Central giant cell granuloma

  • 文章类型: Case Reports
    中央巨细胞肉芽肿(CGCG)的发生代表了颌面部病理学领域中罕见但具有临床意义的实体。本文介绍了一名17岁女性的CGCG令人信服的案例,阐明在管理这种情况时遇到的诊断和治疗挑战。考虑到CGCG在这个人口统计中的相对不频繁,该病例不仅有助于临床对CGCG的理解,而且强调了量身定制的重要性,针对患者的管理策略。通过这次演讲,我们的目的是阐明CGCG表现的复杂性,诊断,和治疗,同时强调在青少年患者中解决这种病理至关重要的细微差别考虑。
    The occurrence of Central Giant Cell Granuloma (CGCG) represents a rare yet clinically significant entity within the realm of maxillofacial pathology. This article presents a compelling case of CGCG in a 17-year-old female, shedding light on the diagnostic and therapeutic challenges encountered in managing this condition. Given the relative infrequency of CGCG in this demographic, the case not only contributes to the clinical understanding of CGCG but also underscores the importance of tailored, patient-specific management strategies. Through this presentation, we aim to elucidate the intricacies of CGCG manifestation, diagnosis, and treatment while highlighting the nuanced considerations essential in addressing this pathology in adolescent patients.
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  • 文章类型: Journal Article
    背景:中心性巨细胞肉芽肿(CGCG)表现为局部侵袭性,骨内病变的特征是多核巨细胞在渗入骨小梁的出血基质和反应性纤维组织中积累。这种特发性非肿瘤性增生性病变主要影响下颌骨,通常在X射线上表现为单眼或多房性放射像。虽然创伤或骨内出血是潜在的诱因,确切的组织发生和病因仍不清楚。CGCG主要发生在儿童和年轻人中。有轻微的女性偏爱。方法和材料回顾性分析2015-2022年天普大学医院口腔病理/病理科21例CGCG患者的临床资料。每个案例都根据各种参数进行评估,包括年龄,性别,出现症状,射线照相结果,临床鉴别诊断,和组织学确认。用于诊断的主要放射学技术是下颌骨和上颌骨的X射线成像。组织学检查包括将石蜡包埋的组织切成5微米厚的切片,然后使用常规苏木精和伊红(H&E)染色。值得注意的是,在评估过程中没有使用专门的组织化学或免疫组织化学染色.结果在我们的研究中,我们回顾了21例;9例为男性,11是女性,其中一人没有可用的性别数据。年龄范围是15-76岁,平均50年。下颌骨是最常见的受影响位置(17例;81%),而上颌骨较不常见(4例;19%)。许多CGCG病变无症状(13例;62%);8例(38%)有症状,以受影响的牙齿区域的疼痛和丰满为主要表现。在少数情况下,如棕色肿瘤(严重的甲状旁腺功能亢进)和牙源性肿瘤,例如成釉细胞瘤,在临床和影像学上怀疑。所有病例均确诊CGCG伴急性和慢性炎症。常规染色载玻片的组织学评估是使用的主要诊断工具。不需要特殊的染色或分子研究来建立最终诊断。结论我们的调查确定CGCG表现出非肿瘤性,表现出从非攻击性到攻击性倾向的一系列行为。虽然CGCG主要在下颌骨中观察到,上颌骨受累的罕见情况也有记录。重要的是,在我们的分析中,未发现与肿瘤性病变的明确关联.CGCG的临床过程趋于惰性,有些病例与牙齿受累有关。值得注意的是CGCG可以呈现模仿肿瘤状况的特征,例如成釉细胞瘤,或与全身性疾病有关的局部病变,如甲状旁腺功能亢进(棕色肿瘤)。
    Background Central giant cell granuloma (CGCG) presents as a locally invasive, intraosseous lesion characterized by the accumulation of multinucleated giant cells amidst a matrix of hemorrhage and reactive fibrous tissue that infiltrates bone trabeculae. This idiopathic non-neoplastic proliferative lesion primarily affects the mandible, typically presenting as either unilocular or multilocular radiolucencies on X-rays. Although trauma or intraosseous hemorrhages are potential triggers, the precise histogenesis and etiology remain unclear. CGCG predominantly occurs in children and young adults, with a slight female predilection. Methods and materials A retrospective analysis of 21 cases of CGCG diagnosed at the Oral Pathology/Pathology department of Temple University Hospital between 2015 and 2022 was conducted. Each case was evaluated based on various parameters, including