原发性颅内恶性淋巴瘤的临床特点及影像学诊断

目的 探讨原发性颅内恶性淋巴瘤的临床、影像学特征。方法 对25例经手术及病理证实的原发性颅内恶性淋巴瘤的临床、影像学表现及治疗进行了回顾性分析．结果 本病以中老年人多见，病程短．病情进展快．颅压高症状出现早，多位于幕上，可单发或多发，可侵及室管膜和软脑膜并沿之播散；CT扫捕多表现为等密度肿块，MRI显示T1加权像多呈低信号，T2加权像多呈高信号，CT和MRI增强扫捕病灶多呈均匀明显强化：对放疗、化疗敏感。结论 原发性颅内恶性淋巴瘤的临床、影像学表现与某些中枢神经系统病变有类似之处，鉴别比较困难，确诊主要依靠病理检查。最佳治疗方案是手术加放疗、化疗的联合治疗。     

原发性中枢神经系统淋巴瘤的影像学变化及临床(附1例报告及文献复习)

目的分析原发性中枢神经系统淋巴瘤MRI的动态变化及临床特点。方法对1例原发性中枢神经系统淋巴瘤患者的MRI变化、临床表现、病检结果,结合相关文献进行讨论分析。结果患者临床上反复发作,影像学上表现不典型,多在基底节、胼胝体和脑室周围,常误诊为多发性脑梗死或脱髓鞘疾病。结论原发中枢神经系统淋巴瘤临床上和影像学上酷似脱髓鞘病,行CT引导下立体定向穿刺或病灶组织活检有助于确诊。     

中枢神经系统原发性淋巴瘤影像学特征与病理分析

目的探讨中枢神经系统原发性淋巴瘤影像学特征并进行病理分析。方法回顾性分析我院收治的23例中枢神经系统原发性淋巴瘤患者的CT以及MRI表现。结果中枢神经系统原发性淋巴瘤在进行CT以及MRI检查的过程中发现肿瘤多侵入额叶以及颞叶,病理学方面B细胞占据大部分。23例患者中单发病灶16例,多发病灶7例,病灶共39个,其中额叶10个,顶叶6个,枕叶3个,颞叶6个,基底节区5个,胼胝体3个,下丘脑2个,小脑4个,CT为低等密度,T1WI是稍低信号,T2WI为低等信号,中等水肿,增强造影后明显出现均匀强化。造影显示一些病灶位于基底节、胼胝体以及脑室的周围,容易误诊为多发性脑梗死或脱髓鞘。结论绝大多数中枢神经系统原发性淋巴瘤有明显的影像学特征,可以作出正确诊断并为临床治疗提供依据。     

累及脑干的原发性中枢神经系统淋巴瘤的立体定向活检术体会

目的 总结累及脑干的原发性中枢神经系统淋巴瘤（PCNSL）的立体定向活检术经验。方法 回顾性分析2018年1月至2019年1月病理确诊累及脑干的37例PCNSL临床资料。采用Leksell头架辅助立体定向穿刺技术对颅内病变取材进行病理检查。结果 37例中，6例桥脑下部病变采取经小脑路径；31例桥脑上部病变中，5例采取经顶叶路径，26例采取经额叶路径。19例为单发病灶，18例为多发病灶。术后病理均为弥漫大B细胞淋巴瘤。经小脑路径活检术后出血2例，其中死亡1例；经额叶通路活检术后出血2例。结论 立体定向活检术是诊断累及脑干的PCNSL的有效方法，应注意防治术后出血。     

中枢神经系统炎性假瘤的诊断及治疗(一例报告并文献复习)

目的 探讨中枢神经系统炎性假瘤的临床、影像学、病理特点及治疗.方法 回顾性分析1例中枢神经系统炎性假瘤患者的临床资料.临床表现为间断性头痛伴右侧肢体无力,头颅MRI显示左侧顶叶长T1、长T2信号,增强后明显强化.结果 行开颅手术切除病变,术后效果良好,病理同报浆细胞肉芽肿;术后3个月MRI检查显示病变消失,脑组织水肿较术前明显减轻.结论 中枢神经系统炎性假瘤病例少见,临床表现和影像学改变尢特异性,术后病理检查是诊断的主要依据.手术全切除治疗效果好.     

