骨促结缔组织增生性纤维瘤的影像诊断与鉴别诊断

目的分析骨促结缔组织增生性纤维瘤的影像学表现,总结骨促结缔组织增生性纤维瘤的影像学诊断特点和鉴别诊断要点。 方法回顾性分析2002年1月至2019年10月经病理证实的13例骨促结缔组织增生性纤维瘤的X线、CT和MRI,分析影像学表现包括：发病部位、骨质破坏情况、病变内的骨质密度、病变区的MR信号特点及周围侵袭性特点。 结果13例骨促结缔组织增生性纤维瘤患者中,男性7例,女性6例,年龄为2~52岁,中位年龄27岁。本组13例的发病部位是长骨8例,髂骨3例,椎体及跟骨各1例。长骨常见于干骺端,可累及骨干及骨端。丝瓜瓤型6例,溶骨型3例,混合型3例,骨旁型1例。病变区内的CT值30~60 Hu（6例CT值大于45 Hu）。具有MRI检查的8例患者中5例表现为T1WI呈不均匀等或低信号,T2WI呈不均匀等或高信号,其内可见T1WI、T2WI均为低信号区域;3例病变区呈均匀长T1长T2信号。13例DFB中9例病变周围可见软组织肿块。 结论骨促结缔组织增生性纤维瘤具有侵袭性,以丝瓜瓤型多见,病变区存在T1WI、T2WI均为低信号,有助于其诊断及鉴别诊断。     

骨韧带样纤维瘤外科手术疗效分析

目的：探讨骨韧带样纤维瘤外科手术治疗效果。方法：自2000年6月至2010年6月,手术治疗15例骨韧带样纤维瘤患者,男4例,女11例;年龄18～64岁,平均39岁。部位分别是股骨上段4例、股骨下段3例、胫骨下段2例、肱骨上段1例、肱骨下段1例、肩胛骨1例、耻骨支1例、髂骨1例、胸骨柄1例。15例中1例行单纯病灶内刮除,14例按手术方式分两组,广泛切除组（7例）,扩大刮除加灭活组（7例）。观察术后复发情况,采用Enneking功能评分对术后恢复情况进行评定。结果：15例患者均获随访,时间18～132个月,平均56个月。未发现转移。复发2例,1例为单纯病灶内刮除,1例为广泛切除组,复发率13．3％（2/15）。根据Enneking功能评估标准,广泛切除组术后（21．6±3．8）分,扩大刮除加灭活（28．3±1．3）分。广泛切除组优2例,良5例;扩大刮除加灭活组优7例。结论：扩大刮除加灭活手术较广泛切除组功能恢复更好,在重建较为困难的特殊部位建议优先考虑。     

骨母细胞瘤临床病理学研究

探讨良性骨母细胞瘤和恶性母细胞瘤的诊断和鉴别诊断标准，采用临床病理分析及常规病理学观察方法。骨母细胞瘤除出现经典的组织学特征外，尚可出现车辐状排列及血管瘤样变化。骨母细胞增生活跃及出现异型的巨型上皮样骨母细胞可作为恶性骨母细胞瘤的组织学诊断依据。恶性骨母细胞瘤是介于良性骨母细胞瘤和肉瘤之间的中间型病变，有其特殊临床及病理学意义，应与良性骨母细胞瘤和骨母细胞型骨肉瘤进行鉴别。     

骨的韧带样纤维瘤的诊治进展

骨的韧带样纤维瘤是一种罕见的良性肿瘤，其临床表现、化验、影像学检查无特异性表现，诊断绝大部分依靠术后病理检查。本文就其近期诊治进展等方面问题进行综述。     

睾丸富于细胞纤维瘤临床病理观察

目的:探讨睾丸富于细胞纤维瘤的临床病理特征、鉴别诊断和预后。方法:对1例睾丸富于细胞纤维瘤的临床表现、组织形态和免疫组化进行综合分析,并结合文献讨论其病理特点及鉴别诊断。结果:30岁男性患者,右侧睾丸不适并无痛性肿大为主诉。睾丸纤维瘤实性边界清楚,包膜亦厚亦薄,切面灰白色,镜下梭型瘤细胞排列成席纹状、鱼骨状或束状,细胞丰富,细胞核无多形性,核分裂像为0～1个/10HP。免疫组化显示肿瘤细胞波形蛋白(+)、S-100局灶(+)、平滑肌蛋白局灶(+)、肌纤维蛋白(-)、α-抑制素(-)、CD34(-)、CD99(-)、Ki-67阳性率不足1%。结论:睾丸富于细胞纤维瘤是一种罕见的睾丸性索间质肿瘤,与卵巢同源性肿瘤病理特征相同;容易与睾丸的其它梭型细胞肿瘤混淆,形态学结合免疫组化可资鉴别;手术切除预后良好。     