age, gender, presenting symptoms, radiographic findings, clinical differential diagnosis, and histological confirmation. The primary radiographic technique employed for diagnosis was X-ray imaging of the mandible and maxilla. The histological examination involved cutting paraffin-embedded tissue into 5-micrometer-thick sections, which were then stained using routine hematoxylin and eosin (H&E) stain. Notably, no specialized histochemical or immunohistochemical stains were utilized in the evaluation process. Results In our study, we reviewed 21 cases; 9 were male, 11 were female, and one had no available gender data. The age range was 15-76 years, with a mean of 50 years. The mandible was the most commonly affected location (17 cases; 81%) while the maxilla was less commonly involved (4 cases; 19%). Many CGCG lesions were asymptomatic (13 cases; 62%); eight cases (38%) were symptomatic, with pain and fullness of the affected dental region being the main manifestations. In a few cases, conditions such as brown tumor (severe hyperparathyroidism) and odontogenic neoplasms, such as ameloblastoma, were suspected clinically and radiographically. The diagnosis of CGCG with associated acute and chronic inflammation was confirmed in all the cases. Histological evaluation of routinely stained slides was the main diagnostic tool utilized. No special stains or molecular studies were required to establish the final diagnosis. Conclusions Our investigation has determined that CGCG exhibits a non-neoplastic nature, displaying a spectrum of behaviors ranging from non-aggressive to aggressive tendencies. While CGCG is predominantly observed in the mandible, rare instances of involvement in the maxilla have also been documented. Importantly, no confirmed association with neoplastic lesions was identified during our analysis. The clinical course of CGCG tends to be indolent, with some cases presenting in association with impacted teeth. It\'s noteworthy that CGCG can present features mimicking neoplastic conditions, such as ameloblastoma, or localized lesions linked to systemic disorders such as hyperparathyroidism (brown tumor).
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  • 文章类型: Journal Article
    中央和周围巨细胞肉芽肿是良性实体,主要见于女性的下颌前区。通常观察到复发。其病因尚不清楚,治疗它们的最佳方法也是如此。这项研究的目的是评估发病率,治疗方法,复发率,中央和周围巨细胞肉芽肿的初步和明确的相关性。在2013年至2023年期间转诊至我们诊所并具有明确诊断为“中央性巨细胞肉芽肿”(CGCG)或“周围性巨细胞肉芽肿”(PGCG)的患者纳入研究。人口统计数据,复发率,治疗方法,病变位置,临床行为,报告中注明了大小。在这项研究中,对23例患者(14例PGCG和9例CGCG)中的30个病变进行了评估。平均随访时间为62.6个月;23例患者中有8例患有全身性疾病。虽然只有1例患者在PCGC中观察到皮质骨破坏,所有患者均发现CGCG中皮质骨破坏(p<0.05)。在这两个病变中,评估了初步和最终诊断的相关性,PGCG中的比例为50%,CGCG中的比例为77.7%。PGCG和CGCG的复发率分别为21.4%和33.3%。所有患者均采用刮宫术。额外的治疗(病灶内注射类固醇,denasumab应用,切除,和移植物应用)在5例发现CGCG的患者中进行(p=0.004)。然而,CGCG的治疗方法与复发无明显关系(p>0.05)。各种外周病变可以模拟PGCG;因此,在PGCG的治疗中,刮宫疗法可能是合适的。然而,在CGCG的某些情况下,额外的治疗方法可能更有效地预防复发和任何其他并发症.
