脑膜血管周细胞瘤临床病理分析

目的:探讨脑膜血管周细胞瘤(hemangiopericytoma,HPC)的临床病理特点及鉴别诊断.方法:对9例脑膜HPC患临床病理资料进行回顾性分析和随访15~95个月,并复习相关文献.结果:脑膜HPC常见症状为头痛头昏、肿瘤压迫和颅内高压;CT/MRI检查见肿瘤与脑膜相连,增强扫描明显强化.光镜下肿瘤细胞丰富,核圆形或卵圆形,核仁不明显;间质富含"鹿角状"血管,网状纤维包绕单个瘤细胞.免疫标记瘤细胞CD34局灶阳性,PR阴性,1例EMA局灶弱阳性.7例随访15~95个月,4例术后复发,复发病例中1例术后35月腰椎转移.结论:脑膜HPC是一种少见肿瘤,确诊需依靠病理学检查.脑膜HPC影像学上与脑膜瘤相似,但其生物学行为、病理组织学及预后不同于脑膜瘤,易复发和远处转移,术后需长期随访.     

甲状腺孤立性纤维性肿瘤1例报道并文献复习

目的探讨甲状腺孤立性纤维性肿瘤的临床病理学特点。方法对1例甲状腺孤立性纤维性肿瘤进行组织学和免疫组织化学观察,并复习相关文献。结果患者女性,于甲状腺右叶触及一肿块。光镜下见肿块境界清楚,梭形及卵圆形的肿瘤细胞排列成条束状、短席纹状,其间含有胶原纤维束,肿瘤细胞间散在少量大小不一的滤泡。肿瘤细胞CD34和bcl-2均呈阳性。结论孤立性纤维性肿瘤发生于甲状腺者较为罕见,并且临床中其生物学行为仍然未能确定,有必要长期随访。     

枕骨大孔-第二颈段血管周细胞瘤1例报道并文献复习

目的:探讨血管周细胞瘤的临床病理学特点及诊断要点.方法:对1例血管周细胞瘤进行临床资料、病理形态学及免疫组化观察,并结合文献对其诊断及鉴别诊断进行探讨.结果:本例镜下观肿瘤细胞为梭形,血管丰富,形成弥漫的网状结构,鹿角状的血管将肿瘤细胞分割小叶状,瘤细胞体积较大,胞浆丰富,边界不清,核呈圆形、卵圆形,病理性核分裂相多.免疫组化染色结果显示肿瘤细胞Vimentin(+)、CD34(+)、FVIII(+)、S-100(?)、CEA(?)和GFAP(?).结论:血管周细胞瘤,非常少见,因此缺乏对其认识,从而易与其他肿瘤混淆导致误诊.提高对血管周细胞瘤的认识,对避免误诊是至关重要的.     

肾脏原发性孤立性纤维性肿瘤3例报道并文献复习

目的 探讨肾孤立性纤维性肿瘤(solitary fibrous tumor,SFT)的临床病理学特征、诊断、鉴别诊断及预后.方法 对3例罕见的肾SFT进行临床、病理组织学形态观察及免疫组化染色,并复习相关文献.结果 原发于肾脏的SFT临床与影像学检查结果缺乏特征性表现,镜下细胞形态基本为长梭形,部分为卵圆形或上皮样细胞.其主要特点为含丰富的胶原纤维和形成多量瘢痕.免疫组化染色示瘤细胞CD34和CD99均(+),BCL-2(±).结论 发生于肾脏的SFT较为罕见,因缺乏特异性的术前诊断依据而极易误诊,确诊需依赖病理学检查,免疫组化染色有助于诊断及鉴别诊断.     

