弥漫性原浆型星形细胞瘤

每日病例读片主读：李志宇2014.12.15上海市东方医院南院影像科11/10/2019影像号:M13060280姓名:吴万忠性别:男年龄:45岁检查日期：2014-10-1911/10/2019症状及体征：十年前体检发现右侧额叶病灶，无症状；本次入院前两周突发意识丧失、四肢抽搐，持续5分钟后缓解。11/10/2019WuWanZhong,WuWanZhong,1436060717945411436060717945411969-8-221969-8-2245年45年MM页：9之16页：9之16AQM:205\256AQM:205\256TE:1.92TE:1.92TR:116.08TR:116.08FA:80FA:80GRGREC:1EC:1NEX:2NEX:2SENSE-NV-16SENSE-NV-16SHEastHospital(southcampus)SHEastHospital(southcampus)H+CH+CT1W_FFET1W_FFE2014-10-1916:31:002014-10-1916:31:00M13060280M13060280------LOC:64LOC:64THK:7SP:8THK:7SP:8HFSHFSIM:9SE:601IM:9SE:601DFOV:23x23cmDFOV:23x23cmW:1651W:1651C:1066C:1066Z:2Z:2RRLLAAPPcmcmWuWanZhong,WuWanZhong,1436060717945411436060717945411969-8-221969-8-2245年45年MM页：9之16页：9之16AQM:309\420AQM:309\420TE:100TE:100TR:3500TR:3500FA:90FA:90SESEEC:1EC:1NEX:1NEX:1SENSE-NV-16SENSE-NV-16SHEastHospital(southcampus)SHEastHospital(southcampus)H+CH+CT2W_TSET2W_TSE2014-10-1916:30:092014-10-1916:30:09M13060280M13060280------LOC:65.60LOC:65.60THK:7SP:8.20THK:7SP:8.20HFSHFSIM:9SE:501IM:9SE:501DFOV:23x23cmDFOV:23x23cmW:1847W:1847C:1087C:1087Z:1Z:1RRLLAAPPcmcmWuWanZhong,WuWanZhong,1436060717945411436060717945411969-8-221969-8-2245年45年MM页：10之16页：10之16AQM:194\256AQM:194\256TE:140TE:140TR:10000TR:10000FA:90FA:90IRIREC:1EC:1NEX:1NEX:1SENSE-NV-16SENSE-NV-16SHEastHospital(southcampus)SHEastHospital(southcampus)H+CH+CFLAIR_longTRFLAIR_longTR2014-10-1916:31:422014-10-1916:31:42M13060280M13060280------LOC:72LOC:72THK:7SP:8THK:7SP:8HFSHFSIM:10SE:701IM:10SE:701DFOV:23x23cmDFOV:23x23cmW:1816W:1816C:1044C:1044Z:2Z:2RRLLAAPPcmcmWuWanZhong,WuWanZhong,1436060717945411436060717945411969-8-221969-8-2245年45年MM页：25之32页：25之32AQM:152\106AQM:152\106TE:102.12TE:102.12TR:2448.25TR:2448.25FA:90FA:90SESEEC:1EC:1NEX:1NEX:1SENSE-NV-16SENSE-NV-16SHEastHospital(southcampus)SHEastHospital(southcampus)H+CH+CDWIDWI2014-10-1916:29:202014-10-1916:29:20M13060280M13060280------LOC:65.60LOC:65.60THK:7SP:8.20THK:7SP:8.20HFSHFSIM:121SE:401IM:121SE:401DFOV:23x23cmDFOV:23x23cmW:442W:442C:278C:278Z:2Z:2RRLLAAPPcmcm11/10/2019WuWanZhong,WuWanZhong,1436060717945411436060717945411969-8-221969-8-2245年45年MM页：9之16页：9之16AQM:205\256AQM:205\256TE:1.92TE:1.92TR:116.08TR:116.08FA:80FA:80GRGREC:1EC:1NEX:2NEX:2SENSE-NV-16SENSE-NV-16SHEastHospital(southcampus)SHEastHospital(southcampus)H+CH+CT1W_FFET1W_FFE2014-10-1916:31:002014-10-1916:31:00M13060280M13060280------LOC:64LOC:64THK:7SP:8THK:7SP:8HFSHFSIM:9SE:601IM:9SE:601DFOV:23x23cmDFOV:23x23cmW:1651W:1651C:1066C:1066Z:2Z:2RRLLAAPPcmcmWuWanZhong,WuWanZhong,1436