    Central and peripheral giant cell granulomas are benign entities mostly seen in mandibular anterior region at female individuals, usually with observed recurrence. Their etiology is still unclear, as is the optimal method for treating them. The aim of this study was to evaluate the incidence, treatment methods, recurrence rates, and initial and definitive correlation of central and peripheral giant cell granulomas. Patients who were referred to our clinic between 2013 and 2023 and who had the lesions\' definitive diagnosis as \"central giant cell granuloma\" (CGCG) or \"peripheral giant cell granuloma\" (PGCG) were included in the study. Demographic data, recurrence rates, treatment methods, lesion location, clinical behaviors, and sizes were noted on the reports. A total of 30 lesions in 23 patients (14 PGCG and 9 CGCG) were evaluated in this study. The mean follow-up time was 62.6 months; 8 of 23 patients had systemic disease. While only 1 patient was observed to have cortical bone destruction in PCGC, all patients were found to have cortical bone destruction in CGCG (p < 0.05). In both lesions, the correlation of preliminary and definitive diagnosis was evaluated, and it was found to be 50% in PGCG while it was 77.7% in CGCG. The recurrence rates were 21.4% in PGCG and 33.3% in CGCG. Curettage was applied in all patients. Additional treatments (intralesional steroid injections, denasumab applications, resection, and graft application) were performed in 5 patients who were found to have CGCG (p = 0.004). However, there was no significant relation between treatment method and recurrence in CGCG (p > 0.05). Various peripheral lesions could mimic PGCG; thus, curettage therapy could be appropriate in the treatment of PGCG. Nevertheless, in some cases of CGCG, additional treatment methods could be more effective for preventing recurrence and any other complications.
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  • 文章类型: Journal Article
    中央巨细胞肉芽肿(CGCG)是涉及颌骨的相当常见的病变。CGCG可以显示出相对无害的生物学行为,或者可能显示出暗示侵袭性生物学行为的临床放射特征。迄今为止,没有组织学参数可以用来预测这些病变的行为。这项研究是为了评估血管生成参数的实用性,即,血管总面积(TVA),平均血管面积(MVA)和微血管密度(MVD),侵袭性和非侵袭性CGCG中肌成纤维细胞的密度。
    本研究是一项回顾性研究。研究中包括总共20例先前诊断的CGCG病例(10例非侵袭性病例和10例侵袭性病例)。使用标记CD34和α-SMA对切片进行免疫组织化学。对于血管参数的评估,使用图像J软件。使用Sridhara等人给出的标准,在每种情况下确定肌成纤维细胞的密度,范围为1至4。使用Mann-WhitneyU检验评估血管参数的平均值和肌成纤维细胞密度与CGCG侵袭性之间的相关性。
    Mann-WhitneyU检验的结果表明,TVA值之间的差异(P<0.001),MVA(P<0.003)和肌成纤维细胞密度,即,SMA均值(P<0.001)和SMA评分(P<0.001),两组均有统计学意义。使用判别分析获得了评估攻击性的公式。
    在CGCG的侵袭性和非侵袭性病例中,肌成纤维细胞的血管生成和密度显着不同。通过输入血管参数和肌成纤维细胞的值,可以使用获得的公式来预测CGCG病例的侵袭性。
    UNASSIGNED: Central giant cell granuloma (CGCG) is a fairly common lesion involving the jaw bones. CGCG can show relatively innocuous biological behaviour or it may show clinicoradiological features suggestive of aggressive biological behaviour. To date, there are no histological parameters which can be used to predict the behaviour of these lesions. This study was conducted to assess the utility of parameters of angiogenesis, i.e., total vascular area (TVA), mean vascular area (MVA) and microvessel density (MVD), and density of myofibroblasts in aggressive and non-aggressive CGCGs.
    UNASSIGNED: The study was undertaken as a retrospective study. A total of 20 previously diagnosed cases (10 non-aggressive and 10 aggressive) of CGCGs were included in the study. The sections were subjected to immunohistochemistry using the markers CD34 and α-SMA. For the assessment of vascular parameters, image J software was used. The density of myofibroblasts was determined in each case ranging from score-1 to 4, using the criteria given by Sridhara et al. The correlation between mean values of vascular parameters and density of myofibroblasts with aggressiveness of CGCG was assessed using Mann-Whitney U test.
