肿瘤样脱髓鞘病及原发性中枢神经系统血管炎的临床、影像及病理学研究

发布时间：2018-07-26 13:31

【摘要】：目的：分析肿瘤样脱髓鞘患者的影像学(MRI)特征。方法：对2006年1月至2014年1月期间就诊于解放军总医院、因被误诊为脑肿瘤等占位性疾病而行手术或者需同肿瘤相鉴别而行活检的肿瘤样脱髓鞘病患者的影像学资料(MRI)进行回顾性分析,将以下影像学参数,包括病灶数量、位置、大小、水肿及占位效应及强化特点进行分析,并将末次随访时病灶数量与术前进行对比。结果：47.1%的肿瘤样脱髓鞘患者只有1个颅内病灶,52.9%的患者颅内病灶数量≥3个(其中2例合并出现脊髓病灶)；52.9%的患者病灶位于皮层下或深部白质、47.1%位于例脑室旁、35.3%位于近皮层或皮层；82.4%的患者病灶周围有不同程度的水肿及占位效应；所有病灶中,直径最小者为2.6cm,最大者为11.5cm,中位数及四分位数为4.8(3.5,5.9)cm,去除病灶周围水肿后,病灶大小范围为1.4-5.1cm,中位数及四分位数为3.4(2.7,3.7)cm；82.4%的患者增强扫描后出现强化,其中边缘或环形强化占50%,斑片状强化占35.7%,21.4%的患者出现血管样强化；经过中位数为4.2年的随访,8例单病灶患者中有7例未出现新病灶,原有病灶呈术后改变,9例多病灶患者中5例未出现新发病灶,4例病灶数量较前增加。结论：肿瘤样脱髓鞘病灶可单发,也可以多发;病灶直径往往大于2cm,且多具有水肿及占位效应；病灶位置以白质为主,但皮层灰质亦可受累；增强扫描后病灶多呈环形、斑片状及血管样强化；无论采用激素治疗与否,肿瘤样脱髓鞘病灶术后可减小或消失,少数患者即使经激素治疗后病灶仍增大或数量增加。目的：分析肿瘤样脱髓鞘病灶的组织病理学特点。方法：对2006年1月至2014年1月期间就诊于解放军总医院,因被误诊为脑肿瘤等占位性疾病而行手术或者需同肿瘤相鉴别而行活检的肿瘤样脱髓鞘病患者进行回顾性研究,收集其术后病理切片或石蜡组织块,并对以下病理学参数进行统计分析：病灶边界、炎症反应分布位置、炎症细胞类型,有无血管周围淋巴套袖,有无髓鞘脱失及轴索相对保留、星形胶质细胞增生情况,有无组织坏死、血管增生或血管壁炎性浸润。对于部分行开颅病灶切除术的患者,根据其详细的手术记录分析其术中肉眼所见及术中冰冻病理结果。结果：35.3%的病灶与周围脑组织边界清楚；全部患者存在活动性脱髓鞘反应过程,镜下可见：脑实质炎症细胞浸润,髓鞘脱失与轴索相对保留。100%的患者病灶内巨噬细胞增生,其中52.9%的患者内含髓鞘吞噬碎片；88.2%的患者可见星形胶质细胞增生,其中29.4%可见Creutzfeldt细胞；76.5%的患者存在血管周围淋巴细胞套,47.1%存在毛细血管增生,17.6%的患者血管壁有炎症细胞浸润,35.3%存在组织结构分解,17.6%可见组织坏死。6例行开颅切除术的患者术中病理切片均误诊为“低级别胶质瘤”结论：TDLs病理学示非特异性的炎性脱髓鞘改变,组织内可见坏死及血管壁炎症反应。即使是病理学资料(特别是冰冻病理切片),在诊断TDLs时也经常误诊；TDLs内常因为星形胶质细胞大量增生,有时是异形性增生而易被误诊为胶质细胞瘤,故建议在病理学鉴别炎性脱髓鞘与胶质瘤时,将LFB-PAS、CD68染色作为常规操作。目的：总结经病理证实的原发性中枢神经系统血管炎的临床、影像、病理特征及与肿瘤样脱髓鞘病的异同点。方法：回顾性分析2004年1月至2014年1月期间就诊于解放军总医院,因误诊为脑肿瘤、感染或其他占位性疾病而行手术或需与肿瘤相鉴别而行活检的原发性中枢神经系统血管炎患者的临床、影像及病理特点,并与经病理证实的肿瘤样脱髓鞘病相对比。结果：共有16例患者入组,其中女性5例,男性11例,发病年龄中位数为40岁。大多数患者急性或亚急性起病,手术距首次出现临床症状的时间间隔中位数为3.2周；75%的患者术前为首次病程,12.5%术前病情有缓解复发,12.5%术前呈进展趋势；在中位数为2.5年的随访中,50%患者术后保持单时相病程,25%呈缓解复发趋势,25%术后病情仍进行性加重；81.3%的患者术前曾被误诊(如脑肿瘤、脑脓肿、脑出血或寄生虫感染)；患者临床表现缺乏特异性,主要包括56.3%肢体无力、43.8%头痛、37.5%感觉障碍及31.3%头昏；68.8%的患者为颅内单发病灶,31.3%颅内病灶数量≥3个；81.3%的患者病灶位于皮层及皮层下,43.8%位于内囊及基底节区,37.5%位于侧脑室旁,31.3%累及丘脑,18.8%位于幕下；所有病灶大小范围3～8 cm,中位数及四分位数为7.3(5.4,7.6)cm,去除病灶周围水肿后,病灶大小范围为1.4～4.4cm,中位数及四分位数为3.3(2.5,3.6)cm；81.3%的患者病灶有中-重度水肿及占位效应；增强扫描后全部患者出现强化,其中边缘或环形强化占50%,软脑膜强化者占43.8%,脑回样、结节样强化各占25%；镜下可见全部患者病灶内：坏死,巨噬细胞、星形胶质细胞增生,血管壁炎细胞浸润(其中50%淋巴细胞性血管炎、37.5%急性坏死性血管炎、12.5%肉芽肿性血管炎),87.5%的患者血管周围炎症细胞浸润,62.5%可见继发性髓鞘部分脱失。7例患者病程中接受甲强龙冲击治疗,但只有2例有效,4例仍进展；3例患者应用环磷酰胺治疗,只有1例有效。总体预后略好于术前：术前Rankin评分中位数及四分位数为2(1,3)分,末次随访时为1.5(0,4)分。结论：原发性中枢神经系统血管炎临床表现缺少特异性,其常规核磁检查及病理改变均与肿瘤样脱髓鞘有重叠之处,二者很难鉴别。但总体上原发性中枢神经系统血管炎病灶多同时累及皮层及皮层下,且水肿及占位较肿瘤样脱髓鞘更加明显。若常规核磁及病理均不能鉴别两者,建议同时行血管造影或MRA检查,且病理标本应同时包括软脑膜、皮层及皮层下组织。目的：总结肿瘤样脱髓鞘病的一般及临床特点。方法：对2006年1月至2014年1月期间就诊于解放军总医院,因被误诊为脑肿瘤而行手术或者需同肿瘤相鉴别而行活检的肿瘤样脱髓鞘病患者进行回顾性研究,对一般流行病学特点、临床表现、误诊情况、病情发展特点、治疗及预后进行分析。结果：共有17例患者入组,其中女性9名,男性8名,发病年龄中位数为47岁；术前有82.4%的患者被误诊为颅内肿瘤、脓肿或寄生虫感染等占位性疾病,17.6%的患者因需要同肿瘤相鉴别而行活检手术；患者多急性或亚急性起病,手术距离首次出现临床症状的时间间隔中位数为5.3周；82.4%的患者术前为首次发病,11.8%术前病情有缓解复发,5.9%术前病情呈进行性加重,经过中位数为4.2年的随访,术后有70.6%的患者呈单时相病程,17.6%病情有缓解复发,11.8%病情仍进行性加重；临床症状多种多样,主要包括41.2%肢体无力、23.5%视物模糊、23.5%吞咽呛咳或复视等脑干症状；8例患者术后接受甲强龙冲击治疗,但只有4例有效,另外9例术后未行任何针对性治疗,但临床及影像均逐渐好转且未再复发；末次随访时64.7%患者诊断为临床孤立综合征,11.8%诊断为MS,另有23.5%不能明确归类于已知脱髓鞘病；术前EDSS评分中位数为2分,末次随访时为0分。结论：肿瘤样脱髓鞘病多急性或亚急性起病,其临床表现差异性较大。大多数患者呈单时相病程,少数可缓解复发或进行性发展。部分患者于病灶切除后可自然缓解,激素治疗只对部分患者有效,本病预后相对良好。
