Central giant cell granuloma of the jaws-long-term clinical and radiological outcomes of surgical and pharmacological management.

OBJECTIVES: To compare long-term results of different treatment modalities in central giant cell granuloma of the maxillofacial-skeleton. Primary resection may result in major defects. Alternative treatments include pharmacological agents. As yet there has been no consensus on the use of the variety of treatment options, and few studies have reported clarifying long-term results. MATERIALS AND METHODS: This retrospective study on 22 patients with 25 lesions evaluated clinical, radiological and histological features, treatment preformed and lesion recurrence. Success was defined as regression/calcification and failure as recurrence, progression or un-responsiveness. RESULTS: Of the presenting patients, 77% were under age 40. Lesion prevalence was higher in the anterior mandible and left posterior maxilla. Most cases exhibited pain, tooth-mobility or mucosal-expansion. The appearance was predominantly unilocular in the maxilla and multilocular in the mandible, which also exhibited higher prevalence of cortical perforation. Up to 80% of lesions were classified as aggressive. Intralesional steroids/calcitonin were used in 7 cases. Mean follow-up was 39.8 months. Two cases showed recurrence. In 71% of the cases treated pharmacologically, calcification/regression were observed. CONCLUSIONS: Our analysis indicates better outcomes using a combined approach, including both pharmacological and surgical treatments in large aggressive lesions. Pharmacological treatment resulted in decreased size or well-defined lesions, thus reducing the need for extensive bone resection. Dual treatment with corticosteroids and calcitonin showed no superior outcomes, but a larger cohort should be assessed. CLINICAL RELEVANCE: There are several protocols for treatment of central-giant-cell-granuloma lesions, but most are not fully established. It is important to report results that contribute to the establishment of proven protocols. This report attempts to establish the relevance of the combined approach: pharmacological treatment followed by surgical resection.

Giant Cell Tumor and Giant Cell Reparative Granuloma of Bone of the Head: CT and MR Imaging Findings.

BACKGROUND: This study aimed to determine the features and differentiation of Giant Cell Reparative Granuloma (GCRG) and Giant Cell Tumor (GCT) of the head on CT and MRI. METHODS: This retrospective study included six patients with histopathology-confirmed head GCRG and 5 patients with histopathology-confirmed head GCT. All images were independently reviewed by two radiologists. The growth pattern, bone changes, MRI signal intensity, enhancement patterns and other image features were recorded. All patients received CT scans and MR images. RESULTS: All the lesions were located centrally in the bone. Osteolytic bone destruction and expansive growth patterns were observed on CT images. Four of six cases broke the cortical bone with residual cortical bone, and the last two showed a thin cortex in GCRG. Five cases broke the cortical bone with residual cortical bone in GCT. There were enhancing septations in GCT lesions on contrast- enhanced T1-Weighted Images (T1WI) while enhancing septations were not present in GCRG cases. The size of GCT lesions was larger than that of GRCG. GCRG and GCT showed iso-low signals on T1WI and iso-high signals on T2-Weighted Images (T2WI). There was a case with cystic or necrotic lesions in each of the two types of lesions. Osteolytic bone destruction and expansive growth patterns were observed in GCTs and GCRGs. CONCLUSION: The size of the GRCG lesion was smaller than that of the GCT. The presence of enhancing septations and the size of the lesion may distinguish GCTs from GCRG.

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.

Dermoscopy of annular elastolytic giant cell granuloma.

Annular elastolytic giant cell granuloma is an uncommon granulomatous cutaneous disease that usually affects sun-exposed skin. Non-scarring alopecia is a possible presentation. Although histopathology is mandatory for the diagnosis, dermoscopy may help to narrow down the clinical differential diagnosis. The authors report a case of annular elastolytic giant cell granuloma in the scalp of a female adult patient, showing multiple yellowish/orange follicular dots in a diffuse erythemato-whitish background in the dermoscopy.

