口腔颌面部朗格汉斯细胞组织细胞增生症30例临床分析

目的：探讨口腔颌面部朗格汉斯细胞组织细胞增生症的临床特点及治疗方法。方法：采用病例回顾性研究方法,对30例口腔颌面部朗格汉斯细胞组织细胞增生症病例的临床表现、治疗和预后进行分析。以1997年wH0的临床分型为依据,采用以切除术或刮治术为主的治疗方法,病变累及范围较广者,辅以放疗和（或）化疗。对12例患者的随访结果进行分析。结果：男18例,女12例,中位年龄8岁;66．7％的病例病变累及下颌骨。影像学主要表现为骨组织破坏和软组织侵犯。对病变局限的14例患者采用单纯手术治疗;病变范围较广、累及骨外软组织的6例患者,采用手术加术后放疗;对有明显全身症状的5例患者,采用手术配合化疗。随访的12例患者中,无病生存10例。结论：口腔颌面部朗格汉斯细胞组织细胞增生症有一定临床特点．采取相应的治疗方法．预后较好。     

下颌骨朗格汉斯细胞组织细胞增生症1例

朗格汉斯细胞组织细胞增生症在临床上以颅骨多见,较少发生于下颌骨。本文报道1例下颌骨朗格汉斯细胞组织细胞增生症,并结合文献探讨朗格汉斯细胞组织细胞增生症的病因、临床病理表现、诊断及治疗。     

晚期口腔颌面-头颈部鳞癌靶向治疗的进展浅析

晚期口腔颌面-头颈部鳞癌的治疗仍然是个巨大的挑战。尽管传统的手术、放疗及化疗已取得了很大的进展,但晚期口腔颌面-头颈部鳞癌的预后仍然较差,分子靶向药物为此带来了新的希望。目前针对口腔颌面-头颈部鳞癌的靶向治疗的靶点主要有表皮生长因子受体及血管内皮生长因子受体。前者的代表药物有西妥昔单抗及尼妥珠单抗,他们无论是单药治疗,还是与放化疗结合治疗,均表现出了良好的前景;后者的代表药物主要是贝伐单抗,也已经进行到临床Ⅲ期实验。靶向治疗为晚期口腔颌面-头颈部鳞癌的治疗提供了新的机遇。     

口腔颌面-头颈部眶外巨细胞血管纤维瘤的临床及病理特征分析

目的: 探讨口腔颌面-头颈部眶外巨细胞血管纤维瘤（extra-orbital giant cell angiofibroma,EO-GCA）的临床病理特征、鉴别诊断及治疗方法。方法: 收集整理Pubmed数据库中已报道的12例口腔颌面-头颈部EO-GCA文献资料,并结合我院2例患者的临床病理资料进行分析。结果: 14例患者（男9例,女5例）年龄24~84岁,平均43.5岁。肿瘤多数生长缓慢,大小与发生部位有关。镜下见肿瘤细胞呈卵圆形或短梭形,以富含血管和多核巨细胞为特征。免疫组织化学CD34及vimentin等间叶组织标志物呈阳性。临床极易误诊,确诊依赖于术后病理学检查,手术完整切除后复发少见。结论: 口腔颌面-头颈部EO-GCA是一种罕见的软组织良性肿瘤,临床易误诊,确诊依赖于组织病理学检查,手术完整切除后预后较好。     

穿支皮瓣修复口腔颌面-头颈部缺损专家共识

穿支皮瓣由皮肤穿支血管供血,是常用的组织缺损修复形式,但头颈部解剖复杂、缺损形式多样以及穿支血管解剖变异等,阻碍了穿支皮瓣在口腔颌面-头颈部缺损修复中的进一步应用。迄今为止,国内外对于穿支皮瓣修复口腔颌面-头颈部缺损临床应用缺少相关治疗指南,穿支血管的术前评价缺乏统一标准,穿支皮瓣的应用设计缺乏科学的规范,穿支皮瓣修复术后缺少客观的监测和评价体系。为进一步统一和规范穿支皮瓣在口腔颌面-头颈部缺损修复中的应用,提高重建效果,本共识总结全国多家著名医学院校及附属医院口腔颌面外科专家团队的实践经验,供临床医师参考。     

朗格汉斯细胞组织细胞增生症一例

朗格汉斯细胞组织细胞增生症(Langerhans cell histiocytosis,LCH)是主要发生于幼儿的一组较少见的疾病,共同的病理改变为朗格汉斯细胞异常增生浸润,从而引起多样化的l临床表现.北京大学口腔医学院·口腔医院牙周科门诊确诊LCH一例,现报告如下.     