[Abstract]:Objective: to analyze the imaging (MRI) characteristics of the patients with tumor like demyelinating. Methods: the imaging data of patients with tumor like demyelinating disease (MRI) were retrospectively analyzed from January 2006 to January 2014 at the General Hospital of the PLA. Analysis of the following imaging parameters, including the number, location, size, edema, occupying effect and intensification of the lesion, and comparing the number of lesions at the last follow-up. Results: 47.1% of the patients with tumor like demyelination had only 1 intracranial lesions and 52.9% of the patients had more than 3 intracranial lesions (2 of them were associated with the spinal cord. " 52.9% of the lesions were located in subcortical or deep white matter, 47.1% in the paraventricular side, 35.3% in the proximal cortex or cortex; 82.4% of the lesions were around the lesion with varying degrees of edema and occupying effect; among all the lesions, the smallest diameter was 2.6cm, the largest was 11.5cm, the median and four quantiles were 4.8 (3.5,5.9) cm, removing the focus. After peripheral edema, the size of the lesion was 1.4-5.1cm, median and four digits were 3.4 (2.7,3.7) cm; 82.4% of the patients were enhanced after enhanced scan, including edge or ring enhancement 50%, patchy enhancement 35.7%, 21.4% of patients with vascular enhancement, and 4.2 years of median follow-up, 7 in 8 patients with single lesion. In the present new lesion, the original lesion was changed after operation. In 9 patients with multiple lesions, 5 cases had no new focus, and 4 cases had more lesions than before. Conclusion: the tumor like demyelinating lesions can be single and multiple, the lesion diameter is often greater than 2cm, and most of the lesions have edema and occupying effect, the focus position is white matter, but cortex gray matter can also be involved. The foci were mostly ring, flaky and vascular enhancement after enhanced scan. The tumor like demyelinating lesions could be reduced or disappeared after the treatment with hormone treatment or not. A few patients increased or increased even after hormone treatment. Objective: to analyze the histopathological features of the tumor like demyelinating lesion. Methods: January 2006 A retrospective study was conducted at the General Hospital of the PLA during January 2014. A retrospective study was conducted for patients with tumor like demyelinating diseases, which were misdiagnosed as brain tumors such as brain tumors, or were required to be identified with the tumor. The postoperative pathological sections or paraffin tissue blocks were collected and the pathological parameters were analyzed. The location of the inflammatory reaction, the type of inflammatory cells, the sleeve of the perivascular lymph nodes, the absence of myelinating and the relative retention of the axons, the proliferation of astrocytes, the necrosis of the tissue, the proliferation of blood vessels, or the inflammatory infiltration of the blood vessels. Results: 35.3% of the pathological results were seen in the intraoperative ice. Results there was a clear boundary between the lesion and the surrounding brain tissue; all the patients had active demyelination process, and the inflammatory cells infiltrated in the parenchyma, and the myelin sheath was lost and the axon retained.100%. In the patients, 52.9% of the patients had phagocytosis in the myelin sheath. 