘤成分分割包绕成小巢状,散在单核或多核瘤巨细胞.可见栅栏状坏死,血管增生明显,构成肾小球样结构;纤维肉瘤区域可见纤维细胞样细胞排列成席纹状;两种分化成分的肿瘤细胞均可见明显的异型性、深染的细胞核以及核分裂象.免疫组织化学染色显示胶质分化成分的肿瘤细胞胞质胶质纤维酸性蛋白表达阳性,部分肿瘤细胞胞质表达S-100蛋白、巨噬细胞表面抗原(CD68)及上皮膜抗原,Ki-67抗原标记(MIB-1)指数<5%.纤维肉瘤分化的肿瘤细胞间有丰富的网状纤维穿插,而胶质分化区域未见明显的网状纤维.结论 胶质肉瘤为临床少见的原发于中枢神经系统恶性肿瘤,含有向胶质及间叶组织分化的两种成分;好发于中年男性,恶性程度高,预后不良.     

Chronic benign lymphocytic meningitis

After considering the causes of chronic meningitis, we present a group of 7 patients who suffered a relatively benign chronic lymphocytic meningitis without focal neurological signs, and with a normal glucose concentration in the CSF. The illness lasted from 32 weeks to more than 10 years. Extensive investigations failed to reveal any cause. The illness was self-limiting in 6 out of 7 cases, but in 1 patient it is continuing after 10 years.Without speculation about the cause or causes, it appears useful to recognize the existence of such a chronic mengitis, in the hope that study of further cases will be stimulated.     

The lymphocytic cathepsins B-1 and D activities in multiple sclerosis

Cathepsin B-1 and D activity in lymphocytes obtained from 25 multiple sclerosis (MS) patients with various degrees of disability due to the disease and from a similar number of control subjects were studied. No differences were found between the enzyme activities of cells obtained from MS patients and controls. Age, sex and the degree of disability in the patients at the time of testing did not correlate with the enzyme activities found. The results from the present study are discussed in relation to the probable role played by lymphocytes in the demyelination process.     

Fingerprint profiles in lymphocytic vacuoles of mucopolysaccharidoses I-H,II, III-A,and III-B

Summary Fingerprint (FP) profiles in vacuolated lymphocytes of mucopolysaccharidoses I-H, II, III-A, and III-B are a numerically rare, but possibly consistent finding as they have not been seen in vacuolated lymphocytes of other non-neuronal lipofuscinosis (NCL) lysosomal diseases. Their nosologic significance is not clear, but they may be as non-specific as tubular inclusions in lymphocytes and they are identical to those FP profiles seen in juvenile NCL.     

Natural course of lymphocytic infundibuloneurohypophysitis

Natural course of lymphocytic infundibuloneurohypophysitis is poorly understood. A 49-year-old male had noticed being unnaturally thirsty since about two years previously. An enlargement of the pituitary stalk and pituitary gland was thus observed by MR at that time. However, no medical care had been given. Two years later, he was admitted to our hospital due to headache in addition to panhypopituitarism. The histologic features included T cell dominant lymphocytes infiltrating prominently the entire pituitary gland with a small amount of multinucleated giant cells, focal and small necrosis, cholesterin crystals and granuloma. Neither tuberculosis nor Langerhans histiocytosis were observed. In addition, the patient was found to have a unique massive well-encapusulated lesion in the sphenoid sinus, just below the pituitary fossa, consisting of serous fluid, normal columnar epithelium and submucosal fibrosis. This patient had a fairly typical clinical manifestation of lymphocytic infundibuloneurohypophysitis with invason of the posterior lobe and the stalk. The lesion became chronic and leaked to the sphenoid sinus. As a result, chronic hypophysitis with granuloma formation thus occurred. This case may show the course of this disease if not treated.     

Leptomeningeal disease in chronic lymphocytic leukemia

Chronic lymphocytic leukemia (CLL) is the most common lymphoproliferative disorder in the western hemisphere, with an annual incidence of 3:100 000. Commonly patients are asymptomatic but not rarely disease progression occurs in the setting of lymphadenopathy and extensive leukemic burden. Leptomeningeal involvement in patients with CLL is infrequent, with presenting symptoms of headache (23%), acute or chronic changes in mental status (28%), cranial nerve abnormalities (54%) including optic neuropathy (28%), weakness of lower extremities (23%) and cerebellar signs (18%). In this report, we discuss a CLL patient with leptomeningeal involvement, who presented with neurological symptoms as the first clinical sign, and a diagnosis of leptomeningeal was made based on CSF cytology and flow cytometry. Treatment consisted of radiation therapy and intrathecal chemotherapy with arabinoside–cytosine and systemic chemotherapy. On the basis of this patient-report together with 37 other previously reported cases, the clinical characteristics together with treatment options and outcome of leptomeningeal involvement in CLL are reviewed. Our case together with data from the literature indicate that a timely diagnosis and intensive treatment of leptomeningeal disease of CLL may lead to longstanding and complete resolution of neurological symptoms.     

Brain invasion by chronic lymphocytic leukemia

Brain invasion by chronic lymphocytic leukemia (CLL) is very rare, and only a handful of cases have been reported. We here report a case of 61‐year‐old woman who had been treated for CLL for 14 years presenting with a progressive mental disturbance. Magnetic resonance imaging (MRI) showed discontinuous ring‐enhancing lesions compatible with the “open ring” sign, which was considered a demyelinating disorder, in both the frontal lobes. However, on histological examination of the biopsied specimen, infiltration of small lymphocytes positive for CD5, CD20, and CD23, indicating brain invasion by CLL, was seen. The leukemia cells occupied the Virchow–Robin space and infiltrated into the brain parenchyma. The arterioles in the Virchow–Robin space were compressed and occluded with the tumor cells, while CD163‐positive cells infiltrated the brain parenchyma. Myelin staining demonstrated myelinoclasis in the infiltrated brain tissue. The MRI findings in the present case probably reflected myelinoclasis, suggesting rare brain invasion by CLL. The possibility of lymphoma should not be eliminated based on the MRI findings.