股骨原发性平滑肌肉瘤2例并文献复习

骨原发性平滑肌肉瘤(primary leiomyosarcoma bone, PLB)极其少见,在临床及影像诊断方面缺乏系统的认识,现报告本院行影像学检查并经病理证实的 PLB 2例,结合国内外文献分析其影像学表现,旨在提高对 PLB 的认识和影像诊断水平。     

原发性甲状旁腺功能亢进合并多发棕色瘤2例报告并文献复习

棕色瘤亦称巨细胞瘤样变,系由原发性甲状旁腺功能亢进症(简称甲旁亢)引起的局限性纤维囊性骨炎,表现为单发或多发的边界清楚的溶骨性病变.临床上易误诊,本文报道2例原发性甲旁亢伴棕色瘤,以提高对本病的诊断及鉴别诊断水平.     

Apert综合征2例报告及文献复习

例1 患儿,男,6岁,因“双手足各指(趾)粘连6年”入院.患儿出生后,即发现双手足2～5指(趾)粘连.随着患儿生长发育,渐发现颜面部发育异常,头颅扁头,突眼,眼距宽,下睑睫毛呈内翻状,眼球常红肿、溢泪.入院查体:患儿身材较同龄儿童矮小,身高110 cm,体重19 kg,智力较低下.心、肺、腹部未及明显异常.专科情况:头颅扁平,前额宽而广,头颅横径16 cm,前后径15 cm.双眼眼球外凸,内眦间距约3.5 cm,内眦高于外眦约1 cm.平视时,双上眼睑缘下垂覆盖至瞳孔中央水平线,双侧下眼睑睫毛呈内翻状,眼球红肿、溢泪.患儿呈张口状,流涎,可见腭裂(图1).头颅X线片示:头颅扁平,呈舟状,脑回压迹多且深,颅缝早期闭合,上颌发育不全,低位前牙,牙列较拥挤(图2).双手足2～5指(趾)完全粘连,甲融合(图3,4).     

静脉内平滑肌瘤病2例并文献复习

目的 探讨静脉内平滑肌瘤病（IVL）的影像特征。方法 回顾性分析2例经手术病理证实的IVL的临床表现及影像特征,并复习相关文献。结果 MRI平扫显示子宫及附件区不规则肿块,T₁WI呈低信号,T₂WI呈稍高信号,子宫周围多发迂曲血管影;扩散加权成像（DWI）上病变扩散受限呈不均匀高信号;增强MRI显示病变明显强化,受累静脉不同程度增宽且强化欠均匀。对比增强磁共振血管成像显示盆腔多发不规则团片状明显不均匀强化灶,受累静脉管腔增宽,内见多发条索状充盈缺损。超声检查显示子宫角不均质回声,部分呈融合状,1例病灶向上与静脉血管相连,病灶内可见丰富的血流信号;另1例病灶内有少量血流信号。结论 IVL影像表现具有一定特征,盆腔肿块伴邻近脉管增宽、管腔内充盈缺损是本病常见的影像征象。     

Spontaneous intracranial hypotension: report of four cases and review of the literature

Spontaneous intracranial hypotension is an unusual syndrome of postural headache and low cerebrospinal fluid pressure without an established cause. We present four cases, analyze those previously reported in the literature, examine the MRI, CT, angiographic and cisternographic finding and discuss the clinical picture, proposed pathophysiologic mechanisms and potential treatment. Received: 18 October 1997 Accepted: 16 January 1998     

Robinow syndrome: report of two cases and review of the literature

We report two patients with Robinow syndrome, review the published literature and stress the importance and limitations of radiographic examination in the diagnosis of this disorder, which shows extreme clinical and radiographic variability. The radiographic differential diagnosis of Robinow syndrome is discussed.     

Rhino-orbito-cerebral mucormycosis: report of two cases and review of the literature

Rhino-orbito-cerebral mucormycosis (ROCM) is a rare, fulminant opportunistic fungal infection that is mostly seen in immunocompromised or diabetic patients. The disease should be recognised and treated immediately. We present here MR imaging findings of two patients with histopathologically proven ROCM. One of the cases had a history of corticosteroid treatment and iatrogenic diabetes mellitus and although amphotericin B was started immediately, the disease progressed and surgical debridement was necessary. The second case was a patient with diabetes mellitus type 1 in whom ROCM had occurred following an abdominal surgery; amphotericin B treatment alone was adequate in this patient.     

Spinal cord herniation: report of two cases and review of the literature

Idiopathic herniation of the spinal cord is an extremely rare disorder which may cause progressive myelopathy. Two cases of this entity reported herein were both examined using MRI and CT myelography. The typical appearance of this disease with or without a dorsal intradural arachnoid cyst is focal ventral displacement of the mid-thoracic spinal cord, mimicking an isolated intradural spinal arachnoid cyst on MRI. CT myelography using thin slice sections is useful in the diagnosis of this disease. Received 14 August 1995; Revision received 30 January 1996; Accepted 4 June 1996     