右胫骨骨化性纤维瘤1例

骨化性纤维瘤多发生于颌骨、颅骨 ,少见于四肢长骨 ,作者诊治 1例 ,报道如下。1　临床资料患者 ,男 ,2 7岁 ,因右小腿酸痛、劳累后加重 3年 ,门诊以骨囊肿收入院。查体 :心肺、肝脾未见异常 ,右小腿上段胫骨压痛 ,皮肤颜色正常 ,无红肿。Ｘ线片示 :右胫骨上段近干骺端有 7 5ｃｍ× 2 5ｃｍ大小类圆形密度减低区 ,密度不均匀 ,呈毛玻璃样改变 ,散在有沙砾样钙化点 ,边缘呈花边样硬化。见图 1。图 1　右胫骨骨化性纤维瘤　　术前Ｘ线正侧位片　　手术行开窗病灶清除。术中见病灶区为灰白色肉芽组织 ,质地较韧 ,界限清 ,周围骨质硬化。刮除后…     

长骨多发性骨化性纤维瘤一例报告

长骨多发性骨化性纤维瘤一例报告张华俦黄建发黄公怡患者男，５７岁。因外伤摄右髋部及右小腿正侧位片偶然发现右股骨颈及右胫骨中下段骨结构异常，于１９９５年６月１４日收治入院。查体示右髋部及右小腿无红肿，无静脉怒张，触之局部皮温不高，局部有轻压痛，右髋关节及...     

Desmoplastic Fibroma of Bone

Background and purpose Specialist hospitals have reported an incidence of early deep infections of < 1% following primary knee replacement. The purpose of this study was to estimate the infection rate in a nationwide series using register-based data.

Methods The Finnish Arthroplasty Register (FAR) was searched for primary unicompartmental, total, and revision knee arthroplasties performed in 1997 through 2003 and eventual revision arthroplasties. The FAR data on revision arthroplasties was supplemented by a search of the national Hospital Discharge Register (HDR) for debridements, partial and total revision knee replacements, resection arthroplasties, arthrodeses, and amputations.

Results During the first postoperative year, 0.33% (95% CI: 0.13–0.84), 0.52% (0.45–0.60) and 1.91% (1.40–2.61) of the primary UKAs, primary TKAs, and revision TKAs, respectively, were reoperated due to infection. The 1-year rate of reoperations due to infection remained constant in all arthroplasty groups over the observation period.

The overall infection rate calculated using FAR data only was 0.77% (95% CI: 0.69–0.86), which was lower, but was not, however, statistically significantly different from the overall infection rate calculated using endpoint data combined from FAR and HDR records (0.89%; 95% CI: 0.80–0.99). FAR registered revision arthroplasties and patellar resurfacing arthroplasties reliably but missed a considerable proportion of other reoperations.

Interpretation More reoperations performed due to infection can be expected as the numbers of knee arthroplasties increase, since there has been no improvement in the early infection rate. Finnish Arthroplasty Register data appear to underestimate the incidence of reoperations performed due to infection.     

Desmoplastic Fibroma of the Pediatric Cranium: An Aggressive Skull Tumor with Local Recurrence

Cranial desmoplastic fibroma (DF) is extremely rare and only 20 cases, including only 7 pediatric cases, have been reported previously. We describe the first case of a child with cranial DF that increased in size over a short-term and recurred after resection. The aim of this case report was to discuss the clinical, radiological, and histological characteristics and optimal treatment for this rare and aggressive skull tumor.     

Desmoplastic Fibroblastoma (Collagenous Fibroma): A Case Identified in the Buccal Mucosa

Desmoplastic fibroblastoma is a rare, benign, soft tissue tumor affecting mainly the subcutaneous and muscle tissue. Only five cases identified in the oral cavity have been reported in prior literature. This article presents a case report of a 56-year-old man, with no previous history of trauma, who presented a slow-growing mass in the buccal mucosa. Histopathology and immunohistochemistry staining studies were performed, and a diagnosis of the desmoplastic fibroblastoma was made. The patient has been disease-free for one year.     

