鼻腔孤立性纤维瘤/血管外皮细胞瘤1例

患者女,48岁,以“左侧鼻塞伴间歇性头痛,左眼突出1年”入院。查体:左眼球轻度突出,活动可,鼻中隔明显右偏,左鼻腔见粉红色新生物,质中,表面尚光滑。CT:左鼻腔见约5.60 cm×6.80 cm×4.90 cm不规则软组织肿块,CT值约60 HU,呈膨胀性生长,边界欠清,双侧上颌骨、眼眶内壁、筛骨及蝶骨骨质受压推移、吸收变薄,并见局部骨质破坏(图1A),增强后病灶不均匀强化;CTA可见供血动脉为左侧上颌窦动脉(图1B);诊断为左侧鼻腔及副鼻窦巨大占位,考虑肉瘤类病变。MRI:左鼻腔见不规则分叶状软组织信号,最大直径约7.40 cm,T1WI呈不均匀低信号,T2WI病灶前半部分为稍高信号、后半部分为低信号(图1C),DWI呈不均匀高信号,邻近眶壁及斜坡骨质明显破坏;诊断为左侧鼻腔及副鼻窦巨大肿块,邻近骨质明显破坏并侵犯颅内,考虑富含纤维的恶性肿瘤。行鼻内窥镜下鼻内病损切除术。术后病理诊断:(鼻腔)孤立性纤维瘤/血管外皮细胞瘤(solitary fibrous tumor/hemangiopericytoma,SFT/HPC)(图1D)。免疫组织化学:CD34(+),Bcl-2(+),Vimentin(+),SMA(灶+),Ki-67(约4%+)。     

DWI联合最小ADC值对鉴别诊断颅内孤立性纤维性肿瘤/血管外皮细胞瘤与脑膜瘤的价值

目的用扩散加权成像(diffusion weighted imaging,DWI)联合最小表观扩散系数(minimum apparent diffusion coefficient,MinADC)值鉴别诊断颅内孤立性纤维性肿瘤/血管外皮细胞瘤(solitary fibrous tumor/hemangiopericytoma,SFT/HPC)与脑膜瘤,以提高术前诊断准确率。材料与方法回顾性分析2012年3月至2018年8月我院经手术病理证实的颅内10例SFT/HPC (WHOⅡ级)患者与22例脑膜瘤(WHOⅡ级)患者的术前临床资料、DWI特征及病理资料,在ADC图上测量两者的MinADC值并进行比较。通过Welch检验进行两组MinADC值的比较,运用受试者工作特征曲线(receiver operating characteristiccurve,ROC曲线),评价MinADC值的鉴别诊断价值。结果在10例SFT/HPC中,肿瘤实性成分在DWI上8例呈等信号,2例呈稍高信号,MinADC平均值为(1.16±0.23)×10-3 mm2/s;在22例脑膜瘤中,肿瘤实性成分在DWI上18例呈稍高信号,2例呈等信号,2例明显高信号,MinADC值平均值为(0.80±0.12)×10-3mm2/s,小于SFT/HPC,两组肿瘤实性成分MinADC值比较差异有统计学意义(P<0.01)。以MinADC值0.90×10-3 mm2/s为阈值诊断SFT/HPC与脑膜瘤,ROC曲线下面积为0.92±0.32,敏感度为97.6%,特异度为94.3%,与病理组织学结果行一致性检验,SFT/HPC的重复测量的Kappa值为0.91,脑膜瘤的Kappa值为0.87。结论 MinADC值可以作为一种量化工具区分颅内SFT/HPC与脑膜瘤,提高术前诊断准确率。     

软组织血管外皮细胞瘤与孤立性纤维性肿瘤概念的变迁

探讨WHO(2002)软组织肿瘤新分类中血管外皮细胞瘤和孤立性纤维性肿瘤概念的变化。复习近几年关于软组织血管外皮细胞瘤和孤立性纤维性肿瘤的文献,认为血管外皮细胞瘤、孤立性纤维性肿瘤、脂肪瘤样血管外皮细胞瘤和巨细胞性血管纤维瘤是关系非常密切的肿瘤。肌外皮细胞瘤、血管球外皮细胞瘤和成人的婴儿型肌纤维瘤病也属于血管外皮细胞瘤。     