Imaging Features of Craniofacial Giant Cell Granulomas: A Large Retrospective Analysis from a Tertiary Care Center.

BACKGROUND AND PURPOSE: Craniofacial giant cell granulomas are rare lesions with varied appearances on imaging. We aimed to describe the imaging features of giant cell granulomas of the craniofacial bones. MATERIALS AND METHODS: A retrospective analysis of the clinical features and imaging findings of 20 histopathology-proved cases of craniofacial giant cell granulomas, dating from 2006 to 2022, was performed. RESULTS: Of the 20 cases, 10 each were seen in men and women. The epicenter of the lesions varied in location: in the maxilla in 8 patients, in the mandible in 5, in the temporal bone in 3, in the sphenoid/clivus in 3, and in the orbit in 1 patient. On the radiographs, the lesions appeared well-circumscribed, expansile, and lytic. On CT, the lesions were predominantly multiloculated, with thin septa, a soft-tissue component, and with expansion and remodeling of the underlying bone. On MR imaging, the solid component of the lesions was isointense on T1WI and hypointense on T2WI, with heterogeneous enhancement of the solid component and rim enhancement of the locules. Fluid-fluid levels were present in 3 patients. CONCLUSIONS: Giant cell granulomas commonly present as locally aggressive, expansile, multiloculated lytic lesions, with solid as well as cystic areas. The solid component is typically hypointense on T2WI. Certain key imaging features of giant cell granulomas can aid the radiologist in narrowing the differential diagnosis.

[Experience with Denosumab in central giant-cell granuloma]. / Experiencia con Denosumab en granuloma central de células gigantes.

INTRODUCTION: Central Giant Cell Granuloma is an infrequent bone lesion located mainly in the maxillary bone. The main treatment is surgery with wide margins, so it sometimes causes great morbidity and esthetic al terations. Denosumab, a RANK-ligand inhibitor monoclonal antibody, has been presented as a valid therapeutic alternative in the treatment of these lesions. OBJECTIVE: to describe the clinical and radio logical response after treatment with Denosumab in a patient with unresected giant cell granuloma. CLINICAL CASE: 12-year-old boy who consulted due to a 24-hour maxillary swelling, without other associated symptoms. Examination revealed a tumor in the upper left maxilla with bulging of the ip- silateral gingiva. A CT scan was performed which showed a large expansive intraosseous lesion in the maxillary alveolar ridge. The biopsy of the lesion was compatible with Central Giant Cell Granuloma. Due to the size and location of the lesion, initial treatment with Denosumab, a human monoclonal antibody with action on RANK-ligand, was indicated. After 10 months of treatment, the patient showed a favorable clinical and radiological response, with a size decrease of the lesion and metabolic activity. As an adverse effect, the boy presented mild hypocalcemia, resolved after supplementation with calcium. CONCLUSION: the use of Denosumab as the first line of treatment in Giant Cell Granu loma may be an adequate therapeutic option in adolescents with lesions that are difficult to resect.

Denosumab for central giant cell granuloma in an Australian tertiary paediatric centre.

BACKGROUND: Central giant cell granulomas (CGCG) are rare osteolytic, benign but often locally aggressive tumours of bone. Surgical curettage may not be possible in extensive lesions and resection carries high morbidity, especially in growing children, and previous medical therapies have had variable efficacy and high recurrence rates. Interruption of receptor activator of nuclear factor-kappa B ligand (RANKL) signalling holds promise as an effective therapeutic strategy for these tumours. AIMS: To evaluate the efficacy and safety of our protocol for denosumab treatment of CGCG in children. METHODS: Retrospective review of 4 patients treated with denosumab using a standardised protocol for CGCG in a tertiary paediatric centre. Denosumab 70 mg/m2 was given 4-weekly, followed by 2 doses of zoledronate 0.025 mg/kg, aimed at preventing rebound hypercalcaemia. RESULTS: Treatment of CGCG resulted in metabolic remission in all patients, but recurrence, detected by positron emission tomography (PET), occurred at 6 months in three patients and 12 months in one patient. Three patients developed symptomatic hypercalcaemia 4-5 months and one patient asymptomatic hypercalcaemia 7 months after cessation of denosumab, with 3 requiring additional bisphosphonate treatment. CONCLUSIONS: Denosumab produced a radiological and metabolic response in our patients, but metabolic recurrence occurred in all patients. PET imaging was effective for monitoring treatment response and early detection of recurrence. Incidence of rebound hypercalcaemia in this paediatric cohort was high. We present proposed changes to our protocol with the aim of producing sustained remission and preventing rebound hypercalcaemia.