婴幼儿朗格汉斯细胞组织细胞增生症自愈1例报告及文献复习

朗格汉斯细胞组织细胞增生症是朗格汉斯细胞及其前体细胞在全身或局部增生所致的一类疾病。3岁以内的婴幼儿多见急性播散型,病变累及全身多脏器多系统,预后较差,不经治疗的自然病程多在6个月以内。本文报告1例19个月龄朗格汉斯细胞组织细胞增生症病例,未经治疗而痊愈,随访9年余无复发。     

面颈及颈胸旋转皮瓣25例临床应用体会

目的观察面颈和颈胸旋转皮瓣的临床应用效果。方法应用这2种皮瓣修复口腔颌面-头颈部肿瘤切除后缺损共计25例。结果1例皮瓣前端静脉淤血,表皮坏死,其余均成活。结论该皮瓣在口腔颌面-头颈部肿瘤切除后缺损的修复重建中仍具有良好的修复效果。     

朗格汉斯细胞组织细胞增生症一例

朗格汉斯细胞组织细胞增生症(Langerhans cell histiocytosis,LCH)是主要发生于幼儿的一组较少见的疾病,共同的病理改变为朗格汉斯细胞异常增生浸润,从而引起多样化的l临床表现.北京大学口腔医学院·口腔医院牙周科门诊确诊LCH一例,现报告如下.     

口腔颌面头颈部鳞癌超声热化疗中国专家共识

口腔颌面头颈部鳞癌确诊时多为中晚期,单纯化疗难以使晚期患者总生存获益。超声热化疗是常规治疗手段之外,目前发展较快的一种新的针对恶性肿瘤的有效治疗方法。然而,目前超声热化疗在口腔颌面头颈部恶性肿瘤治疗中尚未普及,为推广和规范超声热化疗在此领域的临床实践,特制订口腔颌面头颈部鳞癌超声热化疗专家共识, 以提高患者远期生存率和生活质量。     

Tooth development included in the multifocal jaw lesions of Langerhans cell histiocytosis

Many cases demonstrating the oral manifestations of Langerhans cell histiocytosis (LCH) have been reported; however, tooth development in jaw lesions has rarely been mentioned. This paper reports the case of a 3-year-old Japanese girl with LCH suffering from multiple osteolytic lesions of the skull and jaw bones. She was referred to our paediatric clinic because of swelling of the mucogingival folds in the upper and lower primary molar regions. The patient responded well to steroid therapy and the osteolytic lesions resolved. There was an accompanying development of permanent tooth germs included in the lesions. Langerhans cell histiocytosis in children usually has a long-term clinical course and paediatric dentists should be involved with oral health care for affected patients.     

Langerhans cell histiocytosis in the maxillofacial area in adults. Report of three cases

Langerhans cell histiocytosis (LCH) is a disease of unknown etiology, characterized by proliferation of pathological Langerhans cells within different organs. It mainly affects children, but adult cases also occur, with an incidence rate of one to two per million. The head and neck are affected in almost 90% of cases. Diagnosis is made by means of histopathological analysis, and imaging studies are necessary in order to determine extent of the disease. There are no controlled trials proposing an optimal treatment protocol for LCH. Prognosis in adults is generally good due to the slow evolution of the disease and its favourable response to treatment. In our report, we present three cases of LCH in patients aged 16, 24, and 28 years respectively, with primary manifestation in the maxillofacial area. A literature review was also conducted.     

Multidisciplinary approach in a case of Hand–Schüller–Christian disease with maxillary involvement

Langerhans cell histiocytosis (LCH) is a childhood pathology with a peak of incidence ranging from 1 to 4 years of age, though diagnosis is often made in adult age. LCH is clinically classified into three types: eosinophilic granuloma, Hand–Schuller–Christian disease and Abt–Letterer–Siwe disease. We report a case of Hand–Schüller–Christian disease with diabetes insipidus, skull and maxillary involvement in a 16‐year‐old boy referred to our observation for gradual increase in mobility of the teeth and subsequent gradual loss of the second premolars and the first molars of the upper jaw. Due to the extension of the lesion and the age of the patient, surgery, and chemotherapy was chosen as the more fit treatment according to the current protocol. The clinical and radiological evaluation at the end of the therapy and after 5 years showed complete remission. The absence of relapse has allowed to initiate a fixed orthodontic dental alignment treatment with a good response to orthodontic treatment despite the underlying disease. The present case exemplifies the importance of close multidisciplinary dental and medical collaboration including general dentistry, periodontology, oral medicine, oral and maxillofacial pathology, oral radiology, orthodontics and hematology‐oncology for diagnosis, management, treatment monitoring, and decision‐making.     