Collagenous Fibroma (Desmoplastic Fibroblastoma) of the Neck Presenting with Neurological Symptoms

Collagenous fibromas are rare fibrous soft tissue tumours that usually arise in subcutaneous tissue or skeletal muscle at a variety of anatomical sites. These lesions commonly present as painless, slow-growing mobile masses. We describe a unique case of a 41-year-old woman presenting with a posterior neck swelling and longstanding history of severe ongoing pain in the right scapular region, shoulder and neck, weakness of the palmar grip and limited right lateral neck flexion and rotation. A history of trauma to the right neck in adolescence was noted. Histological analysis revealed a paucicellular lesion with spindle and stellate-shaped fibroblasts involving the cervical nerve roots, typical of collagenous fibroma. In a literature search on Medline and Pubmed, we found no reported cases of collagenous fibromas presenting with neurological symptoms. This report highlights the potential of these lesions to present with neurological symptoms due to infiltration of surrounding tissues, and that preceding trauma may contribute to the aetiology.     

胶原纤维瘤的病理学及MRI特征(3例报告及文献复习)

目的 总结胶原纤维瘤的病理学及MR I特征,分析其诊断价值。方法 1997年1月-2007年1月共收治胶原纤维瘤3例,均予切除,总结其MR I特点、形态学观察及免疫组化特征。结果 MR I观察肿瘤边界清楚,质地均匀,呈内中散在的圆形高信号影。镜下观察显示,肿瘤由稀疏的梭形或星状纤维母细胞和大量致密或纤维粘液样的间质组成。免疫组织化学标记显示瘤细胞表达波形蛋白,部分表达平滑肌肌动蛋白、肌特异性肌动蛋白和结蛋白,不表达CD34、细胞角蛋白和S-100。结论 胶原纤维瘤是一种具有独特临床病理学特征的良性纤维母细胞性肿瘤,MR I有助于诊断,而进一步确诊依赖于组织学免疫组化检查。     

123例原发性IgA肾病患者临床特点与病理分析

目的：探讨原发性IgA肾病患者临床表现、病理特点及其相关性。方法：回顾性总结分析123例经肾活检病理确诊为原发性IgA肾病的临床和病理资料。结果：123例IgA肾病患者在21岁～40岁年龄段发病率最高（占65.8%）;临床表现以发作性肉眼血尿最多见（占36.6%）;病理类型分级以Ⅱ级（56.1%）,Ⅲ级（20.3%）为主;病理类型与临床表现呈正相关（P〈0.01）;随着Lee氏病理分级程度的增高,血肌酐、血尿酸、血脂有不同程度的升高（P〈0.05）。结论：IgA肾病临床类型多样,其组织形态学改变轻重不一,宜尽早做肾活检以明确诊断,指导治疗。     

肢体肿瘤所致病理性骨折的早期诊断与治疗

目的 正确认识肢体肿瘤所致病理性骨折的概念和病因,掌握早期诊断与鉴别诊断方法,减少误诊、误治,提高治疗效果。方法 １９８１年１２月～１９９７年１０月共收治肢体肿瘤所致病理性骨折患者１６２例,其中良性肿瘤与肿瘤样病损８５例,恶性肿瘤７７例。治疗原则：良性肿瘤行刮除植骨,根据不同情况分别采用张力带钢丝、钢板螺钉、石膏以及单臂外固定架进行固定。恶性肿瘤要在配合化疗和放疗基础上分别采用肿瘤段切除灭活再植、     

活性煅烧骨的实验研究与临床应用

验证煅烧骨是骨形态发生蛋白的理想载体。方法：将骨形态发生蛋白与自制煅烧骨按１：２０的重量比例，组成活性煅烧骨，植入兔尺骨骨缺损处，术后１４，２８，５６，８４天时进行Ｘ线检查，组织学观察，新骨生成量观察及降解率测定，并应用于临床。     

Desmoplastic melanoma of the lip

BACKGROUND: This retrospective study looks at the prognosis of desmoplastic melanoma of the lip, correlating it with the clinical course, treatment, and patterns of failure. METHOD: Twenty-two patients with desmoplastic melanoma of the lip were seen at the University of Texas M. D. Anderson Cancer Center from 1965 to 1998. RESULTS: Three disease groups: (I) untreated tumor (3 patients), (II) excisional scar (10 patients), and (III) locoregional recurrence (9 patients). Group I had two cures and one failure. In group II six had no recurrences, and there were four failures. In group III, all patients failed. Ten patients (45%) had no evidence of disease, of which three (30%) had an initial misdiagnosis. Twelve patients (55%) died of disease or were living with disease, of which eight (67%) had an initial misdiagnosis. CONCLUSIONS: Desmoplastic melanoma of the lip is often misdiagnosed and, therefore, inappropriately treated with multiple recurrences and poor prognosis. Accurate diagnosis and combined treatment may improve local control and survival.