特殊颅咽管瘤1例

1　病例报告女 ,5 5岁。头痛 30 a余 ,视力下降 1a,右眼视力 0 .4,左眼视力 0 .2 5 ,双眼底视乳头色淡 ,边界清 ,无内分泌症状 ,无视野缺损 ,无其他神经系统阳性体征。头颅 CT示鞍区类圆形均匀一致高密度影 ,边缘光滑 ,约 2 .1cm× 1.9cm大小 ,CT值 5 4～6 6 Hu。头颅 MRI显示 :鞍内及鞍上亚铃形肿物 ,略偏后 ,T1 、T2加权均为高信号 ,大小约 2 .3cm× 2 .0 cm× 3.0 cm,边缘光滑 ,视神经受压 ,向上推移 ,蝶鞍下陷。术前诊断 :垂体腺瘤。行手术治疗 ,术中见鞍区肿物 ,呈囊性 ,包膜似两层蛛网膜厚 ,囊内均为乳白色略粘稠液体。全部吸除囊…     

异位额窦、筛窦颅咽管瘤1例

1病例报告女,61岁。4个月前发现额部眉间处有2.0 cm×2.0 cm大小肿物,自觉右眉弓疼痛,在当地医院行抗炎治疗后肿物逐渐消退,病程中无明显头痛、发热及视力下降等症状。查体:意识清楚,语言清晰流利,应答切题,额部眉间偏右侧可触及约2.0cm×2.0 cm大小骨质缺损,双侧瞳孔等大同圆约2.5 mm,对光反射存在,眼球活动自如,粗测嗅觉正常,视力、视野正常,颅神经查体无异常,四肢肌力V级,肌张力正常,生理反射对称存在,病理反射未引出。头颅CT示:额窦、筛窦内均匀一致略低密度占位影像。术前诊断:额窦、筛窦囊肿。2004-02-17在局麻下行额窦、筛窦占位切…     

MIB-1在颅咽管瘤的表达及意义

目的：通过检测细胞周期相关核抗原Ki-67的抗体（monoclonal antibody of cell proliferation associated nuclear antigen，MIB-1）表达，研究不同病理类型颅咽管瘤细胞增殖性的差异及意义。方法：收集30例经手术治疗的颅咽管瘤病例，采用免疫组化SP法检测MIB-1在肿瘤组织中的表达，并计算MIB-1标记指数（MIB-1 LI）。结果：MIB-1主要在颅咽管瘤基底细胞层表达，在指突样瘤组织及肿瘤组织内孤立的瘤细胞团中也有较多的MIB-1阳性染色细胞，造釉细胞型颅咽管瘤组织MIB-1 LI显著高于鳞状乳头型颅咽管瘤（P〈0．05）。结论：不同病理类型颅咽管瘤细胞增殖性存在明显差畀，不同病理类型颅咽管瘤细胞增殖性的差畀可能影响其预后。     

颅咽管瘤术中超声表现1例

脑胶质瘤患者肿瘤边界、瘤周水肿程度及CDFI血流对Ki-67、IDH1的表达有预测价值,对术前初步判断肿瘤生物学行为及远期预后具有重要意义。     

1例颅咽管瘤摘除术患者的护理

颅咽管瘤发自颅咽管残余上皮细胞,由于颅咽管瘤的所在位置较稳蔽,部位深且位于重要功能区,手术危险性大,术后并发症多,手术后死亡率高。我科于2001年12月,首例开展了颅咽管瘤手术,经过手术前充分准备以及手术后认真观察和全方位的护理,疗效良好,患者病愈出院。现将临床护理介绍如下:     

颅咽管瘤放射治疗后并发脑胶质瘤1例

脑胶质瘤的发病原因不清。一般认为与生物、物理、化学等因素有关。我科最近收治一例经手术和病理证实放射治疗后并发颅内胶质瘤,现报告如下: 患者,男,51岁,25年前(1965年),因双目视力进行性下降去北京宣武医院诊治。检查:左眼视力0.2,右眼视力0.6,双侧视神经乳头原发性萎缩,视野双颞侧偏盲。头部X线平片示蝶鞍扩大,广泛破坏,鞍     

颅咽管瘤误诊为癫痫1例

患儿,男,7岁。因阵发性头痛伴呕吐2年,右眼斜视及双眼复视,双目视力模糊1周入院。追询病史:系第2胎,足月、顺产,无窒息及产伤史,父母非近亲结婚,其姐身体健康。患儿于3岁时开始出现无明显诱因头痛,以前额为主,阵发性发作,不痛时如正常儿,头痛剧烈时常伴恶心呕吐,有时呈喷射状。     

颅咽管瘤30例手术治疗效果分析

颅咽管瘤３０例手术治疗效果分析熊建生黄连泉钱锁开杨奇帆干小强（解放军第９４医院，南昌３３０００２）自１９７８年至１９９５年１２月我院共收治经手术与病理证实的颅咽管瘤３０例，占同期肿瘤４％，现将临床资料分析如下。１临床资料１．１一般资料本组３０例，男２...     

