Giant Cells, Giant Impact: A Case of Aggressive Central Giant Cell Granuloma in the Mandible.

Central giant cell granuloma (CGCG) is a bone lesion characterized by fibrous tissue containing areas of bleeding, giant cells with multiple nuclei, and trabeculae of woven bone. It is considered to be a local bone repair response, possibly triggered by inflammation, bleeding, or local injury. CGCG is more prevalent in females and can occur across a wide age range, typically diagnosed at a young age. Mandibular involvement is more common than maxillary involvement, with most lesions in the posterior region often extending into the ascending ramus. Management of aggressive CGCG can involve non-surgical (medical) and surgical treatment modalities. Surgical approaches vary from simple curettage to en bloc resection depending on various factors discussed in this case report.

Appearances can be deceptive - Innocuous swelling on the gingiva masking an aggressive lesion within the maxilla.

The central giant cell granuloma displays a varied biologic behaviour ranging from simple reactive lesions to aggressive neoplasms. The pathogenicity still remains enigmatic and needs to be differentiated from other giant cell containing lesions. Both maxilla and mandible are affected and 80% involve the region anterior to the first premolar region. CGCL arises centrally within bone, whereas PGCG is a gingival soft tissue lesion. Clinical and radiographic correlation is required to rule out a peripheral giant cell granuloma. The case described here was a rare presentation of a large epulis clinically with involvement of maxilla radiographically and was histologically diagnosed as a central giant cell lesion.

Central Giant Cell Granuloma of the Mandible: A Case Report.

This article presents a clinical case of a central giant cell granuloma (CGCG) resembling a periapical lesion of endodontic origin. A 39-year-old, otherwise healthy male patient was referred to the department of oral and maxillofacial surgery for its diagnosis and subsequent management. The patient presented with an asymptomatic, progressively increasing intraoral swelling associated with the mandibular left para-symphysis region. On radiographic evaluation, a unilocular radiolucent lesion involving 33-34 teeth was noted. An incisional biopsy presented a giant cell lesion, following which surgical curettage was done. Histopathological examination was in accordance with the diagnosis of CGCG. Therefore, it is imperative for clinicians to accurately diagnose and rule out similarly presenting lesions.

Central giant cell granuloma in the posterior region of mandible mimicking a fibro-osseous lesion and hemangioma: a case report.

BACKGROUND: A central giant cell granuloma (CGCG) is a benign, proliferative, intraosseous, and non-odontogenic lesion occurring primarily in children and young adults. On the histological level, it is characterized by numerous multinucleated giant cells scattered randomly throughout a sea of spindle-shaped mesenchymal stromal cells which are dispersed throughout the fibrovascular connective tissue stroma containing areas of haemorrhage. When it comes to radiographic features, CGCG can have an array of variations, ranging from well-defined expansile lesions to ill-defined and destructive lesions, with or without expansion. CASE PRESENTATION: This case report reviews an 11-year-old Caucasian patient with a chief complaint of slow-growing swelling involving the right posterior mandibular region. The cone beam computed tomography (CBCT) revealed an ill-defined mixed lesion mimicking both fibro-osseous lesion and hemangioma. However, microscopic examination revealed multinucleated giant cells in a fibrous stroma suggestive of central giant cell granuloma. CONCLUSION: Our intent in reporting this case is to highlight the importance of thorough clinical, radiographical and histopathological examination for accurate diagnosis and therapeutic interventions as well as to emphasize the importance of taking different possibilities into consideration when examining bony swellings in the head and neck region.

Central Giant Cell Granuloma: Negotiating the Diagnostic and Management Dilemmas.

Introduction: Giant cell lesions of orofacial region although rare in presentation, have diagnostic and treatment challenges due to overlapping clinical, radiological, and histopathological signs. Background: We happened to come across a case, which presented to us with an aggressive jaw lesion of nonodontogenic origin, mimicking a malignancy and putting us in a conundrum with regard to work up and treatment. The sequential work up not only helped us reach a definitive diagnosis but also led us the draw algorithms for diagnosis of Giant cell lesions and management of Central giant cell granuloma. Conclusion: Meticulous planning along with molecular studies helps in better delineating one giant cell lesion from other.

Incidence, treatment method and recurrence rate in giant cell granulomas: Retrospective study.

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.

Evaluation of the relationship between the expression of AgNOR and Ki67 with the recurrence rate in central granulomatous giant cell lesions: A case-control.