    UNASSIGNED: The result of Mann-Whitney U test suggested that the differences between the values of TVA (P < 0.001), MVA (P < 0.003) and density of myofibroblasts, i.e., SMA mean (P < 0.001) and SMA score (P < 0.001), in two groups are statistically significant. The formula for the assessment of aggressiveness was obtained using discriminant analysis.
    UNASSIGNED: Angiogenesis and density of myofibroblasts significantly differ in aggressive and non-aggressive cases of CGCGs. The aggressiveness of CGCG case can be predicted using the obtained formula by entering the values of vascular parameters and myofibroblasts.
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  • 文章类型: Journal Article
    中央巨细胞肉芽肿(CGCG)是一种罕见的颌骨病变,发生在年轻人和青少年中。手术,传统治疗的支柱,与显著的发病率相关。Denosumab,RANKL的人源化单克隆抗体,在相关实体中有效,骨巨细胞瘤(GCTB),但在更惰性CGCG的经验是有限的。这项在三级转诊中心(2015-2021年)对所有denosumab治疗的CGCG进行的前瞻性观察性研究旨在评估安全性。CGCG中使用denosumab的疗效和复发风险低于GCTB。所有收到的标准化,denosumab120mg的限时疗程,根据反应逐步增加给药间隔。他们使用辐射最小化方案进行了长达75个月的随访:3个月的临床,生化和放射学评估(正骨图,锥形束CT)。八个病人,平均年龄20.5岁[IQR6],接受13次初始剂量[IQR10]的denosumab120毫克。5.5次剂量后观察到放射学反应[IQR4.5]:所有骨化和大小减少三个。七项完成治疗中有四项复发,停止后12个月观察[IQR6.5]。较大的基线尺寸,侵袭性亚型和少于12次初始剂量在复发组中更为常见.没有颌骨坏死。在接受改良剂量的患者中发生了低钙血症。这项研究代表了最大的,denosumab治疗CGCG的最多样化队列,文献中随访时间最长。它证明了低频的功效,denosumab的限时疗程,但突出了复发的风险。长期随访至关重要。
    Central giant cell granuloma (CGCG) is a rare lesion of the jaw occurring in young adults and adolescents. Surgery, the traditional mainstay of therapy, is associated with significant morbidity. Denosumab, a humanised monoclonal antibody to RANKL, is effective in a related entity, giant cell tumour of bone (GCTB), but experience in the more indolent CGCG is limited. This prospective observational study of all denosumab-treated CGCG at a tertiary referral centre (2015-2021) aimed to evaluate the safety, efficacy and recurrence risk using denosumab in CGCG at lower-frequency dosing than used for GCTB. All received standardised, time-limited courses of denosumab 120 mg with stepwise increase in dosing interval based on response. They were followed for up to 75 months using a radiation-minimising protocol: 3-monthly clinical, biochemical and radiological assessment (orthopantomograms, cone beam CT). Eight patients, median age 20.5 years [IQR 6], received 13 initial doses [IQR 10] of denosumab 120 mg. Radiologic response was seen after 5.5 doses [IQR 4.5]: ossification in all and size reduction in three. Recurrence occurred in four of seven completing therapy, observed 12 months post-cessation [IQR 6.5]. Larger baseline size, aggressive subtype and fewer than 12 initial doses were more common in the recurrence group. There was no osteonecrosis of the jaw. Hypocalcaemia occurred in one receiving modified dosing. This study represents the largest, most diverse cohort of denosumab-treated CGCG with the longest follow-up in literature. It demonstrates the efficacy of lower-frequency, time-restricted course of denosumab but highlights the risk of recurrence. Long-term follow-up is critical.
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  • 文章类型: Journal Article
    OBJECTIVE: Noonan syndrome is a rare genetic disorder characterized mainly by congenital heart disease, occasional intellectual disability, and varied orthopaedic, rheumatological and haematologic anomalies. Despite potentially serious functional consequences, joint involvement has been rarely studied in the literature. Our objective was to perform a retrospective study evaluating the prevalence and characteristics of joint involvement in Noonan syndrome.
