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...     

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。     

成人口腔多灶性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(Langerhans cell histiocytosis,LCH)是一种朗格汉斯细胞异常增殖和浸润性疾病,通常累及全身骨骼、皮肤、垂体,亦可累及肝脏、脾、血液系统、肺、淋巴结及中枢神经系统.成年人罕见.LCH缺乏公认的治疗方案,最常见的治疗方法包括手术、局部注射类固醇药物、化疗、放疗或联合...     

Langerhans Cell Histiocytosis Mimicking Periapical Pathology in a 39-year-old Man

Langerhans cell histiocytosis (LCH) is a clonal neoplastic proliferation of Langerhans-type dendritic cells, with more than 50% of cases of LCH seen in children younger than 15 years of age. The most common clinical presentation of LCH is solitary or multiple bony lesions. The jaws are affected in approximately 10%–20% of cases, with a strong predilection for the mandible. The maxilla is involved in only 1% of head and neck cases. When the jaws are involved, lesions of LCH may mimic periapical pathology as seen in patients requiring endodontic therapy or bone loss as seen in periodontal disease. We report the case of a 39-year-old man with LCH involving the posterior maxilla. This is a rare presentation of LCH with respect to both location and patient age. Clinicians should consider LCH when developing a differential diagnosis of an apical radiolucency of vital teeth or teeth that fail to respond to endodontic therapy and be aware of its clinical and radiographic mimics.     

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.     

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。     

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。     

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.     

Multifocal Langerhans cell histiocytosis of the jaws: a case report

Langerhans cell histiocytosis is an uncommon disease that includes a spectrum of disorders in which there is an abnormal proliferation of Langerhans cells. This article describes a case of a benign form of multifocal Langerhans cell histiocytosis of the maxilla and mandible. The patient was followed for a period of 2 1/2 years, during which time there was spontaneous remission of some of the lesions.     

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。     

Expression of phosphorylated Akt in oral carcinogenesis and its induction by nicotine and alkaline stimulation

Background:  In Taiwan, it is well documented that cigarette smoking and areca nut chewing contribute to the risk of oral squamous cell carcinoma (OSCC). The role of phosphorylated Akt (p-Akt) in oral carcinogenesis induced by nicotine and alkaline environments was investigated.
Method:  Immunohistochemistry (IHC) was used to detect p-Akt expression in cancerous ( n  = 30) precancerous ( n  = 30), and normal mucosa tissues ( n  = 10). Western blotting was used to detect time-dependent induction of p-Akt by 100 μM nicotine in normal human bronchial epithelial cell (NHBE), normal human oral keratinocytes (NHOK), immortalized human epithelial cells (HaCaT) and OEC-M1 cells, dose-dependent induction of p-Akt in OEC-M1 and HaCaT cells and pH effect of p-Akt in OEC-M1. The unpaired t -test and the Fisher's exact test were used to analyze the p-Akt immunoreactivity in various groups and its association with clinicopathological parameters.
Results:  Higher p-Akt expression in cancerous group than in normal mucosa ( P  = 0.0002) and precancerous ( P  = 0.0049) groups was observed. A time-dependent increase in p-Akt in the NHBE, NHOK, HaCaT and OEC-M1 cell lines was observed with 100 μM nicotine treatment. The dose-dependent increase in p-Akt by nicotine treatment in HaCaT and OEC-M1 cells was obviously observed. Higher p-Akt expression in more alkaline environment (pH 8.0) was observed than at pH 7.4 in OEC-M1 cells.
Conclusion:  A potential role for increased p-Akt may relate to the dose and time of nicotine use. The potential role of an alkaline environment to enhance nicotine-related oral carcinogenesis may exist.     

Progressive destruction of jaws caused by the delayed treatment of Langerhans cell histiocytosis in a 2-year-old boy: A case report

Oral Radiology - Langerhans cell histiocytosis (LCH) is a disease with varied clinic manifestations. The oral symptoms and signs of LCH localized to the jaws are nonspecific, which may lead to...     

Hutchinson-Gilford progeria syndrome: oral and craniofacial phenotypes

Objective:  Hutchinson-Gilford progeria syndrome (HGPS) is a rare early-onset accelerated senescence syndrome. In HGPS, a recently identified de novo dominant mutation of the lamin A gene ( LMNA ) produces abnormal lamin A, resulting in compromised nuclear membrane integrity. Clinical features include sclerotic skin, cardiovascular and bone abnormalities, and marked growth retardation. Craniofacial features include 'bird-like' facies, alopecia, craniofacial disproportion, and dental crowding. Our prospective study describes dental, oral soft tissue, and craniofacial bone features in HGPS.
Methods:  Fifteen patients with confirmed p.G608G LMNA mutation (1–17 years, seven males, eight females) received comprehensive oral evaluations. Anomalies of oral soft tissue, gnathic bones, and dentition were identified.
Results:  Radiographic findings included hypodontia ( n  = 7), dysmorphic teeth ( n  = 5), steep mandibular angles ( n  = 11), and thin basal bone ( n  = 11). Soft tissue findings included ogival palatal arch ( n  = 8), median sagittal palatal fissure ( n  = 7), and ankyloglossia ( n  = 7). Calculated dental ages (9 months to 11 years 2 months) were significantly lower than chronological ages (1 year 6 months to 17 years 8 months) ( P  = 0.002). Eleven children manifested a shorter mandibular body, anterior/posterior cranial base and ramus, but a larger gonial angle, compared to age/gender/race norms.
Conclusion:  Novel oral-craniofacial phenotypes and quantification of previously reported features are presented. Our findings expand the HGPS phenotype and provide additional insight into the complex pathogenesis of HGPS.     