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。     

原发性中枢神经系统恶性淋巴瘤临床诊疗初步经验

目的探讨原发性中枢神经系统淋巴瘤影像学特点及临床诊疗经验。方法对15例确诊为原发性中枢神经系统恶性B细胞型淋巴瘤患者的临床资料进行回顾性分析。结果颅内中枢神经系统原发性恶性淋巴瘤好发于脑深部组织如基底节区、小脑半球等，肿瘤T1加权像多为等或低信号，R加权像多呈高信号，也可以为等信号，DWI像呈高信号。增强扫描见病变明显均匀强化，边缘有多个小突起，似树枝状改变。10例患者行手术切除治疗，5例行组织活检．术后病理组织学及免疫组化检查证实为B细胞型淋巴瘤，术后14例行直线加速器电子线放疗及激素治疗，1个月后复查提示肿瘤明显变小；1例术后放弃放疗，给予激素治疗，肿瘤明显变小，但易复发。结论病变明显均匀强化及树枝状改变为原发性中枢神经系统淋巴瘤MRI特征，缺乏特异性；MRI表现及诊断性激素治疗有助于术前诊断，手术切除病理组织后行激素及放疗可延长患者生存期，但肿瘤容易复发。     

立体定向活检结合放疗治疗原发性颅内淋巴瘤(附17例临床分析)

目的探讨原发性颅内淋巴瘤的临床特点,总结立体定向活检结合放疗治疗原发性颅内淋巴瘤的疗效。方法本组17例怀疑为原发性颅内淋巴瘤的患者,术前均行CT及MRI检查,明确病变部位后,在MRI引导局麻下行立体定向病变活检术,术后一周拆线,病理报告回报后即行放疗。结果所有患者立体定向活检手术过程均顺利,手术时间均少于60min,出血量均少于50ml。立体定向活检手术的成功率100%,术后病理诊断均为弥漫性大B细胞淋巴瘤。术后一周拆线后即开始行放射治疗,出院随访时间1～3年,平均1.25年,随访发现生存期为6～36个月,中位生存期16.4月。结论 MRI是诊断原发性颅内淋巴瘤的主要影像学手段,病理诊断是确诊的"金标准",立体定向活检结合放疗具有损伤小,风险低,患者生存时间长等优点,具有重要的临床应用价值。     

原发性中枢神经系统淋巴瘤的影像学特征

目的总结原发性中枢神经系统淋巴瘤(PCNSL)的影像学特征,提高PCNSL诊治水平。方法回顾性分析经病理证实的PCNSL的影像学资料。结果 CT检查病灶多为稍高密度影,MRI检查病灶多为长T1、等或长T2信号影,多数病灶增强扫描为片状均匀强化,MRI增强扫描时会出现"蝴蝶征"等特征性表现,有助于PCNSL的诊断,磁共振波谱表现为Cho峰升高、NAA峰中度降低、Cr轻度降低,出现高大的Lip峰,对鉴别淋巴瘤与其它实性肿瘤具有高度的特异性。结论 MRI在PCNSL的诊断中具有重要作用,掌握PCNSL的特征性影像学特点有助于提高诊断的阳性率。     

原发性中枢神经系统淋巴瘤非典型MRI影像学表现

目的总结原发性中枢神经系统淋巴瘤(PCNSL)非典型MRI影像学表现,以提高对非典型PCNSL影像诊断的正确率。方法回顾性分析经穿刺或手术病理证实的25例原发性中枢神经系统淋巴瘤的MRI影像学表现,包括非典型的生长部位,非特异的MRI表现和强化方式。结果 25例中单发病灶者16例,多发病灶9例,病灶数共38个。不典型发生部位可见于鞍上、脑干、小脑半球和小脑蚓部、侧脑室、三脑室和第四脑室等等。病灶形态大小不一,表现形式多种多样,可呈类圆形、不规则形,团块状、结节状、斑片状、灶状或散在分布。1例病灶侵及邻近脑膜,出现"脑膜尾征"。20例在T1WI呈稍低或等信号,T2WI呈等或稍高信号,5例T1WI、T2WI呈混杂信号。7例呈实性均质强化,6例呈结节状强化,3例呈斑片状强化,2例呈条纹状强化,2例呈环形强化,1例呈弥漫肿胀伴轻度强化,3例多发病灶多种强化方式并存。5例DWI呈均质高信号,6例呈不均匀稍高信号,4例呈中等信号。MRS检查4例出现较明显的Lip峰。结论对于发生在非典型生长部位、影像学表现非特异性的,以及颅内多发病灶的和侵犯脑膜的中枢神经系统淋巴瘤,术前诊断较为困难,需引起高度重视。     

Autopsy report of primary CNS B-cell lymphoma indistinguishable from multiple sclerosis: diagnosis with the immunoglobulin gene rearrangements analysis.