Central odontogenic fibroma in association with brown tumor of hyperparathyroidism in a patient with neurofibromatosis type 1.

We present a patient with bone abnormalities and a myriad of lesions secondary to his redeveloping renal failure and neurofibromatosis type 1 (NF1). A 21-year-old male renal transplant recipient with NF1 presented with painless masses and large, irregular radiolucent lesions in the maxilla and mandible. After histologic examination, the lesion was diagnosed as a central odontogenic fibroma (COdF) in association with a central giant cell lesion, most consistent with brown tumor of hyperparathyroidism. The bone changes were interpreted to be highly suggestive of renal osteodystrophy. Around 30 cases of hybrid central giant cell granuloma-like lesion in association with central odontogenic fibroma have been reported. This, to our knowledge, is the first reported case of brown tumor in association with COdF. Our case provides further evidence of the giant cell component as the initiating entity in these hybrid lesions.

Granuloma central a células gigantes agresivo, de presentación atípica: reporte de un caso / Aggressive central giant cell granuloma: report of an atypical case

El granuloma central a células gigantes (GCCG) es una lesión osteólitica poco frecuente, no odontogénica, benigna, localizada y potencialmente agresiva, caracterizada por la presencia de células gigantes en un estroma vascular. Se presenta mayoritariamente en mujeres, más frecuentemente en las dos primeras décadas de vida. Radiográficamente el GCCG se puede observar desde una imagen radiolúcida de aspecto quístico, a una imagen multilocular extensa de límites pocos definidos. De acuerdo a la presentación clínica, radiográfica e histológica del GCCG, se ha propuesto un tipo agresivo caracterizado por crecimiento rápido, dolor, parestesia, expansión y perforación de corticales, rizalisis y alta tasa de recidiva. Existe controversia respecto a la etiología. Si bien está descrito como una lesión de carácter reparativo asociada a trauma, se observan características potencialmente destructivas, expansivas e infiltrativas. Se presenta un caso de GCCG agresivo, de presentación atípica en relación con la edad, sexo del paciente y ubicación de la lesión. (AU)

Central giant cell granuloma (CGCG) is an infrequent benign, localized, potentially aggressive, non-odontogenic osteolytic lesion, characterized by the presence of giant cells in a vascular stroma. CGCG is seen mainly in women, more frequently in the first two decades of life. Radiographically, CGCG can be seen from a cystic-like radiolucent image, to an extensive multilocular image, with less defined margins. According to clinical, radiographic and histological aspects of CGCG, an aggressive type has been proposed, which is characterized by rapid growing, pain, paresthesia, expansion/perforation of cortical bone, rizalisis, and high rates of recurrence. Of controversial etiology, CGCG has been described as a reparative lesion, associated to trauma. However, potentially destructive, expansive and infiltrative characteristics have been observed in CGCG. An atypical case of an aggressive CGCG is presented. (AU)

A rare mandibular neoplasm: case report of a Central Giant Cell Granuloma.