Perivascular myoid tumors of the oral region: a clinicopathologic re-evaluation of 35 cases

Background:  Myopericytoma (MPC) is a generic denomination to describe tumors showing differentiation toward perivascular myoid cells /myopericytes. It has been suggested that MPC forms a morphologic continuum with glomus tumor (GT), solitary myofibroma (SMF), and angioleiomyoma (ALM). This proposed relationship has not yet been assessed in the oral region.
Methods:  We reviewed our 28-year experience with 35 oral tumors, originally diagnosed as ALM ( n  = 28), SMF ( n  = 4), GT ( n  = 2), and MPC ( n  = 1) to analyze their overlapping microscopic features, with the assistance of immunohistochemistry.
Results:  Myopericytoma showed a wide range of growth patterns; concentric perivascular whorls, hemangiopericytomatous areas, glomangiopericytoma (GPC)-type vessels and leiomyomatous foci. Intravascular growth was also seen. Among 28 cases studied, three ALM were reclassified as MPC ( n  = 2) and SMF ( n  = 1), based on the present diagnostic criteria. Additional MPC-type components, at varying degrees, were similarly found in four ALM and three SMF, at least focally. One GT featured intravascular whorls of spindle cells. These four interrelated groups of tumors had in common GPC-type vasculature and intraluminal cellular proliferation was nearly ubiquitously present. Diffuse immunoreactivity for α-smooth muscle actin and less staining intensity of muscle-specific actin were observed in all tumors. Only ALM displayed desmin positivity of variable extent. Neither case tested expressed CD34.
Conclusions:  Our data matches with the recent results in extraoral sites that MPC, GT, SMF, and ALM exhibit histologic and immunohistochemical overlap with each other. A common perivascular myoid differentiation between these tumor types is further reinforced by the present oral series.     

Epithelioid hemangioendothelioma of the oral cavity

OBJECTIVE: To investigate the clinicopathological characteristics and biologic behaviour of epithelioid hemangioendothelioma in the oral cavity. MATERIALS AND METHODS: The clinical features and pathological findings of nine cases with intraoral epithelioid hemangioendothelioma were reviewed, including immunohistochemistry study. RESULTS: This series comprised seven males and two females aged 6-53 years (mean 28 years). The sites of the tumour included the tongue (n = 4), lip (n = 1), the gingiva and alveoli of the maxilla (n = 1), the gingiva and alveoli of the mandible (n = 1), buccal mucosa (n = 1), and the floor of the mouth (n = 1). A painless solitary mass was the most common presentation and was found in eight cases. On pathology, the tumour grew in short strands, cords or nests of polygonal to slightly spindled epithelioid cells in fibro-myxoid stroma, with formation of intracytoplasmic lumina. Tumour cells were immunoreactive to CD34, FVIIIRAg, and vimentin. Focal-positive cytokeration were observed in three cases. Immunoreactivity for S-100 protein, epithelial membrane antigen (EMA) and human herpesvirus (HHV)-8 was negative in all cases. Two cases recurred after surgical excision, but no patient developed local or distant metastasis. CONCLUSIONS: Wide local excision with long-term follow-up seems to be the treatment of choice for intraoral epithelioid hemangioendothelioma because of their unpredictable biological behaviour and recurrence potential.     

Fatty acid synthase expression in squamous cell carcinoma of the tongue: clinicopathological findings

Background:  Overexpression of fatty acid synthase (FAS), the cytosolic enzyme responsible for the conversion of dietary carbohydrates to fatty acids, has been reported in several human malignancies and pointed as a potential prognostic marker for some tumors. This study investigated whether FAS immunohistochemical expression is correlated with the clinicopathological characteristics of oral squamous cell carcinoma (OSCC).
Materials and methods:  The clinical features of 102 patients with OSCC of the tongue treated in a single institution were obtained from the medical records and all histopathological diagnoses were reviewed. The expression of FAS was determined by the standard immunoperoxidase technique in formalin-fixed and paraffin-embedded specimens and correlated with the clinicopathological characteristics of the tumors.
Results:  Eighty-one cases (79.41%) were positive for FAS. Microscopic characteristics such as histological grade ( P  < 0.05), lymphatic permeation ( P  < 0.001), perineural infiltration ( P  < 0.05), and nodal metastasis ( P  < 0.02) were associated with FAS status. A significantly lower survival probability for patients with advanced clinical stage (log-rank test, P  < 0.001), lymph nodes metastasis (log-rank test, P  < 0.001), presence of vascular permeation (log-rank test, P  = 0.05), and perineural invasion (log-rank test, P  = 0.01) was observed in the studied samples.
Conclusion:  The expression of FAS in OSCC of the tongue is associated with the microscopic characteristics that determine disease progression and prognosis.     