88.2% of the patients showed astrocyte proliferation, of which 29.4% were Creutzfeldt cells, 76.5% of the patients had perivascular lymphocyte sets, 47.1% had capillary hyperplasia, 17.6% of the patients had inflammatory cell infiltration, 35.3% had tissue structure decomposition, and 17.6% tissue necrosis.6 routine craniotomy patients. Pathological sections were misdiagnosed as "low grade glioma" conclusion: TDLs pathology shows nonspecific inflammatory demyelinating changes, necrosis and vascular wall inflammation in tissues. Even pathological data (especially frozen pathological sections) are often misdiagnosed in the diagnosis of TDLs; TDLs often occurs because astrocytes proliferate in large numbers. Sometimes it is heteromorphic hyperplasia and easily misdiagnosed as glioblastoma. Therefore, it is suggested that LFB-PAS, CD68 stain be used as a routine operation in the pathological identification of inflammatory demyelinating and glioma. Objective: To summarize the clinical, imaging, pathophysiological features of primary central nervous system vasculitis confirmed by pathology and the similarities and differences with the tumor like demyelinating disease. Methods: a retrospective analysis of the clinical, imaging and pathological features of primary central nervous system vasculitis in the General Hospital of the PLA from January 2004 to January 2014, which was diagnosed as a brain tumor, infection or other occupying disease, or needed to be identified with a tumor. Results: a total of 16 patients were enrolled in the group, including 5 women and 11 men with a median age of 40. Most patients had acute or subacute onset, and the median of the time interval of the first clinical symptoms was 3.2 weeks; 75% of the patients were the first course before the operation, the 12.5% operation was remission, and 12.5% before the operation. During a median follow-up of 2.5 years, 50% patients maintained a single time course, 25% had a relapse and 25% were still aggravated, and 81.3% of the patients were misdiagnosed before operation (such as brain tumors, brain abscesses, cerebral hemorrhage or parasitic infection), and the patients were lack of specificity, mainly including 56.3% limb weakness, 43.8 % headache, 37.5% sensory disorders and 31.3% dizziness; 68.8% of the patients were single intracranial lesions and 31.3% intracranial lesions were more than 3; 81.3% of the patients were located in the cortex and subcortex, 43.8% were located in the inner capsule and basal ganglia, 37.5% in the lateral ventricle, 31.3% involved in the thalamus and 18.8% in the subtentorium; the size of all lesions was 3~8 cm, median and four. The quantile was 7.3 (5.4,7.6) cm, the size of the lesion was 1.4 to 4.4cm, the median and four digit was 3.3 (2.5,3.6) cm; 81.3% of the patients had moderate to severe edema and occupying effect; all the patients were enhanced after enhanced scan, including edge or ring enhancement 50%, pia mater 43.8%, cerebral gyrus The nodular enhancement was 25%, and the necrosis, macrophage, astrocyte proliferation, infiltration of vascular wall inflammatory cells (50% lymphocytic vasculitis, 37.5% acute necrotizing vasculitis, 12.5% granulomatous vasculitis), 87.5% of patients with perivascular inflammatory cells, and 62.5% secondary myelin sheath were found under the microscope. Partial loss of.7 patients received methylprednisolone therapy in the course of disease, but only 2 cases were effective and 4 were still progressing; 3 patients were treated with cyclophosphamide, only 1 were effective. The