We report a case of primary CNS B-cell lymphoma indistinguishable from multiple sclerosis (MS). MRI of the head showed the spontaneous disappearance of the white matter lesions and the progressive cerebral atrophy. The brain biopsy failed to make a diagnosis of CNS lymphoma but rather suggested MS. Although the primary CNS lymphoma was suspected at autopsy, the immunohistochemical study showed the CNS-infiltrating lymphoid cells comprising both T-cells and B-cells. Analysis of the immunoglobulin and T-cell receptor gene rearrangements first provided evidence of primary CNS B-cell lymphoma.     

Lymphomatosis cerebri presenting as a rapidly progressive dementia: clinical, neuroimaging and pathologic findings

Primary central nervous system lymphoma (PCNSL) usually presents with clinical and neuroimaging findings consistent with single or multiple intracranial mass lesions. On cranial magnetic resonance imaging (MRI), such lesions are nearly always contrast enhancing, reflecting disruption of the blood-brain barrier at the site of tumor nodules. We describe 2 cases from the UCLA Medical Center who developed a rapidly progressive dementia due to extensive gray and white matter cerebral lesions involving much of the brain. In the patient who came to autopsy, widely infiltrating, focally necrotic B-cell plasmacytoid lymphoma was noted throughout the cerebral neuraxis. MRI findings in case 2 were consistent with diffuse lymphomatous brain infiltration without mass lesions, which was biopsy proven. We conclude that PCNSL may occur in a diffusely infiltrating form which may occur without MRI evidence of mass lesions or blood-brain barrier compromise. We refer to this entity as 'lymphomatosis cerebri' and add it to the differential diagnosis of a rapidly progressive dementia.     

Demyelination preceding a diagnosis of central nervous system lymphoma

We present a case of primary central nervous system lymphoma (PCNSL) co-existing with demyelination in a young immunocompetent woman. The patient presented with an expansile, enhancing lesion in the right occipital lobe which was initially attributed to tumefactive demyelination and subsequently proven to be PCNSL. PCNSL is an uncommon malignancy, particularly in young immunocompetent patients, and on MRI classically manifests as a homogeneously enhancing solitary mass with a predilection for periventricular and superficial locations, often contacting ventricular and meningeal surfaces. Tumefactive demyelinating lesions typically present as large white matter lesions with little mass effect or vasogenic oedema and “open-ring” enhancement, with the incomplete portion of the ring on the grey matter side of the lesion. PCNSL and tumefactive demyelinating lesions share some radiological features and thus, as our case report highlights, differentiating between them can be challenging. We discuss how the application of conventional and advanced MRI techniques combined with clinical and laboratory findings can lead to a precise diagnosis, potentially obviating the need for biopsy and facilitating prompt and appropriate treatment.     

Neuroinflammation preceding primary central nervous system lymphoma (PCNSL) – Case reports and literature review

Primary central nervous system lymphoma (PCNSL) is a rare and aggressive form of extra-nodal non-Hodgkin’s lymphoma. Corticosteroids cause transient regression of PCNSL at the radiological and histological level. A growing number of case reports describe histologically confirmed neuroinflammation (sentinel lesions) heralding the development of PCNSL. We present two further cases of sentinel lesions contextualised by a review of past literature. Our aims are to collate existing knowledge on sentinel lesions in PCNSL and explore their pathophysiological significance. Two cases were identified (n = 2) from a cohort of 104 patients with PCNSL referred to a tertiary neurosurgery centre. A literature search identified previously reported cases (n = 14). Median age was 57.5 (range; 26–72); pre-biopsy corticosteroid administration was reported in 50% of cases (n = 8); mean time between biopsies was 10 months (range; 3–60). Common MRI features were homogenous enhancement (10;71.4%) and T2-hyperintensity (11;100%). Histochemical analysis of sentinel lesion biopsy revealed inflammatory CD3/4/5/8-positive T-cells (14; 100%), demyelination (13; 81.3%), rare/scattered CD20-postive B-cells (11;78.6%) and CD68-positive macrophages (10;71.4%). Repeat biopsy confirmed PCNSL in all cases. Waxing and waning CNS inflammation has been identified in 16 patients ultimately diagnosed with PCNSL. Neuro-specialists should be aware of this atypical presentation and maintain a high index of suspicion for lymphoma despite histopathology negative for lymphoma when clinical or radiological features indicate PCNSL.     