儿童罕见斜坡颅咽管瘤一例并文献复习

颅咽管瘤为颅内较少见的原发性肿瘤,男女发病率无明显差异,约占颅内原发性肿瘤的2％～5％,占儿童颅内肿瘤的5．6％～15％。由于颅咽管瘤常毗邻非常重要的神经结构,如下丘脑、垂体柄、视神经及三脑室底部等,在治疗中可能加重上述结构的功能损害,给治疗带来很大难度,病死率、致残率、复发率均较高,因此称其为恶性效果的良性肿瘤。颅咽管瘤好发于鞍区,鞍上多见,少数在鞍内,或者鞍内、鞍上同时发生,也可偶见于鞍下鼻咽部、第三脑室内等,而累及斜坡的极为罕见。在此笔者报道1例儿童罕见巨大斜坡颅咽管瘤患者,现结合文献复习如下。     

结肠恶性黑色素性神经鞘瘤1例并文献复习

目的 探讨恶性黑色素性神经鞘瘤的病理形态学特征及鉴别诊断要点。方法 应用光镜、免疫组化及电镜等方法对1例结肠恶性黑色素性神经鞘瘤进行组织学观察并复习文献。结果 组织形态主要由大小较一致的圆形、多边形细胞和束状、编织状排列的梭形细胞构成，细胞异型性明显。免疫组化以MBP、NSE、HMB45、S-100和vimentin阳性为特点。电镜下细胞内可见Ⅱ～Ⅳ期黑色素小体，细胞外有不完整的基底膜及长间距胶原。结论恶性黑色素性神经鞘瘤少见，发生于结肠尤为罕见。病理诊断应结合病史、形态学特征、免疫组化及超微结构。超微结构特点是确诊的最重要依据。     

心包原始神经外胚层肿瘤1例报道并文献复习

目的探讨心包外周原始神经外胚层肿瘤(pPNET)的临床及病理组织学特征。方法通过光镜及免疫组化染色分析1例心包原发的外周原始神经外胚层肿瘤,同时复习相关文献。结果肿瘤位于心包腔内,肿瘤细胞呈片状弥漫分布,为单一的小圆形细胞,胞质少,核圆。免疫组化示CD99、NSE和Vim( )。结论pPNET是一种少见的起源于原始神经外胚层的恶性肿瘤,好发于深部软组织,极罕见于心包及心脏肌层,具有高度侵袭性,预后差;免疫组化有助于PNET的诊断;目前治疗主要是手术切除加化、放疗。     

恶性乳腺腺肌上皮瘤1例报告及文献复习

目的:探讨恶性乳腺腺肌上皮瘤(AME)的临床病理特点及其诊断、鉴别诊断要点。方法:患者左乳外下象限质硬、活动度差肿块。用细针吸取组织行细胞学、常规HE切片观察和采用免疫组化EnVision染色。结果:组织学以异型增生梭形细胞为主,少量腺管散在分布。梭形细胞表达P63、S-100、SMA、34βE12和波形蛋白(vimentin),30%细胞表达ki67,不表达平滑肌特异性蛋白(MSA);腺上皮细胞ER阳性表达、PR呈阴性;梭形细胞和腺上皮细胞共同表达CKpan、CK8和CK5。本例诊断为AME。结论:恶性AME较罕见,患者以老年女性居多,恶性成分以肌上皮为主。     

胰腺实性假乳头状癌2例及文献复习

目的 分析胰腺实性假乳头状癌的临床病理特点,提高对该病的认识.方法 对2例胰腺实性假乳头状癌的临床资料、大体观察、镜下观察及免疫组化结果进行分析.结果 组织学:肿瘤有实性区、囊性区及假乳头区,并见大片出血、坏死.假乳头区肿瘤细胞围绕血管周围排列,以纤细的纤维血管为轴心形成假乳头结构.肿瘤细胞中等大小,大小较一致,胞浆嗜酸或空泡状,细胞核圆形、卵圆形,可见核沟,有轻度异形性,核分裂相1～2 个/HP,可见核固缩.并有脾或腹膜受累.免疫组化:抗胰蛋白(α-1-AT)、抗糜蛋白(α-1-AACT)、神经元特异丙烯醇(NSE)、波形蛋白(VIM)阳性,胰酶等阴性,Ki-67 20%～25%阳性.结论 胰腺实性假乳头状癌与胰腺实性假乳头状肿瘤一样有特征性的假乳头结构,免疫组化结果也无特殊.但坏死更显著,有轻度核异形性及核分裂相,有周围组织侵犯,有低度恶性.掌握以上要点有助于病理诊断.     

Malignant fibrous histiocytoma of the spleen: report of a case and literature review

There is a marked paucity of reports on malignant fibrous histiocytoma (MFH) of the spleen in the literature, and there are no previous reports of its color Doppler sonographic (US) and contrast-enhanced US findings. We report on an 82-year-old male with splenic MFH (inflammatory subtype), with an emphasis on color Doppler and contrast-enhanced US findings.     