胸膜肺结节病2例病例报告及文献复习

目的 介绍胸膜肺结节病(PPS)的概念,临床、影像学特征,诊断,鉴别诊断以及诊疗进展.方法 报告2例PPS的临床表现、诊疗经过及预后转归.通过文献复习,总结国内外PPS的临床特征、诊疗进展和鉴别诊断要点.结果 病例特征:青年男性,因"呼吸困难"就诊,对诊断性抗结核治疗不敏感;肺功能检查显示弥散障碍,胸部高分辨CT(HRCT)提示Ⅲ期肺结节病分别合并弥漫性胸膜增厚/胸腔积液;通过胸膜(胸腔镜)、肺或支气管活检,组织病理发现胸膜、肺或支气管非干酪样肉芽肿浸润,除外结核、真菌感染后,确诊PPS.患者对糖皮质激素治疗敏感,1例患者恢复正常,另1例在治疗过程中发生皮质类固醇相关性肺结核,全身播散,死于结核性脑膜脑炎.通过文献复习,总结归纳如下:PPS以咳嗽、胸痛、呼吸困难为主要就诊症状,体征为胸腔积液、胸膜增厚或气胸.由于临床表现缺乏特异性,PPs易误诊为结核性胸膜炎.胸部放射影像学检查尤其是HRCT可发现与胸膜病变同时存在或继发的Ⅰ-Ⅳ期肺结节病,Ⅲ或Ⅳ期为主;肺功能检查多提示弥散障碍,二者均有助于鉴别诊断.结论 PPS相对罕见,病理生理基础为肺实质、胸膜非干酪肉芽肿浸润,确诊依赖胸膜、肺、支气管活检.HRCT可灵敏准确地反映胸膜、纵隔及肺实质病变.目前推荐激素治疗,起始剂量为泼尼松0.5mg/kg,1次/d,治疗过程中应密切随访皮质类固醇相关性结核的早期征象.     

结节病肾损害2例报告并文献复习

目的分析2例结节病肾损害患者的临床病理特征及疗效,旨在提高结节病肾损害的诊断及治疗水平。方法回顾性分析2例女性结节病肾损害患者的临床表现、病理改变及疗效,并通过文献复习总结结节病肾损害的诊治经验。结果2例患者临床均出现血清肌酐升高。例1为肾脏局限性结节病,病理改变为肉芽肿性间质性肾炎。例2为典型结节病,肾外累及肺、眼、淋巴结、腮腺,出现高钙血症及肾结石,肾脏病理改变为肉芽肿性间质性肾炎与钙质在肾组织沉积。2例患者自出现临床症状到明确诊断的时间分别为1年和5年。糖皮质激素联合免疫抑制剂治疗1个月后,2例患者尿蛋白、血清肌酐均降至正常,例2血钙亦降至正常。结论结节病肾损害常常延误诊断;临床表现为肾小管间质损害的患者应及早行肾组织活检,明确诊断后应采用糖皮质激素联合免疫抑制剂治疗。     

原发性卵巢平滑肌肉瘤3例报告并文献复习

目的 分析原发性卵巢平滑肌肉瘤的病因、组织学特性、临床特点、治疗方法及预后.方法 回顾性分析解放军总医院收治的3例原发性卵巢平滑肌肉瘤的诊疗经过,并结合近10年来国内外的文献资料,总结原发性卵巢平滑肌肉瘤的诊治经验.结果 本组3例原发性卵巢平滑肌肉瘤均发生于>50岁的女性,发病原因不明确,临床表现无特异性,病情进展快....     

Trans-sphenoidal and sphenoethmoidal encephalocele: report of two cases and review of the literature

Basal encephaloceles are the least common form of encephalocele. Due to the critical position of the bony defect, visual and endocrinological abnormalities are frequently associated with basal encephaloceles. There is significant confusion in the classification of basal encephaloceles, particularly among trans-sphenoidal, sphenoethmoidal and intrasphenoidal subtypes. Two cases of basal encephaloceles are presented (one trans-sphenoidal and one sphenoethmoidal), along with a review of the literature. The relationship between the basal encephaloceles and endocrinological abnormalities is also emphasized.     

气管-支气管肌上皮瘤的CT表现(附2例报告及文献复习)

气管-支气管肌上皮瘤极为罕见,是起源于支气管黏液腺肌上皮的低度恶性肿瘤.目前的诊断主要依赖于病理学,电镜下瘤细胞胞质内有大量的肌微丝或微丝,S100蛋白、Vimentin和平滑肌肌动蛋白等免疫组化的多种标记物联合检测的结果可为确诊或鉴别诊断提供有价值的依据.笔者收集了本院2009-2010年2例经病理证实的气管-支气管肌上皮瘤患者的CT和临床资料,并复习相关文献.     

Stafne's bone cavity: a review of the literature and report of two cases

This paper reports two cases of Stafne's bone cavity, one of which shows apparent regression over a period of 8 years: an unusual feature that has not previously been reported. A comprehensive review of the literature dealing with this well demarcated mandibular radiolucency of unknown aetiology accompanies these two case reports.     

原发骨脉管性肿瘤(附三例报告及文献复习)

提高对骨脉管性肿瘤的影像学认识。方法报告３例经病理证实的罕见骨脉管性肿瘤的Ｘ线平片和ＣＴ所风和文献复习。结果本组３例罕有脉管性肿瘤,１例为多发性血管内皮细胞,２例为恶性血管外皮细胞瘤,Ｘ线表现均以溶骨性破坏为特征。结论原发骨脉管性肿瘤在临床和Ｘ线表现上都缺乏特异性,确诊仍需依赖病理组织学检查。     

神经梅毒的MRI表现（2例报告并文献复习）

目的：探讨神经梅毒的MRI表现。方法：1例血清和脑脊液密螺旋体抗体检测阳性,经青霉素治疗后临床症状消退。另1例血清密螺旋体抗体阳性手术后病理证实为脊髓梅毒树胶样肿。2例均行MRI检查。结果：1例脑实质性梅毒表现为右侧颞叶内侧T1WI呈略低信号,T2WI呈均匀高信号,DWI及FLAIR呈高信号。1例脊髓树胶样肿表现为T1WI为低信号或等、低混杂信号灶,呈类圆形,T2WI为等高混杂信号灶;钆喷替酸葡甲胺（Gd-DTPA）增强T1WI示病灶呈不规则环形强化,并与背侧脊膜以钝角相交。结论：神经梅毒的MRI表现多种多样,对其的认识很重要以达到早期诊断和治疗的目的。