    METHODS: We recorded articular symptoms, including their type and frequency, in patients with Noonan syndrome followed up in French hospitals. Patients were included if the diagnosis was confirmed before the age of 20 based on the van der Burgt criteria or genetic analysis. Data are presented as frequencies or medians (ranges), and patient groups were compared using chi-square or Fisher tests.
    RESULTS: Seventy-one patients were included from 4 centres. The average age was 12.5 years (range: 2-36). Musculoskeletal pain was found in 18 patients (25%) and joint stiffness in 10 (14%) located in the wrists, elbows, ankles, knees and hips, which was usually bilateral. Only one destructive form was described (multiple villonodular synovitis and a giant cell lesion of the jaw). There were no cases of systemic lupus erythaematosus (SLE) or other autoimmune arthritis. Raynaud\'s phenomenon was observed in 3 patients. Only 50% of joint complaints led to additional exploration. SOS1 mutations (P<0.05) and treatment with growth hormone (GH) (P<0.05) were the only factors significantly related to musculoskeletal pain. Patients treated with GH did not have more SOS1 mutations. Patients experiencing pain were not more likely to experience stiffness, joint hypermobility, or coagulation abnormalities.
    CONCLUSIONS: Joint manifestations were frequent in Noonan syndrome, predominant in large joints, and rarely explored. Multiple villonodular synovitis is characteristic but rare. Auto-immune disorders were not described in this cohort. A more multidisciplinary approach could be recommended for the early detection of possibly disabling rheumatologic manifestations.
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  • 文章类型: Journal Article
    回顾分析中心性巨细胞肉芽肿患者的临床和影像学特征,并复习相关文献,为中心性巨细胞肉芽肿的诊断和临床表现提供参考。
    回顾性选择7例中央性巨细胞肉芽肿患者作为研究对象,所有病例均经病理证实,并进行了相关影像学检查.所有七例病例都接受了CT扫描,3例接受了MRI扫描。将详细的临床特征与影像学发现进行比较,并根据其表现和影像学特征进行分析。
    临床特征,影像学特征因病变部位而异.CT特征包括不均匀致密的膨胀性肿块,导致骨破坏和皮质变薄。而MRI在T1和T2加权图像中具有低到等强度的特征。可能存在囊性变性,出血或含铁血黄素沉积或类骨质形成,这可能导致T1和T2信号变化。在对比研究中,病变不增强,但周围可能轻度增强。
    在T1加权和T2加权图像中,具有骨骼破坏和皮质变薄的不均匀致密的膨胀质量,并且在外周轻度增强,应考虑中枢巨细胞肉芽肿。
    UNASSIGNED: To review and analyze the clinical and imaging features of central giant cell granuloma patients and to review the relevant literatures for the diagnosis and clinical manifestation of central giant cell granuloma.
    UNASSIGNED: Seven cases of central giant cell granuloma were retrospectively selected for the study, all of which were confirmed by pathology and had relevant imaging investigations. All seven cases had undergone CT scan, three cases had undergone MRI scan. Detailed clinical features were compared along with the imaging findings and analysis was done on the basis of their presentation and imaging features.
    UNASSIGNED: The clinical features, radiologic features were varied according to the site of the lesion. CT features include unevenly dense expansile mass causing bone destruction and cortical thinning. While MRI features with low to iso-intensity in T1- and T2 weighted images. There may be presence of cystic degeneration, hemorrhage or hemosiderin deposits or osteoid formation, which can cause T1 and T2 signal changes. On contrast study, the lesion doesn\'t enhance but periphery may enhance mildly.
    UNASSIGNED: Unevenly dense expansile mass with bone destruction and cortical thinning with low to iso-intensity in T1 weighted and T2 weighted images and mildly enhance peripherally, Central giant cell granuloma should be considered.