overall prognosis was slightly better than before operation: the median and four quantiles of the preoperative Rankin score were 2 (1,3), and the last visit was 1.5 (0,4). Conclusion: primary central nervous system The clinical manifestations of vasculitis are lack of specificity. The conventional MRI and pathological changes are overlapped with the tumor like demyelinating. The two cases are difficult to identify. However, the primary central nervous system vasculitis is mostly involved in the cortex and subcortex, and the edema and the position of the tumor are more obvious than that of the tumor like demyelination. It is suggested that both angiograms or MRA examination should be performed at the same time, and the pathological specimens should include the pial, cortical and subcortical tissues at the same time. Objective: To summarize the general and clinical features of the tumor like demyelinating disease. Methods: to be treated at the General Hospital of the Liberation Army from January 2006 to January 2014, and to be operated on for being misdiagnosed as a brain tumor or A retrospective study was conducted for the patients with tumor like demyelinating disease with tumor identification and biopsy. The general epidemiological characteristics, clinical manifestations, misdiagnosis, disease development, treatment and prognosis were analyzed. Results: 17 patients were enrolled in the group, of which 9 were female, 8 men were male, the median age of onset was 47, and 82.4% of the patients were preoperatively. The patients were misdiagnosed as intracranial tumor, abscess or parasitic infection, and 17.6% of the patients were diagnosed with the need to be identified with the tumor. The patients with multiple acute or subacute onset, the median of the time interval of the first appearance of the clinical symptoms were 5.3 weeks; 82.4% of the patients were first onset before operation, and the condition of 11.8% was slow before operation. After 5.9%, the disease was progressively aggravated. After a median of 4.2 years of follow-up, 70.6% of the patients had a single time course, 17.6% were remission, 11.8% were still aggravated, and the clinical symptoms were varied, including 41.2% limb weakness, 23.5% eyes blurred, 23.5% swallowing choking cough or diplopia; 8 The patients received methylprednisolone therapy after operation, but only 4 cases were effective. The other 9 cases were not treated with any specific treatment, but the clinical and image were gradually improved and did not relapse. At the last follow-up, 64.7% patients were diagnosed as clinical isolated syndrome, 11.8% were diagnosed as MS, and 23.5% could not be categorized in the known demyelinating disease; EDSS evaluation before operation. The median of the scores was 2 and the last follow-up was 0. Conclusion: the clinical manifestations of the tumor like demyelinating disease are multiple acute or subacute, and the clinical manifestations are different. Most patients have a single phase course, and a few can relieve the recurrence or progressive development. Some patients can be naturally relieved after the resection of the focus. Hormone therapy is only effective for some patients, this disease is pretreated The later is relatively good.
【学位授予单位】：中国人民解放军医学院
【学位级别】：博士
【学位授予年份】：2015
【分类号】：R744.5

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