OBJECTIVES: Giant cell granuloma is a local nonneoplastic lesion that is divided into two categories, based on its site of occurrence: Central and peripheral giant cell granuloma. Central giant cell granuloma is an intraosseous lesion that has a tendency to recure even in surgically treated cases. Several studies have proven that there is an association between different lesions clinical behavior and their histological features. The aim of this study was to evaluate the expression of AgNOR and Ki67 in lesions with and without recurrency. MATERIAL AND METHODS: Files and records of 35 patients who had been histologically diagnosed with central giant cell granuloma were investigated. Histological features were studied after performing AgNOR staining and Ki67 marker. The data were analyzed by chi-square, Fisher, and T-test. RESULTS: Acquired data indicated that the count of AgNOR staining and Ki67 marker was significantly higher in lesions with recurrency than the lesions with no recurrency. The same results were attained from Ki67 intensity. CONCLUSION: The current study indicated that AgNOR staining and Ki67 marker have prognostic value in predicting recurrency of central giant cell granuloma lesions.

Denosumab as treatment of central giant cell granuloma of the jaws. a scoping review.

Denosumab has been considered a treatment option for Central Giant Cell Granuloma (CGCG) a benign locally aggressive osteolytic lesion of the jaws. This study aimed to perform a scoping review of CGCG treated with Denosumab. The research question was: What is Denosumab's effectiveness in treating CGCG of the jaws? Studies that used Denosumab as a treatment for CGCGs in the jaws were selected following PRISMA-ScR guidelines, using Pubmed/Medline, Scopus, and Springer Link databases, among others. Demographics, clinical information, dosing, efficacy, adverse drug reactions (ADRs), and imaging tests used to assess the evolution of the lesions were extracted. Twenty-one studies were selected. Sixty patients with a mean age of 23.2 years were treated with Denosumab, 42% with 120 mg subcutaneously monthly, additional doses on days 1, 8, and 15 for month 1 in adults. In children, dosing was adjusted by weight to 60 or 70 mg. To avoid ADRs 500 mg of calcium and 400 IU of vitamin D orally were used. Initial effective response was reported after 1-3 months, with recurrence of 19.6% and ADRs in 74% of cases. Denosumab is effective for CGCG with monthly subcutaneous doses of 120 mg, 60 or 70 mg in patients < 45 or 50 kg for ≥ 12 months with calcium and vitamin D supplementation until remission changes are observed. Extensive or refractory lesions were the main indications. Common ADRs were hypo and hypercalcemia. Further studies are needed to define dose and supplementation protocols to avoid ADRs during and after therapy.

Central giant-cell granuloma in a patient with neurofibromatosis type 1: 7 years of follow-up.

Neurofibromatosis type 1 (NF1) is an autosomally dominant tumor suppressor syndrome and multisystem disease. Central giant-cell granulomas (CGCGs) can be seen in patients with NF1. A 21-year-old female was diagnosed with two CGCGs, one in the mandible and then one in the maxilla, in a 7-year period. Increased incidence of CGCGs in NF1 patients was thought to be caused by an underlying susceptibility to developing CGCG-like lesions in qualitatively abnormal bone, such as fibrous dysplasia. However, germline and somatic truncating second-hit mutations in the NF1 gene have been detected in NF1 patients with CGCGs, validating that they are NF1-associated lesions. Oral manifestations in patients with NF1 are very common. Knowledge of these manifestations and the genetic link between NF1 and CGCGs will enhance early detection and enable optimal patient care.

Imaging of Fibro-osseous Lesions and Other Bone Conditions of the Jaws.

This review directs the focus on the imaging features of various fibro-osseous lesions and other bone lesions that can be of similar presentation. Broad diagnosis of "fibrous osseous lesion" may culminate in improper treatment and management. Radiographic discriminating factors between these entities are highlighted and summarized to improve the diagnostic process when encountering these lesions.

Multiple central giant cell granuloma of the jaws: diagnostic signposts of Noonan syndrome and RASopathy.

Noonan syndrome (NS) is a phenotypically variable inherited multi-system disorder. Maxillofacial findings can be diagnostic, especially in the evaluation of discrete facial dysmorphia. Diagnostic landmark findings of therapeutic relevance for the jaws such as central giant cell granuloma (CGCG) are rare in NS. However, recent molecular genetic studies indicate that these rare, benign lesions are neoplasms and more common in specific syndromes grouped under the umbrella term RASopathies. A specialist surgical diagnosis can be helpful in identifying the underlying disease. This report outlines diagnosis and treatment of a case of CGCG for which jaw diagnosis became the key to identifying a syndromic disease.

Central giant cell granuloma formation in an edentulous area in the posterior portion of mandible: A case report.