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  • 文章类型: Journal Article
    BACKGROUND: The central giant cell granuloma(CGCG) of bone constitutes about 10% of benign jawbone lesions. It affects females more often than males, mandible than maxilla. Biological behavior of CGCG ranges from a slow growing asymptomatic swelling to an aggressive process. True giant cell tumor (GCT) should be distinguished from CGCG. The histological distinction between these lesions depends on quite subtle differences. Expression of p63 has been demonstrated in GCT of bone conversely, has not been detected in CGCG. Therefore this short study attempts to study the expression of p63 in CGCG in conjunction with clinicopathological profile of the cases reported in the institute.
    OBJECTIVE: To review all the cases of CGCGs of the jaws reported in the institute from 1998 to 2015 and study their clinicopathological profile.To study the immunohistochemical (IHC) expression of p63 in CGCG cases.
    METHODS: The retrospective study reviewed records for clinically and histopathologically diagnosed cases of CGCG from the archives of department of Oral pathology. Data was recorded and analyzed. These cases were subjected for IHC analysis for expression of p63, also RANK, RANKL in selected cases to study the nature of giant cells.
    CONCLUSIONS: This paper is an institutional experience of clinicopathological profile of diagnosed cases of CGCG. Clinicopathological findings were in concurrent with previous literature. Total number of cases was ten. Six occurred in females and four in males. Most of them occurred in the second decade, more commonly involving mandible. Three cases showed recurrence. Histologically most showed classical features. Expression of p63 showed negativity in all the cases in accordance with the previous studies. RANK and RANKL showed strong and diffuse immunoexpression in both mononuclear and giant cells. Thus study supports the finding that p63 expression can be used to differentiate between CGCG and GCT. However, more number of studies with larger sample size are required to confirm reliability of using p63 as a distinguishing marker between GCT and CGCG.
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  • 文章类型: Journal Article
    OBJECTIVE: This is a retrospective cohort study of patients with a central giant cell granuloma (CGCG) treated at a single center to assess and compare the different surgical and non-surgical approaches.
    METHODS: A cohort with a single histologically proven non-syndrome-related CGCG was selected and reviewed. Patients were allocated to group I (surgery), group II (pharmacotherapy), and group III (pharmacotherapy and surgery). The primary outcome was long-term radiologic response using computed tomography. Secondary outcomes were intermediate radiologic responses and occurrence and severity of side effects.
    RESULTS: Thirty-three subjects were included in the study. The surgical group (n = 4) included 1 patient with progression during follow-up and a relatively high post-surgical morbidity. Twenty-nine patients started on various pharmacological treatment regimens (groups II and III). Fourteen patients could be managed without additional surgery. One of these lesions showed progression during follow-up. The other 15 lesions underwent additional surgery, and none showed progression during follow-up. Interferon treatment was associated with the most side effects.
    CONCLUSIONS: Pharmacological agents have a role in the treatment of aggressive and non-aggressive CGCGs by limiting the renewed progression during long-term follow up and the extent and morbidity of surgical treatment.
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  • 文章类型: Journal Article
    Recurrence is a major problem following the treatment of aggressive central giant cell granuloma (CGCG). The aim of this study was to compare the frequency of recurrence between patients who received calcitonin nasal spray after curettage of CGCGs and those who did not. A double-blind clinical trial was designed. Patients were allocated to one of two groups: those in the calcitonin group underwent curettage and received calcitonin salmon nasal spray 200IU/day once a day for 3 months after surgery; those in the control group underwent curettage of CGCGs and received a placebo once a day for 3 months after surgery. All patients were followed for 5 years after surgery. Twenty-four patients were treated in the two groups. There was no difference in age, sex, tumour size, or tumour location between the two groups (P>0.05). Eight of the 24 patients (33.3%) had recurrences during the follow-up period: one in the calcitonin group (9.1%) and seven in the control group (53.8%). Analysis of the data demonstrated a significant difference between the two study groups (P=0.033). It appears that calcitonin nasal spray may reduce the frequency of recurrence in aggressive CGCGs in the mandible and maxilla.
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