Langerhans cell histiocytosis: oral/periodontal involvement in adult patients

Objective:  Langerhans cell histiocytosis (LCH) is a clonal proliferative multisystem disease. Although bone and mucosae have been classified as non-risk organs, their involvement may increase the risk of disease progression. Oral and periodontal lesions are burdened with a significant impairment of quality of life for associated signs, symptoms and loss of function. Most of information regards paediatric disease; the disease in adults has received limited attention.
Subjects and Methods:  A total of 31 adult patients affected by immuno-histopathology confirmed LCH have been prospectively examined; attention was paid to the occurrence and characterization of oral lesions.
Results:  Twelve patients developed oral lesions. Posterior regions of jawbones were always affected; the involvement of anterior regions was not constant. Unifocal oral involvement was significantly associated with multisystemic disease while multifocal lesions were associated with unisystemic disease. Oral disease presented with soft tissue ulcers (50% of cases), gingival bleeding (66.7%), pain (83.4%), periodontal damage (50%), tooth mobility (16.7%), non-healing extraction socket (8.3%); 41.6% of patients complained of negative outcomes on quality of life. Oral lesions were easily handled with local measures.
Conclusions:  Posterior regions require attention; single oral lesions may be part of multisystemic disease; oral and periodontal lesions may be early signs of disease reactivation.     

婴儿腭部郎格汉斯细胞组织细胞增生症1例报告

婴儿腭部郎格汉斯细胞组织细胞增生症（Langerhans cell histiocytosis，LCH）在临床上较少见。本文报告1例婴儿腭部LCH病例，结合文献复习，从发病率、临床表现、影像学特点、诊断、临床分型、分级、治疗方法和预后等方面进行讨论。本例患者采用局部肿块刮除加术后化疗，效果良好。     

累及下颌骨及硬腭的成人郎格罕细胞组织细胞增生症1例报道及文献回顾

郎格罕细胞组织细胞增生症(Langerhans cell histiocytosis,LCH)是一组病因不明、以病理性郎格罕细胞增生、浸润为主要特点的疾病,临床上成人LCH较少见,亦少累及上颌骨和腭部。本文报道了一例以腭部溃疡为主诉的成年LCH病例,并通过对近年相关文献的复习,介绍LCH的病因、临床损害特点、诊断标准、分类、治疗等。     

Langerhans cell histiocytosis: impact on the permanent dentition after an 8-year follow-up

Langerhans cell histiocytosis (LCH) is a rare disorder which mainly affects children. Oral changes, such as gingival ulceration, halitosis, osseous lesions associated with tooth mobility, and early loss of primary teeth, comprise the disease's first manifestations for most patients. In the long term, dental changes may occur as a result of dental and systemic treatment. Therefore, this study's purpose is to report Langerhans cell histiocytosis' effects on the permanent dentition 8 years after diagnosis, which had been established by performing a biopsy of gingival tissue on a 3-year-old patient.     

S-100蛋白、CD1a、CD83及Ki-67在口腔朗格汉斯细胞组织细胞增生症中的表达及意义

目的对口腔朗格汉斯细胞组织细胞增生症（LCH）病例进行临床病理回顾性研究及多种免疫表型检测,观察该类疾病的临床病理特征,并对其诊断、鉴别诊断等进行探讨。方法选择原病理诊断为LCH的29例患者为研究对象,分析其临床病理特点;采用链亲和素-生物素-过氧化物酶（SP）法和Elivison二步法检测S-100蛋白、CD1a、CD83及Ki-67在LCH中的表达情况,观察其免疫组织化学结果并进行统计学分析。结果29例LCH中有5例S-100蛋白、CD1a检测为阴性,排除LCH诊断。在24例LCH中,男性15例,女性9例;患者中位年龄为7.50岁;14例发生于下颌骨,5例发生于上颌骨,5例发生于上下颌骨;按照Bartnick分类,Ⅰ类9例,Ⅱ类13例,Ⅲ类2例;S-100蛋白、CD1a均为阳性表达;颌面部单发骨病损与侵及软组织的颌面部病损相比,Ki-67阳性率较低,而CD83阳性率较高。结论S-100蛋白、CD1a对于LCH的诊断具有重要意义。颌面部单发的骨LCH可能具有较低的增殖活性,并处于较高的成熟状态;侵及软组织的颌面部LCH可能具有较高的增殖活性,并处于较低的成熟状态。     

S-100蛋白、CD1a、CD83及Ki-67在口腔朗格汉斯细胞组织细胞增生症中的表达及意义

目的 对口腔朗格汉斯细胞组织细胞增生症(LCH)病例进行临床病理回顾性研究及多种免疫表型检测,观察该类疾病的临床病理特征,并对其诊断、鉴别诊断等进行探讨.方法 选择原病理诊断为LCH的29例患者为研究对象,分析其临床病理特点;采用链亲和素-生物素-过氧化物酶(SP)法和Elivison二步法检测S-100蛋白、CD1a...