060717945411436060717945411969-8-221969-8-2245年45年MM页：9之16页：9之16AQM:205\256AQM:205\256TE:1.77TE:1.77TR:508.36TR:508.36FA:80FA:80GRGREC:1EC:1NEX:1NEX:1SENSE-NV-16SENSE-NV-16SHEastHospital(southcampus)SHEastHospital(southcampus)H+CH+CT1W_FFE//FS+CT1W_FFE//FS+C2014-10-1916:34:262014-10-1916:34:26M13060280M13060280------LOC:64LOC:64THK:7SP:8THK:7SP:8HFSHFSIM:9SE:801IM:9SE:801DFOV:23x23cmDFOV:23x23cmW:1793W:1793C:844C:844Z:2Z:2RRLLAAPPcmcmWuWanZhong,WuWanZhong,1436060717945411436060717945411969-8-221969-8-2245年45年MM页：5之16页：5之16AQM:187\232AQM:187\232TE:1.74TE:1.74TR:508.36TR:508.36FA:80FA:80GRGREC:1EC:1NEX:1NEX:1SENSE-NV-16SENSE-NV-16SHEastHospital(southcampus)SHEastHospital(southcampus)H+CH+CT1W_FFE//FS+CT1W_FFE//FS+C2014-10-1916:37:542014-10-1916:37:54M13060280M13060280------LOC:32LOC:32THK:7SP:8THK:7SP:8HFSHFSIM:5SE:1001IM:5SE:1001DFOV:21x21cmDFOV:21x21cmW:1642W:1642C:988C:988Z:2.13Z:2.13RRLLHHFFcmcmWuWanZhong,WuWanZhong,1436060717945411436060717945411969-8-221969-8-2245年45年MM页：10之16页：10之16AQM:205\256AQM:205\256TE:1.75TE:1.75TR:508.98TR:508.98FA:80FA:80GRGREC:1EC:1NEX:1NEX:1SENSE-NV-16SENSE-NV-16SHEastHospital(southcampus)SHEastHospital(southcampus)H+CH+CT1W_FFE//FS+CT1W_FFE//FS+C2014-10-1916:35:562014-10-1916:35:56M13060280M13060280------LOC:63LOC:63THK:6SP:7THK:6SP:7HFSHFSIM:10SE:901IM:10SE:901DFOV:23x23cmDFOV:23x23cmW:1370W:1370C:877C:877Z:2Z:2AAPPHHFFcmcm11/10/2019•MRI表现：右额叶可见斑片状异常信号影，病灶大小约为36*29mm，T1WI呈稍低信号，T2WI及FLAIR呈不均匀高信号，增强后病灶轻度强化，病灶周围可见斑片状T1WI稍低、T2WI高信号水肿带，注入对比剂后无强化。右侧脑室额角明显受压变形，余诸脑室大小、形态未见明显异常，诸脑沟、脑池未见明显扩大，中线结构居中。•诊断：11/10/2019病理弥漫性原浆型星形细胞瘤Ⅱ级11/10/2019星形细胞瘤2007年WHo中枢神经系统肿瘤最新分级：•WHOI级包括毛细胞型星形细胞瘤和室管膜下巨细胞星形细胞瘤；•WHOII级包括弥漫性星形细胞瘤和多形性黄色星形细胞瘤，其中弥漫性星形细胞瘤又可分为纤维型，原浆型和肥胖型3种；•WH0Ⅲ级包括间变性星形细胞瘤；•WHOIV级包括胶质母细胞瘤。11/10/2019•弥漫性星形细胞瘤分化程度好，但呈浸润性缓慢生长，占星形细胞来源肿瘤的10％～15％；•发病高峰年龄是30-40岁，10%发生在20岁以下，60%发生在20-45岁之间，30%超过45岁，平均年龄为34岁，男女比例为1.18:1。•这类肿瘤常有转变为间变性星形细胞瘤和胶质母细胞瘤的恶性倾向，其中位生存期为5～10年。•临床上主要表现为癫痫样抽搐，以及头痛、头晕、呕吐症状为主，这与肿瘤的浸润生长及颅内压升高有关。11/10/2019•大体病理上肿瘤边界不清，受侵犯的脑结构常扩大、扭曲但无破坏，当伴有大小不等的囊变时局部可呈海绵样改变，病灶位于额叶者可侵犯至对侧。•镜下以肿瘤细胞高度分化，在脑组织中弥漫浸润生长为特点，主要以分化好的纤维型及肥胖型星形细胞分布在微囊样疏松肿瘤基质中，其细胞结构略增加。偶见核异型，缺乏核分裂，无坏死及微血管增生。其中肥胖型星形细胞瘤有恶变为更高级别间变性星形细胞瘤及胶质母细胞瘤的倾向。11/10/2019•弥漫性星形细胞瘤好发于幕上，以额叶、颞叶受累多见，其次还可发生于脑干、脊髓，丘脑、脑干、胼胝体也是好发部位，发生于小脑者少见。•有报道认为，Ⅱ级星形细胞瘤中偏良性者常位于灰质，而大多恶性进展的肿瘤位于白质内。11/10/2019CT：•平扫上病灶为低密度或低、等混合密度，边缘常不清楚，钙化和出血少见；•增强后无强化或轻度强化。11/10/2019MRI:•肿瘤大多表现为边界不清的异常信号病灶，部分边界清楚者可形成类圆形肿块样影；•T1WI呈等低信号，T2WI呈高信号，病灶内信号欠均匀，部分可伴有大小不一囊变信号影，病变周围大多无水肿，或仅伴轻中度水肿，占位效应轻。•MRI增强后病变不强化，或仅轻中度强化，表现为结节样、小片状或环形强化，伴有囊变者多表现为大小不一的不规则环形