Dental panoramic radiographic evaluation in bisphosphonate-associated osteonecrosis of the jaws

Objectives:  To determine the extent to which clinical and radiographic features of bisphosphonate-associated osteonecrosis of the jaw (BONJ) are correlated.
Design:  Retrospective case review.
Methods:  The records of 39 patients diagnosed with BONJ and examined by panoramic radiography were retrospectively evaluated. The arches were divided into sextants ( n  = 234) and evaluated for the following signs: sclerosis, surface irregularity, sockets, fragmentation and lysis.
Main outcome measures:  The McNemar, Kruskall–Wallis and equivalency tests were performed to analyze the association between clinical and radiographic signs and BONJ severity.
Results:  Sixty-two out of 234 sextants were abnormal by clinical criteria and 61 out of 234 sextants demonstrated at least one radiographic abnormality. There was agreement between clinical and radiographic detection in 41 sextants. The data showed equivalency between BONJ diagnosis and both sclerosis and surface irregularity. The correlation between number of clinical sites and any radiographic finding was significant in the maxilla ( P  < 0.001) but not in the mandible ( P  = 0.178). The total number of radiographic signs per patient increased with BONJ stage.
Conclusion:  Focal panoramic radiographic findings of sclerosis and surface irregularity correlate with clinical sites of BONJ. This may be a useful and reliable tool to detect early changes of BONJ or to confirm a clinical diagnosis.     

Comparison of biomaterial implants in the dental socket: histological analysis in dogs

Background: Bone graft procedures have been used commonly in buco-maxillo-facial surgery. For this reason, many researchers have evaluated the bone substitutes.
Purpose: The present study evaluated soft and hard tissue reactions to two different hydroxyapatites HAs (synthetic HA and natural HA) and bioactive glass implanted into the sockets immediately after extraction.
Materials and Methods: First and third upper and lower premolars, on both sides, were extracted from six female dogs. The alveolar sockets were randomly assigned to four groups: Group 1 – control (unfilled), Group 2 – filled with synthetic hydroxyapatite, Group 3 – filled with bovine bone mineral (natural HA), and Group 4 – filled with bioactive glass. The animals were euthanized at 4 weeks ( n  = 2), 8 weeks ( n  = 2), and 28 weeks ( n  = 2) after extraction. The mandible and maxilla of each animal were removed for histological analysis to determine soft tissue reactions, newly formed bone, bone characteristics, and presence or absence of implanted materials.
Results: Most particles of synthetic hydroxyapatite had bone formation on their surface, although some particles showed a layer of fibrous connective tissue. The bovine bone mineral group exhibited particles partially replaced with bone formation. The bioactive glass group showed particles with a thin layer of calcified tissue, but was absent in some specimens, suggesting complete resorption.
Conclusion: All biomaterials had similar behavior. Bovine bone mineral, compared to synthetic hydroxyapatite and bioactive glass, showed a larger number of particles covered with osseous tissue. All biomaterials interfered with the socket repair process.     

Langerhans cell histiocytosis in a child: a 10-year follow-up

BACKGROUND: This report describes a case of the benign form of Langerhans cell histiocytosis, which involves only the maxilla and mandible, and has no extraskeletal manifestations. CASE REPORT: A 2.9-year-old boy was referred to a paediatric dentistry clinic, where clinical, radiographic and histological findings were found to be consistent with Langerhans cell histiocytosis. The difficulties involved in the diagnosis and the subject's functional oral rehabilitation are described. In view of the presence of large bone lesions, systematic treatment consisted of oral chemotherapy and the administration of methylprednisolone by intralesional infiltration. Oral examination revealed complete resolution of the ulcerative lesions. The soft tissue appeared healthy and had a normal colour. There was no tooth mobility and radiography provided evidence of bony healing. CONCLUSION: This case is particularly interesting because follow-up clinical and radiographic observations have been made over the past 10 years. The successful treatment had several objectives, including providing the patient with an aesthetic appearance and recovering the lost vertical dimension of occlusion.     

36例口腔颌面-头颈部朗格汉斯细胞组织细胞增多症临床及预后分析

目的:探讨口腔颌面-头颈部朗格汉斯细胞组织细胞增多症(Langerhans cell histiocytosis,LCH)的临床特征、诊断、治疗方法及预后,为临床治疗提供更强的循证医学证据.方法:回顾分析上海交通大学医学院附属第九人民医院口腔颌面-头颈肿瘤科收治的36例成人及儿童口腔颌面-头颈部朗格汉斯细胞组织细胞增多...