INTRODUCTION: Central Giant Cell Granuloma (CGCG) is a non-neoplastic benign lesion. It is primarily observed in the maxilla and mandible, with the mandible being the more reported site of the lesion. The lesion often manifests in the anterior regions of the mandible, extending occasionally across the midline. This case reports a rare presentation in the posterior portion of the mandible, in an edentulous area. PRESENTATION OF CASE: A 33-year-old female with a history of extraction of teeth 36 and 37 six months ago presented with a main complaint of a mass in the oral cavity. The oral examination revealed an expansive multilocular mass (4 × 3 cm) located on the alveolar ridge in the left posterior portion of the mandible, extending around tooth 33 with an intact masseter muscle. The histopathological findings were consistent with CGCG. Consequently, the lesion was surgically removed with no clinical or radiological recurrence observed during the 4-month post-operative follow-up. DISCUSSION: While previous reports of CGCG in the posterior portion of the jaw showed destructive lesions that caused mandibular ramus destruction along with swollen masseter muscle, this case reports no involvement of the masseter muscle. Also, while some studies linked CGCG to tooth-bearing regions, our case suggests a possible traumatic link even after extraction. CONCLUSIONS: This case presents a rare CGCG occurrence in the posterior jaw, notably without masseter muscle involvement. It also indicates that CGCG can manifest in edentulous regions.

Bony Canal Method of Dexamethasone Injections in Aggressive Form of Central Giant Cell Granuloma-Case Series.

Central Giant Cell Granuloma constitutes approximately 7% of benign tumors of the jaws. The aggressive form of CGCG clinically behaves like a classic semi-malignant neoplasm. In the literature, the suggested method of treatment of aggressive forms of CGCG is curettage or resection with the margin of 0.5 cm. Surgical treatment, especially in the developmental age, entails disturbances in the growth and differentiation of tissues and deforms and disturbs the functioning of the stomatognathic system. Alternative treatment methods of the CGCG presented in this article lead to the patient avoiding a mutilating procedure and improve their quality of life. The aim was to present alternative method of treatment of aggressive forms of Central Giant Cell Lesion of the jaws-injections of dexamethasone into the tumor mass through drilled bony canals. Here, we present the three cases of aggressive forms of CGCG of jaws treated with dexamethasone injections into the tumor mass. Two cases resulted in regression of the tumor, which was confirmed in histologic evaluation after remodeling surgery. Those two patients were uneventful and showed no signs of tumor recurrence at 8 and 9 years of thorough follow-up, respectively. The third patient was qualified for the mandible resection due to the enlargement of the lesion and destruction of the cortical bone. According to our observations, if the proper patient discipline, and thorough, careful clinical and radiological examinations are provided, the dexamethasone injections could be a recommended method of treatment of intraosseous giant cell granuloma. The indication is restricted to the cases with preserved bony borders despite deformation. Additionally, leaving vital teeth in the lesion is also possible.

Significant association between FGFR1 mutation frequency and age in central giant cell granuloma.

Central giant cell granulomas (CGCG) are rare intraosseous osteolytic lesions of uncertain aetiology. Despite the benign nature of this neoplasia, the lesions can rapidly grow and become large, painful, invasive, and destructive. The identification of molecular drivers could help in the selection of targeted therapies for specific cases. TRPV4, KRAS and FGFR1 mutations have been associated with these lesions but no correlation between the mutations and patient features was observed so far. In this study, we analysed 17 CGCG cases of an Italian cohort and identified an interesting and significant (p=0.0021) correlation between FGFR1 mutations and age. In detail, FGFR1 mutations were observed frequently and exclusively in CGCG from young (<18 years old) patients (4/5 lesions, 80%). Furthermore, the combination between ours and previously published data confirmed a significant difference in the frequency of FGFR1 mutations in CGCG from patients younger than 18 years at the time of diagnosis (9/23 lesions, 39%) when compared to older patients (1/31 lesions, 0.03%; p=0.0011), thus corroborating our observation in a cohort of 54 patients. FGFR1 variants in young CGCG patients could favour fast lesion growth, implying that they seek medical attention earlier. Our observation might help prioritise candidates for FGFR1 testing, thus opening treatment options with FGFR inhibitors.

Rare hybrid tumor of odontogenic fibromyxoma and central giant cell granuloma in maxilla: First reported case.