Primary central nervous system lymphoma initially mimicking lymphomatosis cerebri: An autopsy case report

A 59‐year‐old immunocompetent man was admitted to our hospital because of progressive dementia with concomitant bilateral uveitis. The first brain MRI revealed diffuse hyperintense lesions in the cerebral white matter of both hemispheres on a T2‐weighted image and fluid‐attenuated inversion recovery image. However, another MRI taken more than 1 month later revealed enhanced cohesive mass lesions in the bilateral thalami, in addition to the white matter lesions. The white matter lesions were slightly hyperintense on a diffusion‐weighted image and apparent diffusion coefficient map image, suggesting vasogenic edema. One year after the onset of uveitis, he died of respiratory failure. Pathological diagnosis was diffuse large B‐cell lymphoma with perivascular proliferation and diffuse scattered infiltration in the cerebrum and brainstem. Microscopically, cohesive mass lesions in the bilateral thalami were a massive cluster of lymphoma cells. This is a case of primary CNS lymphoma (PCNSL) mimicking ‘lymphomatosis cerebri (LC)’ at first but later exhibiting typical mass lesions, giving rise to the possibility that cases of LC might unmask features of regular lymphomas in their later course more often than believed thus far.     

An autopsy case of primary central nervous system lymphoma with diffuse intracerebral lesion]

A case of primary central nervous system lymphoma (PCNSL) who initially showed clinical pictures like encephalitis and diffuse lesions on MRI was reported, including postmortem pathologic examinations. A 68-year-old woman was seen in March, 1990 with a 1-month history of the progressive gait disturbance. She was very unstable and could barely stand by herself, though she did not show any focal neurologic deficits. She showed no evidence of systemic diseases. The cerebrospinal fluid analysis was normal. T2-weighted image of MRI demonstrated the diffuse symmetric hyperintense lesions mainly in the periventricular white matter. The progressive intellectual decline and the spasticity of four limbs developed as the diffuse lesions on MRI gradually extended. Despite the administration of corticosteroids under the presumptive diagnosis of PCNSL, she rapidly fell into the apallic syndrome within two months. Her EEG showed periodic synchronous discharges. Three months later, she suddenly developed signs of right uncal herniation. CT showed a large mass lesion in the right hemisphere. After the anti-edema therapy, signs of herniation regressed. The serial CT scans demonstrated a gradual decrease in the mass effect, while another multi-nodular lesions appeared and then disappeared one after another bilaterally. Eventually, the diffuse low densities in the cerebral white matter and the ventricular enlargement had remained. She died of bronchopneumonia eight month after the onset of symptoms. The clinical importance of the diagnosis of PCNSL which initially shows diffuse symmetric lesions without a mass is stressed. Postmortem examinations revealed PCNSL, diffuse, large cell type according to the Lymphoma Study Group classification. The lymphoma cells were proved to be B cell origin from the immunohistochemical study of frozen tissue.(ABSTRACT TRUNCATED AT 250 WORDS)     

Absence of thallium-201 brain uptake in progressive multifocal leukoencephalopathy in AIDS patients

OBJECTIVE: To evaluate the presence of thallium-201 brain uptake determined by thallium-201 brain SPECT (Tl-201 SPECT) in patients with progressive multifocal leukoencephalopathy (PML) and AIDS. MATERIAL AND METHODS: Six AIDS patients with stereotactic biopsy diagnosis of PML were prospectively evaluated with Tl-201 SPECT, Magnetic Resonance Imaging (MRI), and proton magnetic resonance spectroscopy (1H-MRS). Tl-201 SPECT results were compared with 2 patients with AIDS and biopsy proven primary CNS lymphoma. RESULTS: In all patients with PML, Tl-201 SPECT studies showed lack of uptake while MRI demonstrated subcortical white matter focal brain lesions and 1H-MRS disclosed metabolic abnormalities. Intense thallium uptake (uptake ratios of 3.2 and 5.6) was demonstrated in the 2 patients with primary CNS lymphoma. CONCLUSIONS: The present study shows that PML lesions are not detectable on Tl-201 SPECT while MRI and 1H-MRS demonstrate abnormalities, and intense thallium-201 uptake may be detected in primary CNS lymphoma. These results suggest that Tl-201 SPECT is a method which, combined with other non-invasive techniques such as MRI and 1H-MRS, may help in the diagnostic approach of PML and to differentiate PML from other high proliferative brain lesions characterized by positive thallium uptake.     