Mandible can be affected by a great variety of neoformations, like aneurysmal bone cyst, odontogenic myxoma, CGCG (Central Giant Cell Granuloma), GCT (giant cell tumor), sarcoma, ameloblastoma, lymphoma, ossifyng fibroma, odontogenic mixoma, granuloma, arteriovenous malformations and Schwannoma. Occasionally is not possible to find clinical or radiological distinctive findings so is usefull to perform additional exams, think about rare disease and perform an explorative surgical treatment which can be adapted to the intraoperatory findings. This attitude may help to reduce overtreatment but also to be radical especially in case of rare condition like the case presented: a Central Giant Cell Granuloma of the jaws. In this case report the authors present a 19-year-old female with a slowly enlarging, painfull swelling on the left side of the lower jaw. Ortopantomography exam revealed an osteolytic bone formation confirmed by Tomographic Dental Scan, MRI and Eco-Doppler exam. No one of these procedures, however, allowed to characterize the neoformation. For that reason was planned immediately an explorative surgical treatment, instead of an agosbiopsy. Macroscopic free margins resection provided radicality on one side and saved much bone tissue as possible on the other; morever it would have permitted to be more demolitive with a further procedure if the histopathological examination of specimen didn't show complete neoformation removal. KEY WORDS: Central Giant Cell Granuloma, Rare Mandibular Neoplasm, Explorative Surgical Treatment.

Blindness Secondary to Orbital Giant Cell Granuloma Mass-Effect in Noonan Syndrome With Return of Vision Following Surgical Decompression.

ABSTRACT: Noonan syndrome is a rare, autosomal dominant disorder encompassing multiple congenital defects, as well as association with solid tumor and lesion development. The authors present a 26-year-old female with known Noonan syndrome and ongoing complaint of worsening unilateral vision, progressing to vision loss due to lesion mass effect. Decompressive surgery was performed, restoring patient's vision to baseline immediately postoperative. The lesion was confirmed to be giant cell granuloma. In this paper we discuss the unique presentation of vision loss due to orbital giant cell granuloma in Noonan syndrome with postoperative return of vision; the importance of a multi-disciplinary team evaluation, thorough preoperative clinical and image-based work up, intraoperative findings, postoperative outcome, and complexity of definitive management.

Peripheral Giant Cell Granuloma of Posterior Maxilla in a 9-Year Child.

Peripheral giant cell granuloma is a relatively uncommon benign reactive gingival lesion of the oral cavity. A 9-year boy presented with a painless, slow-growing, reddish-blue, soft tissue lesion on attached gingiva adjacent to maxillary right first and second premolars, which was interfering with eating. A periapical radiograph demonstrated focal alveolar bone loss and slight teeth displacement adjacent to the lesion. Diagnosis of peripheral giant cell granuloma was made through clinical and radiographic evaluation, by its typical presentation and correlation with histopathologic findings. Complete excision was carried out down to the underlying bone. A follow-up visit was scheduled after 7 days and deep scaling was performed. There was no recurrence three months post-excision. Timely detection and excision of this lesion is important to avoid future dentoalveolar problems. Key Words: Giant cell granuloma, Benign, Peripheral, Child.

Radiological features of central giant cell granuloma: comparative study of 7 cases and literature review.

OBJECTIVE: 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. METHODS: 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. RESULTS: 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. CONCLUSION: 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.

Denosumab for the management of central giant cell granuloma of the jaws-a case series.

Denosumab has been suggested as a medical treatment for central giant cell granuloma of the jaws. This study included eight patients, seven female and one male, aged between 19 and 32 years, with biopsy-proven central giant cell granuloma of the mandible. The patients were treated with subcutaneous injections of 120 mg of denosumab in a regime consisting of three injections at weekly intervals followed by five injections at monthly intervals over a 6-month period. They were followed up for between 60 and 71 months clinically and radiographically with panoramic radiographs and cone beam computed tomography scans. All of the lesions became calcified radiographically and asymptomatic clinically. They did not reduce in size, but to date only one patient has requested surgical remodeling. There has been no recurrence or regrowth in over 5 years.

Central giant cell granuloma of the maxilla: Long-term follow-up of a patient treated with an adjuvant corticosteroid.