Fibromyxoma is a locally aggressive rare benign tumor of mesenchymal origin with or without odontogenic epithelium. The etiology of this tumor remains unknown and it is responsible for approximately 3-8% of all cysts and tumors. Another locally destructive benign lesion is central giant cell granuloma (CGCG) which contains osteoclast-like multinucleated giant cells. CGCG accounts for about 7% of all benign jaw tumors, which usually affects younger females. A hybrid lesion with histologic features of both central fibromyxoma and CGCG has not been reported in the literature so far. In the present article, we report the first case of a hybrid tumor comprising odontogenic fibromyxoma with CGCG in a female along with a brief review of its clinical presentation, radiographic features, histological features, and management.

Central Giant Cell Granuloma of Maxilla: A Case Report.

Central giant cell granuloma formerly called giant cell reparative granuloma is a non neoplastic proliferative lesion of an unknown aetiology. It occurs most commonly in mandible, but can also occur in maxilla. The case described here involved maxilla which was treated with surgical excision.

Rare Case of a Peripheral Giant Cell Granuloma of the Jaw as First Manifestation of Primary Hyperparathyroidism.

Giant cell granulomas (GCG) are uncommon benign tumor-like lesions mostly arising in the oro-facial area. They are more common in women and occur in patients younger than 30 years. Lesions restricted to the bone are referred to as central giant cell granulomas (CGCG), and those developing primarily on soft tissues are termed peripheral giant cell granulomas (PGCG). Both types are histologically identical. The combination of both clinical examination and radiography allows for the differentiation of those two variants. On rare occasions GCG, and especially CGCG, may develop in relation to hypercalcemia linked to hyperparathyroidism (HPT). In those cases, the GCG treatment prognosis is closely linked to the HPT management. Therefore, patients diagnosed with a GCG must be investigated to search for an HPT. Reported herein is a rare clinical case of a mandibular PGCG which led to the diagnosis of primary HPT.

Novel regimen of combined intralesional triamcinolone and salmon calcitonin nasal spray to treat a large central giant cell granuloma.

About 10% benign tumors of the jaw are known to be central giant cell granulomas (CGCGs) affecting mandible more than maxilla. They are more commonly seen among young females, mean age range being 10-25 years. The aggressive variants of CGCG require surgical intervention, leaving colossal disfiguring defects. This being the reason for many nonsurgical alternative therapies as calcitonin injections and nasal spray, intralesional steroid injections and subcutaneous interferon injections advocated for its management. Although the exact success rate of using these nonsurgical therapies are not fully known, they provide the advantage of being conservative in nature, as majority of the patients are young adults. This lack of accurate regimen is due to paucity of randomized control trials and systematic reviews addressing the topic. This manuscript attempts to present a novel regimen protocol which was followed for a case of CGCG, right mandible on a 22-year-old female patient, for a period of 1.5 years and trailed by a follow-up of 2 years.

A Unique Case of Aggressive Central Giant Cell Granuloma in a 10-Year-Old Boy With 16p13.11 Microdeletion Syndrome.

Central giant cell granuloma (CGCG) is a rare disease characterized by sporadic, benign, intraosseous mandibular lesions of unknown etiology. Histologically, these lesions are indistinguishable from brown tumors of hyperparathyroidism and cherubism, and occasionally have been associated with different syndromes raising a question for genetic etiology. The CGCG has varied presentation ranging from nonaggressive and indolent to aggressive, destructive, and recurrent, often posing diagnostic and therapeutic challenges. Herein, we present the first case of a 10-year-old boy with CGCG and 16p13.11 microdeletion syndrome, highlight the diagnostic challenges inherent to this heterogeneous disorder, and discuss the genetics and treatment approaches of these complex lesions.

Recurrent Mandibular Giant Cell Lesion in Neurofibromatosis Type 1: Second Hit Mutation on the NF1 Gene in the Osseous Lesion.

BACKGROUND/AIM: In the autosomal dominant hereditary disease neurofibromatosis type 1 (NF1), lesions of the jaw develop in isolated cases, which are diagnosed as central giant cell granuloma (CGCG). This study aimed to clarify the genetic basis of a bone lesion in a syndromic patient. CASE REPORT: The NF1 patient had developed a CGCG that recurred after local excision. Blood and tumor tissue were studied for NF1 mutations using advanced molecular genetic methods. Examinations of blood and tumor tissue provided evidence of the constitutive mutation in both samples. A further mutation was detected in the tumor, which was interpreted as a somatic mutation. The detection of somatic mutation in the tissue was successful both on native and routinely fixed material. CONCLUSION: The study supports current assessments of CGCG as a benign neoplasm. In NF1 patients, the phenotype seems to imply bi-allelic loss of the NF1 gene. The detection of both mutations in routinely fixed tissue allows studies of archived tissue samples with this diagnosis.