Inflammatory demyelinating brain lesions heralding primary CNS lymphoma

Brain biopsy plays a crucial role in the exploration of suspect white matter lesions in the differential diagnosis of primary central nervous system lymphoma (PCNSL) and inflammatory demyelination. We present the case of a previously healthy, immunocompetent woman, aged fifty-nine, who developed a histologically confirmed demyelinating white matter lesion months prior to the manifestation of a PCNSL. Similar cases of "sentinel lesions" preceding a PCNSL have been reported. In a literature review, we compared the diagnostic features that may be useful to differentiate a PCNSL from inflammatory demyelinating disease in older age. We conclude that the occurrence of large, contrast-enhancing cerebral lesions in older patients with a relapsing-remitting disease course and steroid-resistant vision disorders should lead to the consideration of a PCNSL.     

“Ghost and Mimicry-Tumor”– Primäres ZNS-Lymphom

In recent years, the frequency of primary cerebral lymphoma (PCNSL) has increased, even among immunocompetent patients. In order to treat the disease optimally, early diagnosis is important. We present three patients with atypical courses of this disease and stress the importance of PCNSL in the differential diagnosis for optimal treatment. In a 75-year-old man, a space-occupying, radiopaque, enhancing CNS lesion disappeared completely after biopsy and short steroid therapy. One year later, the tumor recurred on the other side and again regressed after steroid therapy. The first biopsy showed signs of a papillary tumor, so a choroid plexus papilloma was suspected initially. A 57-year-old woman developed progressive bilateral hearing dysfunction. Lymphocytic pleocytosis led to a primary diagnosis of chronic lymphocytic meningitis. During the further course of disease, the patient developed multiple space-occupying cerebral lesions. Stereotactic biopsy revealed PCNSL. Despite combined chemo- and radiotherapy, a relapse occurred. A 49-year-old woman rapidly developed memory and concentration disturbances. Computed tomography revealed diffuse edema in both hemispheres and MRI detected severe, diffuse, white matter lesions. The CSF revealed lymphocytic pleocytosis including plasma cells. Herpes encephalitis and, after lack of clinical improvement and progression of the MRI changes, acute disseminated encephalomyelitis (ADEM) were suspected. Corticosteroid treatment was initiated. After enormous clinical improvement, a clinical relapse occurred and MRI detected bitemporal and singular space-occupying lesions in the corpus callosum and hypothalamus. Finally, open biopsy showed PCNSL. However, on examination of the treatment history of patient 1, the initial diagnosis must be revised; a PCNSL seems most probable. The phenomenon of tumor remission under steroid administration is rare. In patients 2 and 3, atypical clinical signs and symptoms delayed diagnosis of PCNSL. This tumor can mimic diverse neurological diseases and remit following corticosteroid treatment alone. In unclear cerebral disease, biopsy should be performed early for exact diagnosis and optimal treatment.     

Primary CNS lymphoma: a whole-brain disease?

BACKGROUND: Autopsy studies reveal that most primary CNS lymphomas (PCNSL) extensively infiltrate the brain. To date, there has been no correlation of autopsy findings with a modern neuroimaging assessment of tumor burden. OBJECTIVE: To correlate autopsy findings in PCNSL to MRI findings. METHOD: From the authors' database of immunocompetent patients with PCNSL diagnosed between 1985 and 2001, 10 patients who died and had PCNSL at autopsy were identified. Their pathology was compared to MR scans obtained shortly before death. RESULTS: The median patient age was 60 years (range 44 to 80 years). There were six men and four women. Scans were performed within 4 weeks of death in seven patients, within 3 months in two patients, and within 4 months in one. Seven had enhancing lesions consistent with recurrence, and two of the three had focal T2 abnormalities. All had periventricular white matter abnormalities on T2 MR images. At autopsy, all 10 patients had widespread lymphoma throughout the CNS, but no tumor was found systemically. All had tumor infiltration in CNS regions that were normal radiographically, including T2 sequences. CONCLUSIONS: MRI underestimates the tumor burden of PCNSL. Bulky disease is seen as a contrast-enhancing lesion because of disruption of the blood-brain barrier, but microscopic tumor infiltration may lead to T2 hyperintensity or be completely normal radiographically.