颅内孤立性纤维瘤/血管外皮瘤与脑膜瘤的临床和MRI对比分析

目的 探讨颅内孤立性纤维瘤（SFT）/血管外皮瘤（HPC）MRI影像征象,并与脑膜瘤进行对比分析。方法 分析经手术和病理证实的SFT/HPC患者28例（SFT/HPC组）和脑膜瘤患者68例（脑膜瘤组）的临床及MRI征象,并比较各观察指标。结果 肿瘤形态、信号均匀性、瘤内血管流空信号、瘤内短T2信号强化结节、瘤内囊变坏死情况、脑膜尾征、骨质改变、性别、Ki-67%水平、术中出血量在两组间差异均有统计学意义（P均<0.05）。结论 SFT/HPC及脑膜瘤临床及MRI表现存在一定差异,对比分析两者的影像征象有助于两者的鉴别诊断。     

中枢神经系统血管外皮细胞瘤诊断与治疗

目的：探讨中枢神经系统血管外皮细胞瘤的诊断及治疗.方法：回顾性分析1988年以来四川大学华西医院收治的15例中枢神经系统血管外皮细胞瘤.结果：9例以头痛为首发症状,12例首次发病者全部误诊,3例再发者中进行化疗的两例术后6、7年转移或复发,未进行化疗的1例术后2、6、7年复发3次.结论：其多以头痛为首发和主要症状,较难诊断,多需病理检查才能最终确诊,手术全切为其最佳选择,术后放疗明显改善预后.     

颅内血管周细胞瘤与孤立性纤维瘤的影像与病理对照

目的对比分析颅内血管周细胞瘤与孤立性纤维瘤的影像学差异。材料与方法回顾性分析经手术病理证实的颅内血管周细胞瘤18例,孤立性纤维瘤10例,所有病例均行MR平扫及增强扫描,对其影像征象进行对照分析。统计学方法采用单因素分析,计数资料组间比较采用卡方检验。结果血管周细胞瘤与孤立性纤维瘤在与附着硬膜的关系、硬膜尾征征象方面无统计学意义(P＞0.05);在肿瘤形态、T2WI信号、囊变坏死及出血、瘤周水肿、骨质破坏及强化方式方面差异有统计学意义(P＜0.05)。前者肿瘤形态多呈分叶状、不规则形, T2WI等高信号,囊变坏死及出血多见,瘤周水肿较明显,颅骨破坏可见,增强后明显强化;后者肿瘤形态多呈类圆形或椭圆形,T2WI有低信号区,囊变坏死及出血少见,瘤周水肿轻,无颅骨破坏,增强后明显强化,并有延迟强化。结论颅内血管周细胞瘤与孤立性纤维瘤影像表现存在一定差异。     

颅内血管外皮细胞瘤7例MRI诊断与鉴别分析

目的 探讨颅内血管外皮细胞瘤(HPC)的影像表现,提高影像学认知水平.方法 回顾性分析7例HPC的影像学特点,并结合文献进行复习.结果 HPC好发于硬脑膜附近,广基底与其相连.类圆形、分叶形或不规则形脑外占位性病变,界清,等T1、等T2信号或混杂信号,可伴有囊变、坏死,周围有流空血管影及水肿,增强扫描明显强化.结论 HPC的MRI表现具有一定特异性,结合多种检查方法有助于提高其诊断水平.     

盆腔巨大型孤立性纤维瘤1例

患者,男,25岁,因车祸入院,行超声检查发现盆腔巨大占位.MRI平扫示盆腔内前列腺右前侧巨大的椭圆形稍长T1稍长T2异常信号,大小约9 cm×12 cm×12 cm,境界清晰,信号均匀,内可见血管流空信号影,增强扫描肿块明显均匀强化(图1、2),膀胱及前列腺明显受压变扁,膀胱向左上移位,邻近腰大肌及骨质信号未见明显异常;IVP示盆腔隐约可见巨大椭圆形占位,膀胱受压明显上移,左侧输尿管轻度扩张;CT平扫示盆腔内巨大软组织块影,密度均匀,境界尚清晰,膀胱明显受压向左上方移位(图3).影像诊断为间叶组织起源恶性肿瘤可能.     