恶性血管周上皮样细胞肿瘤2例并文献复习

目的 探讨恶性血管周上皮样细胞肿瘤(malignant perivascular epitheliod cell tumor,PEComa)的临床、病理特征及鉴别诊断.方法 对2例恶性PEComa进行临床、病理形态学、免疫表型及电镜观察,并复习相关文献.结果 镜下见肿瘤组织由梭形细胞及上皮样细胞构成,细胞异型性明显,细胞胞质透明或嗜酸,细胞核大,有核仁,瘤细胞由纤细的纤维结缔组织及薄壁血管将其分隔成巢状、腺泡状、片状结构,部分区域可见多核瘤巨细胞伴肿瘤坏死,核分裂多见.免疫表型:肿瘤细胞表达HMB-45、S-100、SMA及desmin;电镜下见肿瘤细胞胞质内可见高电子密度核心的内分泌小体和少许不成熟的黑色素小体.结论 恶性PEComa罕见,病理形态多样,熟悉其临床、病理形态、免疫表型及电镜下改变,有助于临床、病理医师对其正确诊断及鉴别诊断.     

双肺转移性脑膜孤立性纤维性肿瘤/血管外皮细胞瘤一例

孤立性纤维性肿瘤/血管外皮细胞瘤(SFT/HPC)占原发脑膜肿瘤的0.5%,最大特点是容易复发和转移。肺部作为全身肿瘤最易转移器官,对于肺穿刺活检病理诊断存在极大的难度。作者收集1例双肺转移性脑膜SFT/HPC患者资料,分析其临床病理学特征,为肺部转移性肿瘤诊断及鉴别诊断提供依据。     

子宫血管周上皮样细胞肿瘤2例报道及文献复习

子宫血管周上皮样细胞肿瘤（perivascular epithelioid cell tumor,PEComa）是一种少见的肿瘤,有其特有的镜下特点和免疫组化特征。现收集2例子宫PEComa病例,并结合文献探讨其诊断和鉴别诊断,以提高对该病的认识。     

右颌下血管周肌细胞瘤一例

患者女,48岁。因发现右颌下结节1年余,长大2个月,于2003年11月10日入院。患者入院前1年无明显诱因出现右颌下结节,约豌豆大小,不痛,未予处理。2个月前发现结节进行性长大。体检：右颌下扪及一直径2cm类圆形肿块,固定,表面光滑,边界尚清,无压痛,未扪及血管搏动。     

肺恶性孤立性纤维性肿瘤1例并文献复习

目的：探讨肺恶性孤立性纤维性肿瘤的临床病理特点、鉴别诊断、治疗及预后。方法对1例肺恶性孤立性纤维性肿瘤进行临床病理学分析及免疫组化SP法染色,并结合相关文献复习。结果患者女性,71岁。左肺有一肿物,7．5 cm×7 cm×5 cm大小,呈灰白色,分叶状,质硬,包膜完整。镜下显示在瘤细胞分化较好的区域,细胞多为形态温和的梭形细胞,呈束状或编织状排列,可见纤维瘢痕组织及瘤细胞围绕分支状血管呈血管外皮瘤样结构;在瘤细胞分化较差区域,瘤细胞丰富,异型性明显,可见少数瘤巨细胞,核分裂象多见(>20个/10 HPF),并见多个小坏死灶,局部瘤细胞突破包膜,向周围肺组织浸润性生长。免疫表型：CD34、CD99和vimentin 均呈弥漫强阳性, BCL-2呈弱阳性, Ki-67增殖指数>20%。术后随访16个月无复发,患者一般情况良好。结论肺恶性孤立性纤维性肿瘤极为罕见,诊断和鉴别诊断主要依靠病理形态学和免疫表型,治疗以手术完整切除肿瘤为主,术后应对患者进行长期随访。     

脑膜不典型孤立性纤维性肿瘤一例

患者男 ,46岁。因阵发性头痛半个月 ,于 2 0 0 1年 12月 3日入院。患者半月前无诱因出现双侧颞部疼痛 ,尤以右侧为甚 ,傍晚常加重。疼痛呈阵发性 ,持续 10～ 2 0ｍｉｎ ,采取卧位及服用镇痛药可缓解。不伴呕吐、复视、抽搐及行走困难等症状。既往有酗酒史及 3年双手静止性震颤史。体检 :一般情况检查无异常。神经系统检查 :右侧面部不完全中枢性面瘫。双侧视乳头水肿 ,右侧较左侧重。双上肢静止性震颤 ,左侧更为明显。左侧肢体肌力Ｖ-级。深浅感觉和腱反射对称 ,病理反射阴性。小脑功能正常。头颅ＣＴ显示右颞叶有一大小约 5 8ｃｍ× 6 4ｃ…     