BACKGROUND: Central giant cell granuloma (CGCG) is one of the most intriguing lesions of the jaws and its nature has not yet been fully elucidated. Clinically, some CGCG behave more aggressively, while others have an indolent course. In cases of aggressive CGCG of the maxilla, effective personalized therapies are worth understanding. CASE REPORT: We report here a challenging case of aggressive CGCG in a 15-year-old girl which was misdiagnosed as an endodontic lesion. Radiographically, a large osteolytic lesion involving the hard palate from the central incisor to the second premolar, extending into the nasal cavity, with loss of the lamina dura and cortical resorption was observed. The lesion expanded aggressively after extensive curettage. With possible mutilation and defects due to a more radical approach to the lesion, treatment with systemic prednisone and intralesional triamcinolone hexacetonide associated with a calcitonin nasal spray was instituted. The decision in favor of this therapeutic strategy was made after careful immunohistochemical analysis of calcitonin and glucocorticoid receptors. The H-score for the staining of glucocorticoid and calcitonin receptors in multinucleated giant cells was 222 and 153.6, respectively. The lesion reduced in size, and no adverse effects associated with medications were observed. Another curettage was performed, and only fibrous connective tissue was found. The patient is in follow-up for 11 years without evidence of recurrence. CONCLUSION: Pharmacological agents hold clinical promise in cases of aggressive CGCG affecting the maxilla of pediatric patients. Investigating the expression of calcitonin and glucocorticoid receptors in order to plan treatment is very helpful in the decision to manage aggressive CGCG.

Pediatric Mandibular Central Giant Cell Granuloma: Neoadjuvant Immunotherapy to Minimize Surgical Resection.

ABSTRACT: Central giant cell granuloma (CGCG) is a relatively uncommon benign bony lesion accounting for approximately 7% of all non-neoplastic lesions of the jaw. The clinical behavior of CGCG can vary from a slow-growing, painless lesion to fast-growing and locally destructive. When such a lesion involves the mandible, this can be quite debilitating for the patient, inhibiting oral intake and requiring an extensive resection and bone graft reconstruction. The authors present a case of effectively decreasing the surgical morbidity associated with a large and rapidly growing CGCG of the mandible in a pediatric patient. Neoadjuvant immunotherapy with denosumab (human monoclonal antibody) facilitated treatment of the tumor without the need for a large resection which would have otherwise necessitated a vascularized bony mandibular reconstruction. Consideration of neoadjuvant medical management of CGCG as the primary treatment is advocated.

A systematic review of the clinical and radiographic features of hybrid central giant cell granuloma lesions of the jaws.

OBJECTIVE: Central giant cell granuloma (CGCG) can coexist with other benign lesions of the jaw. These hybrid lesions are diagnostically challenging to both oral pathologists and radiologists. This work systematically reviews the clinical and radiographic features of hybrid-CGCG lesions in the jaws. MATERIALS AND METHODS: Three reviewers conducted an electronic search of five databases for histologically diagnosed hybrid-CGCG lesions in human jaws. RESULTS: Thirty-four of 1224 articles met the inclusion criteria. Of 39 hybrid-CGCG lesions, 14 (35.9%) were central odontogenic fibroma, 11 (28.2%) were central ossifying fibroma, seven (17.9%) were fibrous dysplasia, and seven (17.9%) were other bone conditions. There were 22 females and 17 males with a mean age of 30.5 ± 19.9 years. 89.5% of hybrid-CGCG lesions were well defined, 57.9% were non-corticated, 60.5% were radiolucent, and 66.7% were in the posterior mandible. Most hybrid lesions affected the cortical plates by thinning, expansion, or perforation (93.1%), displaced, or resorbed teeth (60%). CONCLUSION: The radiographic features of hybrid-CGCG lesions vary according to the concurrent bony lesion. Hybrid-CGCG lesions altered the radiographic appearance with the following entities: fibrous dysplasia, melorheostosis, and Paget's disease. Optimal imaging modalities are crucial to detail radiographic features and direct representative biopsy of suspicious sites that may host a CGCG hybridisation.