【摘要】：目的：分析腫瘤樣脫髓鞘患者的影像學(xué)(MRI)特征。方法：對2006年1月至2014年1月期間就診于解放軍總醫(yī)院、因被誤診為腦腫瘤等占位性疾病而行手術(shù)或者需同腫瘤相鑒別而行活檢的腫瘤樣脫髓鞘病患者的影像學(xué)資料(MRI)進(jìn)行回顧性分析,將以下影像學(xué)參數(shù),包括病灶數(shù)量、位置、大小、水腫及占位效應(yīng)及強(qiáng)化特點(diǎn)進(jìn)行分析,并將末次隨訪時病灶數(shù)量與術(shù)前進(jìn)行對比。結(jié)果：47.1%的腫瘤樣脫髓鞘患者只有1個顱內(nèi)病灶,52.9%的患者顱內(nèi)病灶數(shù)量≥3個(其中2例合并出現(xiàn)脊髓病灶)；52.9%的患者病灶位于皮層下或深部白質(zhì)、47.1%位于例腦室旁、35.3%位于近皮層或皮層；82.4%的患者病灶周圍有不同程度的水腫及占位效應(yīng)；所有病灶中,直徑最小者為2.6cm,最大者為11.5cm,中位數(shù)及四分位數(shù)為4.8(3.5,5.9)cm,去除病灶周圍水腫后,病灶大小范圍為1.4-5.1cm,中位數(shù)及四分位數(shù)為3.4(2.7,3.7)cm；82.4%的患者增強(qiáng)掃描后出現(xiàn)強(qiáng)化,其中邊緣或環(huán)形強(qiáng)化占50%,斑片狀強(qiáng)化占35.7%,21.4%的患者出現(xiàn)血管樣強(qiáng)化；經(jīng)過中位數(shù)為4.2年的隨訪,8例單病灶患者中有7例未出現(xiàn)新病灶,原有病灶呈術(shù)后改變,9例多病灶患者中5例未出現(xiàn)新發(fā)病灶,4例病灶數(shù)量較前增加。結(jié)論：腫瘤樣脫髓鞘病灶可單發(fā),也可以多發(fā);病灶直徑往往大于2cm,且多具有水腫及占位效應(yīng)；病灶位置以白質(zhì)為主,但皮層灰質(zhì)亦可受累；增強(qiáng)掃描后病灶多呈環(huán)形、斑片狀及血管樣強(qiáng)化；無論采用激素治療與否,腫瘤樣脫髓鞘病灶術(shù)后可減小或消失,少數(shù)患者即使經(jīng)激素治療后病灶仍增大或數(shù)量增加。目的：分析腫瘤樣脫髓鞘病灶的組織病理學(xué)特點(diǎn)。方法：對2006年1月至2014年1月期間就診于解放軍總醫(yī)院,因被誤診為腦腫瘤等占位性疾病而行手術(shù)或者需同腫瘤相鑒別而行活檢的腫瘤樣脫髓鞘病患者進(jìn)行回顧性研究,收集其術(shù)后病理切片或石蠟組織塊,并對以下病理學(xué)參數(shù)進(jìn)行統(tǒng)計分析：病灶邊界、炎癥反應(yīng)分布位置、炎癥細(xì)胞類型,有無血管周圍淋巴套袖,有無髓鞘脫失及軸索相對保留、星形膠質(zhì)細(xì)胞增生情況,有無組織壞死、血管增生或血管壁炎性浸潤。對于部分行開顱病灶切除術(shù)的患者,根據(jù)其詳細(xì)的手術(shù)記錄分析其術(shù)中肉眼所見及術(shù)中冰凍病理結(jié)果。結(jié)果：35.3%的病灶與周圍腦組織邊界清楚；全部患者存在活動性脫髓鞘反應(yīng)過程,鏡下可見：腦實(shí)質(zhì)炎癥細(xì)胞浸潤,髓鞘脫失與軸索相對保留。