Meningeal melanocytoma: case report and review of the literature

We report a case of meningeal melanocytoma in the thoracic spinal cord of a 44-year-old woman and review previously documented cases. Our patient experienced numbness and tingling in her left leg for 8 years, and low back pains with intermittent claudication for the previous 2 months. A histologically benign 20-mm tumour was totally resected. Radiation therapy was not given. The tumour showed the histological, immunohistochemical and ultrastructural features of a meningeal melanocytoma. The patient is alive without recurrence 4.5 years after surgery.     

Meningeal melanocytoma: case report and review of the literature

We report a case of meningeal melanocytoma in the thoracic spinal cord of a 44-year-old woman and review previously documented cases. Our patient experienced numbness and tingling in her left leg for 8 years, and low back pains with intermittent claudication for the previous 2 months. A histologically benign 20-mm tumour was totally resected. Radiation therapy was not given. The tumour showed the histological, immunohistochemical and ultrastructural features of a meningeal melanocytoma. The patient is alive without recurrence 4.5 years after surgery.     

软骨发育不全二例报告及文献复习

目的研究软骨发育不全的临床、影像学和病理学表现。方法复习1447例尸检中诊断为软骨发育不全的2例作回顾性研究。结果软骨发育不全患者身体长度基本正常而肢体较短,短肢以臂、大腿肢体近端为主。头颅增大,鼻梁塌陷,腰椎前凹,胸腔扁而小,肋骨异常的短。手指粗而短分开似＂三叉戟＂,肘生长受限。结论软骨发育不全是侏儒最常见的原因,在病理学上具有明显的特征性改变。     

Pigmented neurofibroma: report of two cases and literature review

Two cases of pigmented neurofibroma of the skin are reported. In case 1, the tumor was removed from the back of a 55-year-old man with no associated neurofibromatosis. In case 2, the tumor was removed from the abdominal wall of a 21-year-old woman with neurofibromatosis. Both tumors consisted of benign, short spindle cells and multiple foci of scattered melanin-laden cells. In case 1, the spindle cells were arranged in a storiform pattern, resembling features of dermatofibrosarcoma protuberans. Immunohistochemically, the spindle cells of both cases were demonstrated to be positive for S-100 protein and CD34. The melanin-laden cells stained positively for HMB-45. This report describes an additional two cases of pigmented neurofibroma that conform to the new diagnostic criteria for this disease.     

Primary gliosarcoma with long-survival: report of two cases and review of literature

Background: Gliosarcoma (GS) is a rare high-grade malignant tumor with poor prognosis. The survival period of GS ranges from 4 to 18.5 months. Rarely would it be over 40 months. Survival of intraventricular GS is less than 8 months. Methods: There were 2 cases of primary gliosarcoma in our hospital with long-term survival after resection, with one of pure intraventricular origin. We confirmed that our diagnosis was correct by light microscopy, GFAP immunohistochemistry and histochemistry of reticular fiber staining. Results: In the first case, a 47-year-old man with intraventricular gliosarcoma survived for 130 months after surgery. In another case, a 63-year-old woman survived for 4 years after resection. Both cases of GS exhibited biphasic glioblastoma and fibrosarcoma with necrosis. According to the review of surgical records, complete tumor resections, including extended resections were carried out in both cases. The two patients received postoperative radiation therapy and chemotherapy without any further recurrence and metastasis. Conclusions: We reported two cases of GS with long survival. The presented cases demonstrate that, in rare instances, gliosarcoma may show prolonged survival with after surgical excision combined with radiotherapy and chemotherapy.     