Malignant solitary fibrous tumor of the pancreas with systemic metastasis: A case report and review of the literature

BACKGROUND Pancreatic solitary fibrous tumor(SFT) is a rare neoplasm of intermediate biological potential. So far, only 22 cases have been reported since 1999. All the cases, except one, exhibited benign features. Here, we report the first case of malignant pancreatic SFT with typical Doege-Potter syndrome, along with the clinical and pathologic evidence of its systemic metastasis.CASE SUMMARY The patient was a 48-year-old man with a 1-year history of pancreatic and liver masses and refractory hypoglycemia. Increased uptake of the tracer fluorodeoxyglucose(FDG) was found in the liver and bones by fluorine-18 FDG positron emission tomography/computed tomography. After multidisciplinary discussion, a distal pancreatectomy procedure was performed, and histological examination showed a lesion composed of abundant heterogeneous spindle cells with localized necrosis. On immunohistochemistry evaluation, STAT6 was found to be diffusely expressed in the tumor. Based on the overall evidence, the patient was diagnosed with malignant pancreatic SFT with liver and bone metastases.CONCLUSION The diagnosis of malignant SFT requires comprehensive evidence including clinical, immunohistochemistry, and histological features. This case may be presented as a reference for diagnoses and management of malignant pancreatic SFTs with systemic metastasis.     

胰腺实性假乳头状肿瘤8例临床病理分析

目的 分析胰腺实性假乳头状肿瘤(SPTP)的临床病理形态特征及免疫组化特点.方法 对8例SPTP患者的临床资料、大体观察、镜下观察及免疫组化结果进行分析.结果 8例SPTP患者中男性3例,女性4例,年龄11～76岁,平均年龄38.5岁.临床表现为不同程度的上腹痛或无症状.肿块位于胰尾4例,胰头和胰体各2例.肿块大小3cm×3 cm×2.7 cm～12 cm×11 cm×10 cm.8例均有包膜,切面见囊实性区混合.组织学:肿瘤有实性区、囊性区及假乳头区.假乳头区肿瘤细胞围绕血管周围排列,以纤细的纤维血管为轴心形成假乳头结构.免疫组化:抗胰蛋白(α-1-AT)、抗糜蛋白(α-1-AACT)、神经元特异性烯醇化酶(NSE)、波形蛋白(VIM)阳性,胰酶等阴性.结论 SPTP好发于年青女性,有特征性的假乳头结构.α-1-AT、α-1-AACT、VIM阳性,胰酶(-)、Insulin阴性.掌握以上要点有助于病理诊断.     

肺恶性外周神经鞘膜瘤1例

患者男,23岁。2个月前无明显诱因出现轻度间断性胸闷。2周前咳出4块深红色分泌物,量约20ml,未予诊治。10h前,打喷嚏后,突发持续性右侧胸部钝痛,伴头晕、心悸、眼花、冷汗、呼吸困难来院就诊。X线平片示:右侧胸腔占位性病变,伴少量胸腔积液。胸部CT示:右肺上叶见类圆形软组织团块影,边缘光滑,大小约6cm×5.5cm,密度不均,CT值约27~44.6Hu,其周围见片状高密度影,边界不清。右侧胸腔大量积液,其内密度不均,纵隔及肺门未见肿大淋巴结(图1、2)。增强扫描,病变不均匀强化,CT值约45~58.4Hu,边界较清(图3)。胸穿:呈鲜红色血性液体。拟行手术治疗,…     

恶性萎缩性丘疹病尸检1例并文献复习

目的 探讨恶性萎缩性丘疹病的临床病理特征。方法 对1例恶性萎缩性丘疹病进行尸检，结合文献讨论该病的临床表现、病理形态特点。结果 病理特点主要为小—中动脉血管管腔狭窄、血栓形成、血管闭塞和组织梗死。结论 恶性萎缩性丘疹病是一种罕见、多系统、不明原因的血管性疾病，主要累及皮肤、消化道及中枢神经系统。尚无有效的治疗手段，死亡率高。     

Malignant fibrous histiocytoma of the bone in a traumatic amputation stump: A case report and review of the literature

BACKGROUNDMalignant fibrous histiocytoma (MFH) is one of the most common soft tissue sarcomas among adults. It is characterized by large size, high grade, and biological aggressiveness. There are many reports of MFH after local stimulation, such as bone fracture, implants, and chronic osteomyelitis. In this paper, we report a patient who developed MFH 6 years after amputation, suggesting that wound healing and mechanical force play a role in the local stimulation of this disease.CASE SUMMARYA 66-year-old man complained of persistent pain in his residual mid-thigh. He had undergone amputation surgery due to a traffic accident 6 years prior. Physical examination showed tenderness but no abnormalities in appearance. X-ray radiographs and magnetic resonance imaging supported the diagnosis of a tumor, and a biopsy confirmed that the lesion was MFH. The patient received neoadjuvant chemotherapy and left hip disarticulation. During the 6-mo follow-up, there were no symptoms of recurrence.CONCLUSIONPostsurgery MFH has been reported before, and many studies have attributed it to the biological effects of implants. Our case report shows that this disease can develop without an implant and thus highlights the importance of local stimulation. The wound-healing process and mechanical force can both promote this tumor, but whether they directly cause MFH needs further investigation.