100%的患者病灶內(nèi)巨噬細(xì)胞增生,其中52.9%的患者內(nèi)含髓鞘吞噬碎片；88.2%的患者可見星形膠質(zhì)細(xì)胞增生,其中29.4%可見Creutzfeldt細(xì)胞；76.5%的患者存在血管周圍淋巴細(xì)胞套,47.1%存在毛細(xì)血管增生,17.6%的患者血管壁有炎癥細(xì)胞浸潤,35.3%存在組織結(jié)構(gòu)分解,17.6%可見組織壞死。6例行開顱切除術(shù)的患者術(shù)中病理切片均誤診為“低級別膠質(zhì)瘤”結(jié)論：TDLs病理學(xué)示非特異性的炎性脫髓鞘改變,組織內(nèi)可見壞死及血管壁炎癥反應(yīng)。即使是病理學(xué)資料(特別是冰凍病理切片),在診斷TDLs時也經(jīng)常誤診；TDLs內(nèi)常因?yàn)樾切文z質(zhì)細(xì)胞大量增生,有時是異形性增生而易被誤診為膠質(zhì)細(xì)胞瘤,故建議在病理學(xué)鑒別炎性脫髓鞘與膠質(zhì)瘤時,將LFB-PAS、CD68染色作為常規(guī)操作。目的：總結(jié)經(jīng)病理證實(shí)的原發(fā)性中樞神經(jīng)系統(tǒng)血管炎的臨床、影像、病理特征及與腫瘤樣脫髓鞘病的異同點(diǎn)。方法：回顧性分析2004年1月至2014年1月期間就診于解放軍總醫(yī)院,因誤診為腦腫瘤、感染或其他占位性疾病而行手術(shù)或需與腫瘤相鑒別而行活檢的原發(fā)性中樞神經(jīng)系統(tǒng)血管炎患者的臨床、影像及病理特點(diǎn),并與經(jīng)病理證實(shí)的腫瘤樣脫髓鞘病相對比。結(jié)果：共有16例患者入組,其中女性5例,男性11例,發(fā)病年齡中位數(shù)為40歲。大多數(shù)患者急性或亞急性起病,手術(shù)距首次出現(xiàn)臨床癥狀的時間間隔中位數(shù)為3.2周；75%的患者術(shù)前為首次病程,12.5%術(shù)前病情有緩解復(fù)發(fā),12.5%術(shù)前呈進(jìn)展趨勢；在中位數(shù)為2.5年的隨訪中,50%患者術(shù)后保持單時相病程,25%呈緩解復(fù)發(fā)趨勢,25%術(shù)后病情仍進(jìn)行性加重；81.3%的患者術(shù)前曾被誤診(如腦腫瘤、腦膿腫、腦出血或寄生蟲感染)；患者臨床表現(xiàn)缺乏特異性,主要包括56.3%肢體無力、43.8%頭痛、37.5%感覺障礙及31.3%頭昏；68.8%的患者為顱內(nèi)單發(fā)病灶,31.3%顱內(nèi)病灶數(shù)量≥3個；81.3%的患者病灶位于皮層及皮層下,43.8%位于內(nèi)囊及基底節(jié)區(qū),37.5%位于側(cè)腦室旁,31.3%累及丘腦,18.8%位于幕下；所有病灶大小范圍3～8 cm,中位數(shù)及四分位數(shù)為7.3(5.4,7.6)cm,去除病灶周圍水腫后,病灶大小范圍為1.4～4.4cm,中位數(shù)及四分位數(shù)為3.3(2.5,3.6)cm；81.3%的患者病灶有中-重度水腫及占位效應(yīng)；增強(qiáng)掃描后全部患者出現(xiàn)強(qiáng)化,其中邊緣或環(huán)形強(qiáng)化占50%,軟腦膜強(qiáng)化者占43.8%,腦回樣、結(jié)節(jié)樣強(qiáng)化各占25%；鏡下可見全部患者病灶內(nèi)：壞死,巨噬細(xì)胞、星形膠質(zhì)細(xì)胞增生,血管壁炎細(xì)胞浸潤(其中50%淋巴細(xì)胞性血管炎、37.5%急性壞死性血管炎、12.5%肉芽腫性血管炎),87.5%的患者血管周圍炎癥細(xì)胞浸潤,62.5%可見繼發(fā)性髓鞘部分脫失。