CAJ | 학술논문

目的：总结经病理证实的58例颅内肿瘤样脱髓鞘病（ TDL）发病不同时期的临床、影像及病理特点以减少误诊率。方法收集2005—2013年在海军总医院就诊，并经病理证实的58例TDL的临床、影像及病理资料进行回顾性分析，病理切片采用苏木精伊红染色（ HE）、髓鞘染色[神经髓鞘固蓝染色法（ LFB）、过碘酸－雪夫染色（ PAS）或髓磷脂碱性蛋白（ MBP）的免疫组织化学染色]和吞噬细胞特异抗体染色（KiM1P或CD68免疫组织化学染色）以及轴索染色[神经微丝蛋白（NF）免疫组化染色]，综合对TDL病灶组织进行病理观察。结果58例病例中男31例，女27例，发病年龄6～56（36±13）岁。急性期21例（36％），亚急性期27例（46．5％），慢性期10例（17．5％）。2例患者为2次活检证实（3．4％）。急性期病理特点为大量神经髓鞘脱失而轴索相对保留，炎细胞（以吞噬细胞为主）大量浸润，反应性星形胶质细胞增多；慢性活动期病灶病理特点为相对的轴索保留，病灶与周围组织界限清楚。吞噬了髓鞘的吞噬细胞主要集中在病灶边缘，其细胞数较急性期减少，且处于非活动期，纤维型星形胶质细胞增多。结论 TDL为特殊类型的脱髓鞘病，虽影像上貌似肿瘤，但其病灶多为双侧且彼此孤立，急性期与慢性期病理特点改变有差异。其病理演变特点对TDL早期诊断与鉴别诊断的临床价值显著，有助于与脑肿瘤或中枢神经系统血管炎相鉴别。
목적：총결경병리증실적58례로내종류양탈수초병（ TDL）발병불동시기적림상、영상급병리특점이감소오진솔。방법수집2005—2013년재해군총의원취진，병경병리증실적58례TDL적림상、영상급병리자료진행회고성분석，병리절편채용소목정이홍염색（ HE）、수초염색[신경수초고람염색법（ LFB）、과전산－설부염색（ PAS）혹수린지감성단백（ MBP）적면역조직화학염색]화탄서세포특이항체염색（KiM1P혹CD68면역조직화학염색）이급축색염색[신경미사단백（NF）면역조화염색]，종합대TDL병조조직진행병리관찰。결과58례병례중남31례，녀27례，발병년령6～56（36±13）세。급성기21례（36％），아급성기27례（46．5％），만성기10례（17．5％）。2례환자위2차활검증실（3．4％）。급성기병리특점위대량신경수초탈실이축색상대보류，염세포（이탄서세포위주）대량침윤，반응성성형효질세포증다；만성활동기병조병리특점위상대적축색보류，병조여주위조직계한청초。탄서료수초적탄서세포주요집중재병조변연，기세포수교급성기감소，차처우비활동기，섬유형성형효질세포증다。결론 TDL위특수류형적탈수초병，수영상상모사종류，단기병조다위쌍측차피차고립，급성기여만성기병리특점개변유차이。기병리연변특점대TDL조기진단여감별진단적림상개치현저，유조우여뇌종류혹중추신경계통혈관염상감별。
Objective To summarize the clinical features, neuroimaging findings and pathological characteristics of pathologically confirmed tumefactive demyelinating lesions (TDL).Methods The clinical features, neuroimaging findings and pathological characteristics were retrospectively collected and analyzed for 58 patients with pathologically confirmed TDLs.For pathological studies, a combination of hematoxylin and eosin staining, myelin staining ( Luxol fast blue/periodic acid-Schiff or immunohistochemistry for myelin basic protein), macrophage-specific marker (immunohistochemistry for KiM1P or CD68) and staining for axons ( Bielschowski silver impregnation or immunohistochemistry for neurofilament protein ) were employed.Results The mean age of onset was 6 -56(36 ±13) years.The onsets were acute (n=21, 36%), subacute (n=27, 46.5%) and chronic (n=10, 17.5%).The diagnoses of TDL were confirmed by repeat biopsy and pathological examinations (n=2, 3.4%).In acute phase, the plaques of lesions were characterized by massive demyelination with relatively axonal preservation associated with prominent reactive astrocytosis and profound infiltrates of macrophages.In plaques of chronic lesions, demyelinated lesions with relative axonal preservation and sharply defined margins were major findings.And myelin-laden macrophages accumulated at the edges of plaques and stayed relatively inactive with densely gliotic center and processbearing astrocytosis.Conclusion TDL is a distinct entity of demyelinating disease.Even though it is often misdiagnosed as neoplasm in brain, bilateral brain involvements and multiple lesions are more common in TDL.The pathological features of TDL are important for the early diagnosis of this disease and helpful for differentiating with brain tumors and central nervous system vasculitis.