血管内大B细胞淋巴瘤尸体解剖临床病理分析

目的 探讨血管内大B细胞淋巴瘤(IVLBCL)的临床病理特征及病理诊断.方法 对2例IVLBCL的尸体解剖检查(简称尸检)病例进行了临床病理、免疫表型(SP法染色)及抗原受体基因(TCRγ、IgH)重排检测.结果 (1)一般情况:2例尸检分别于2005年和1979年进行,均为男性患者,年龄为70岁和50岁.2人均以神经系统功能障碍(双下肢麻木或无力)为首发表现,起病急,病程短(分别为30 d和20 d),病情进展迅速,并在短期内出现多器官功能衰竭而死亡,生存期分别为85 d和44 d.(2)尸检主要发现:除例1患者有多发性皮肤和左心外膜下结节(直径0.5～2.5 cm)外,全身均未见明显占位性病变,但双肺、肾脏、脑、脾脏等脏器有不同程度地均匀肿大和重量增加.例1患者还有多浆膜腔积液.组织学观察见多脏器、组织(脑、垂体、脊髓和脊神经根、心、肺、肾、肝、脾、消化管、淋巴结、胰、肾上腺、甲状腺、睾丸等)的小血管内见数量不等的异形淋巴样细胞存在,且各处之肿瘤细胞形态相似.瘤细胞中等偏大,呈圆形或卵圆形,细胞质丰富,核质比高.细胞核为圆形或卵圆形,核膜较厚,核染色质呈斑块状散在分布,可见1～3个嗜碱性的小核仁.(3)免疫表型及抗原受体基因重排检测:肿瘤细胞均表达B淋巴细胞分化抗原CD20和CD79a;部分瘤细胞表达bcl-2蛋白和CD5抗原;2例之肿瘤细胞均不表达T细胞及髓系分化抗原等[CD3、髓过氧化物酶(MPO)、bcl-6、CD30、CD10和PCK等];Ki-67阳性率均为80%.PCR检测均检出免疫球蛋白重链基因(IgH)克隆性重排,而TCRγ基因呈胚系构型.结论 2例IVLBCL均以神经系统功能障碍为首发表现,有相似的病理形态学特征,免疫表型及基因型,预后差.     

富于细胞性血管脂肪瘤的临床病理学观察

目的 探讨富于细胞性血管脂肪瘤的临床病理学特点、免疫学表型和鉴别诊断.方法 回顾性复习2例富于细胞性血管脂肪瘤的临床资料和组织学形态,行网状纤维染色,并采用CD31、CD34、FⅧRAg、UEA-1、α-SMA和vimentin等抗体进行免疫组化标记(EnVision法).结果 患者均为男性,年龄分别为38岁和33岁.临床上分别表现为左上臂皮下(例1)、躯干和四肢皮下(例2)多发性小结节,部分结节有疼痛感和触痛感.大体上,结节均有包膜,直径为1～1.5 cm.镜下,例1中的1枚结节呈经典的血管脂肪瘤形态,另2枚结节主要由大量的血管组成,而脂肪组织较少(<5%).网状纤维染色清晰显示丰富的血管结构.部分小血管内可见纤维素性血栓.例2中的2枚结节主要由大量的血管和丰富的梭形间质细胞组成,脂肪组织仅占5%和10%.免疫组化标记显示,富于小血管的区域弥漫强阳性表达CD31、CD34、FⅧRAg、UEA-1和α-SMA,血管之间的梭形间质细胞主要表达vimentin.结论 富于细胞血管脂肪瘤是血管脂肪瘤的一种极为罕见的亚型,可被误诊为梭形细胞脂肪瘤、梭形细胞血管瘤、富于细胞性血管瘤和Kaposi肉瘤等肿瘤.     

Intravascular lymphoma: report of two autopsic cases, literature review

Intravacular large B-cell lymphoma (LIV) is a rare entity individualized in the WHO classification since 2001 as a subtype of extranodal diffuse large B-cell lymphoma. We report two autopsic cases of LIV: a 77-year-old woman presenting with fever, dyspnea, antehypophyseal failure and a 54-year-old man presenting with fever, weight-loss, night-sweats and encephalopathy. They died respectively 10 and 7 months after the beginning of symptoms, without diagnosis. Neither infectious disease nor lymphomatous proliferation had been identified. From these two cases and our literature review, we insist on the importance of histopathological diagnosis on biopsy for this rare pathology which clinical diagnosis remains difficult.