Clinical characteristics and prognosis of orbital solitary fibrous tumor in patients from a Chinese tertiary eye hospital

BACKGROUNDSolitary fibrous tumor (SFT) is predominant within the pleura but very rare in the orbit, which is why the diagnosis of orbital SFT poses challenges in clinical practice. Accordingly, an integrated approach that incorporates specific clinical features, histological, histopathological, and immunohistochemical (IHC) examinations, and molecular analyses is warranted.AIMTo retrospectively explore the clinical and imaging characteristics, treatment, outcomes of a series of patients with orbital SFT.METHODSWe conducted a retrospective review of a series of patients diagnosed with a histopathologic orbital SFT treated at a single institution. All data on demogra/phics, clinical characteristics, imaging, treatment, postoperative histopathological and IHC examinations, and prognosis were collected. RESULTSIn total, 13 patients were enrolled, 7 (53.8%) of whom had the tumor located in the superomedial quadrant of the orbit. Computed tomography revealed a solitary ovoid lesion in 10 (76.9%) patients and irregular lesion in 3 (23.1%) patients. Magnetic resonance imaging results were as follows: On T1 weighted images, 3 (23.1%) patients had hypointense mixed signals, whereas 10 (76.9%) patients showed isointense mixed signals; on T2 weighted images (T2WI), 3 (23.1%), 4 (30.8%), and 6 (46.2%) patients exhibited hypointense mixed, isointense mixed, and hyperintense signals, respectively. Notably, 12 (92.3%) patients showed significant enhancement, whereas there were patchy slightly enhanced areas in the tumor. All patients were treated by surgery. IHC analysis demonstrated that the tumor cells were immunoreactive for CD34, CD99, STAT-6, and vimentin in all patients. The lesions showed Ki-67 positivity < 5% in 1 (7.7) patient, 5%-10% in 10 (76.9%), and > 10% in 2 (15.4%). Two (15.4%) patients exhibited tumor recurrence. CONCLUSIONThe clinical manifestations and radiologic characteristics of orbital SFT are diverse and not specific. Accurate diagnosis and treatment require detailed radiological and histopathological/IHC evaluation.     

中枢神经系统孤立性纤维瘤的影像学表现

目的 探讨中枢神经系统孤立性纤维瘤（SFT）的影像学特征。方法 回顾性分析9例经手术及病理证实的SFT影像学资料,并与病理学结果进行对照分析。结果 ①9例SFT均为单发病灶,发生于幕上者5例,幕下1例,枕部中线跨天幕1例、伴颈椎管种植转移,颈椎管、右侧脑室体部各1例。肿瘤边界清楚,椭圆形或类圆形6例,不规则形3例;2例可见分叶征,病灶最大径15.26~78.13 mm。②4例CT平扫为略高密度,2例密度均匀,2例不均匀,1例瘤内伴灶性坏死,1例合并出血。③与脑实质对比,6例T1WI呈等信号为主的不均匀信号,2例呈均匀等信号;6例T2WI为低、等（或略高）混杂信号,2例为均匀等或略高信号。④增强扫描病变不均匀强化5例,均匀强化2例;3例可见“脑膜尾征”;2例见血管流空。⑤镜下显示肿瘤由疏密不均的梭性细胞、致密胶原纤维以及大量薄壁血管组成。结论 中枢神经系统SFT的MRI表现具有一定特征性,与临床病理有密切的内在联系,是诊断SFT有价值的检查方法。     

Malignant solitary fibrous tumor of the greater omentum: A case report and review of literature

BACKGROUNDMalignant solitary fibrous tumors (SFTs) account for 15%-20% of all SFTs, and malignant SFTs arising from the greater omentum are extremely rare. Most malignant SFTs of the greater omentum are diagnosed via pathological examinations after surgery. In this study, we report a case of malignant omental SFT and review the published literature on this rare malignancy.CASE SUMMARYA 64-year-old female presented with an abdominal mass, and underwent exploratory surgery, during which a huge tumor originating from the greater omentum and intraperitoneal implants were identified and resected. The results of the pathological examination, immunohistochemistry staining, and gene sequencing led to the diagnosis of malignant SFT of the greater omentum. The patient died one and a half years later due to tumor recurrence and metastasis.CONCLUSIONThis is the first report of the application of gene sequencing in the diagnosis of malignant SFTs of the greater omentum.     

左心室及心包恶性孤立性纤维性肿瘤1例

患者男,24岁,因发热,上腹部不适9天,胸闷、气促5天来诊。查体:胸骨左缘3~4肋间可闻及Ⅱ/6级收缩期杂音。MRI检查:升主动脉右侧心包内占位;双侧胸腔积液。超声见:左室流出道内等回声团,约32mm×22mm,其蒂似附着于主动脉瓣无冠瓣,瘤体随心脏舒缩在左室流出道及主动脉根部作往复活