7例患者病程中接受甲強(qiáng)龍沖擊治療,但只有2例有效,4例仍進(jìn)展；3例患者應(yīng)用環(huán)磷酰胺治療,只有1例有效�？傮w預(yù)后略好于術(shù)前：術(shù)前Rankin評分中位數(shù)及四分位數(shù)為2(1,3)分,末次隨訪時為1.5(0,4)分。結(jié)論：原發(fā)性中樞神經(jīng)系統(tǒng)血管炎臨床表現(xiàn)缺少特異性,其常規(guī)核磁檢查及病理改變均與腫瘤樣脫髓鞘有重疊之處,二者很難鑒別。但總體上原發(fā)性中樞神經(jīng)系統(tǒng)血管炎病灶多同時累及皮層及皮層下,且水腫及占位較腫瘤樣脫髓鞘更加明顯。若常規(guī)核磁及病理均不能鑒別兩者,建議同時行血管造影或MRA檢查,且病理標(biāo)本應(yīng)同時包括軟腦膜、皮層及皮層下組織。目的：總結(jié)腫瘤樣脫髓鞘病的一般及臨床特點(diǎn)。方法：對2006年1月至2014年1月期間就診于解放軍總醫(yī)院,因被誤診為腦腫瘤而行手術(shù)或者需同腫瘤相鑒別而行活檢的腫瘤樣脫髓鞘病患者進(jìn)行回顧性研究,對一般流行病學(xué)特點(diǎn)、臨床表現(xiàn)、誤診情況、病情發(fā)展特點(diǎn)、治療及預(yù)后進(jìn)行分析。結(jié)果：共有17例患者入組,其中女性9名,男性8名,發(fā)病年齡中位數(shù)為47歲；術(shù)前有82.4%的患者被誤診為顱內(nèi)腫瘤、膿腫或寄生蟲感染等占位性疾病,17.6%的患者因需要同腫瘤相鑒別而行活檢手術(shù)；患者多急性或亞急性起病,手術(shù)距離首次出現(xiàn)臨床癥狀的時間間隔中位數(shù)為5.3周；82.4%的患者術(shù)前為首次發(fā)病,11.8%術(shù)前病情有緩解復(fù)發(fā),5.9%術(shù)前病情呈進(jìn)行性加重,經(jīng)過中位數(shù)為4.2年的隨訪,術(shù)后有70.6%的患者呈單時相病程,17.6%病情有緩解復(fù)發(fā),11.8%病情仍進(jìn)行性加重；臨床癥狀多種多樣,主要包括41.2%肢體無力、23.5%視物模糊、23.5%吞咽嗆咳或復(fù)視等腦干癥狀；8例患者術(shù)后接受甲強(qiáng)龍沖擊治療,但只有4例有效,另外9例術(shù)后未行任何針對性治療,但臨床及影像均逐漸好轉(zhuǎn)且未再復(fù)發(fā)；末次隨訪時64.7%患者診斷為臨床孤立綜合征,11.8%診斷為MS,另有23.5%不能明確歸類于已知脫髓鞘��；術(shù)前EDSS評分中位數(shù)為2分,末次隨訪時為0分。結(jié)論：腫瘤樣脫髓鞘病多急性或亞急性起病,其臨床表現(xiàn)差異性較大。大多數(shù)患者呈單時相病程,少數(shù)可緩解復(fù)發(fā)或進(jìn)行性發(fā)展。部分患者于病灶切除后可自然緩解,激素治療只對部分患者有效,本病預(yù)后相對良好。
[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.
【學(xué)位授予單位】：中國人民解放軍醫(yī)學(xué)院
【學(xué)位級別】：博士
【學(xué)位授予年份】：2015
【分類號】：R744.5

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