Annular elastolytic giant cell granuloma in a woman with metabolic syndrome.

Annular elastolytic giant cell granuloma (AEGCG) is a rare granulomatous skin condition. It belongs to a group of skin and elastic fiber disorders. When it affects sun-exposed skin, it is also called actinic granuloma. The etiology and pathogenesis are still debated. However, sun-induced actinic damage to elastic fibers is acknowledged as the primary triggering factor, though the pathogenesis of instances in sun-covered areas is unknown. The most commonly linked systemic illness is diabetes mellitus. Different case reports show an association of this disease with hematological conditions, infections, sarcoidosis, and protoporphyria. Multisystemic involvement was also reported in a case. The disease is clinically recognized by erythematous non-scaly annular patches and plaques with raised borders and hypopigmented or skin-colored centers, sometimes atrophic. It is usually asymptomatic or mildly itchy. The presence of an inflammatory infiltration with non-palisading granulomas, multinucleate large cells, elastin degradation, and elastophagocytosis, as well as the absence of necrobiosis and mucin, are histopathological characteristics. We report a 5-year history of annular elastolytic giant cell granuloma in a 66-year-old woman with a history of type two diabetes mellitus, hypertension, and fatty liver disease (steatosis). She presented with asymptomatic polymorphic erythematous skin lesions mainly in sun-exposed areas.

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.

Cervical-Thoracic Epidural Giant Cell Reparative Granuloma: Case Report and Review of the Literature.

BACKGROUND: Giant cell reparative granuloma (GCRG) is a rare benign tumor. The jawbone is the most common site of occurrence, followed by sphenoid bone, craniofacial bone, hand and foot bones. The etiology of GCRG is unknown but may be related to an intraosseous hemorrhage following trauma. Despite its benign nature, it could be locally aggressive. To our knowledge, no spinal epidural GCRG case has been reported. CASE DESCRIPTION: A case of man aged 32 years who presented with upper right limb numbness and weakness. Computed tomography showed a round soft tissue mass in the spinal canal at the C7-T1 level. The mass showed isointensity on T1-weighted images, hypointensity on T2-weighted images, and significant enhancement on postcontrast T1-weighted images. The mass localized in the epidural space and was surgically resected. The histologic diagnosis was consistent with GCRG. CONCLUSIONS: Spinal epidural GCRG is rare and is hardly considered in the differential diagnosis. Preoperative diagnosis of GCRG is challenging, and the definitive diagnosis could only be made by pathological examination. Surgical resection is probably an effective therapy for relief of symptoms.

Esthetic Management of Peripheral Giant Cell Granuloma Affecting a Dental Implant in the Esthetic Zone.

INTRODUCTION: A size increase of the gingiva can be a manifestation of gingival disease. While gingival lesions can affect periodontal tissue, only few reports described the association between peripheral giant-cell granuloma (PGCG) with dental implants and their clinical management. Their clinical relevance is of a great interest since some lesions may lead to extensive bone resorption, esthetic alterations, or even tooth/implant loss. To the author's best knowledge, no specific guidelines for the treatment of PGCG affecting dental implant in the esthetic zone have been reported. The aim of this paper was to report an extensive PGCG associated with a dental implant in the esthetic area and its surgical management. CASE PRESENTATION: A 39-year-old suffered from concussive trauma on her anterior maxillary dentition leading to an expanded, purplish soft tissue lesion surrounding the implant provisional crown on right central maxillary incisor. After complete lesion excision, the implant neck was carefully and gently debrided. Histological analysis confirmed the diagnosis of PGCG. The patient was then evaluated weekly for the first 3 weeks then monthly. No episodes of recurrence were identified. After 16 weeks of undisturbed healing, a soft tissue augmentation via tunnel technique was elected as the first treatment option to improve esthetics. Final implant restoration was delivered. Follow up at 22 months from biopsy excluded any recurrence of PGCG with stability of peri-implant soft tissues. CONCLUSION: Excisional biopsy, careful removal of irritating factors, monitoring, and connective tissue grafting can esthetically improve a malpositioned implant and showed no recurrence over a period of 22 months.

Oral pulse granuloma and oral pulse granuloma associated with odontogenic keratocyst: Two clinical cases and a review of the literature.

Two cases of oral pulse granuloma (OPG) or vegetable granuloma (VG) are presented, one of which was concomitant with an odontogenic keratocyst (OKC), which is an unusual finding. OKC is characterized by the presence of hyaline rings which include vessels, giant cells, other inflammatory cells and collagen fibres. There are two hypotheses as to its histogenesis: firstly, as a reaction to vegetable matter, such as legumes (thus the nomenclature "pulse" or edible seed) and secondly as a degenerative change in the vessel walls as a result of localized vasculitis. Due to the deceptive appearance of OPG, diagnosis can be challenging.

Peripheral Giant Cell Granuloma Associated With Periodontal Intrabony Defect.

INTRODUCTION: The peripheral giant cell granuloma (PGCG) is associated with periodontal bony lesions in several situations and excision results in a soft papilla defect and an intrabony defect without soft tissue protection. CASE PRESENTATION: A PGCG associated with loss of periodontal support is described. Following the lesion excision, a specific flap design outlining a surgical papilla in the adjacent area was proposed. The aim of this flap design was to obtain an optimal condition for periodontal regeneration, to treat the lesion excision associated with soft tissue defect, and to avoid a second surgical area. Complete periodontal defect resolution without soft tissue contraction or lesion recurrence was obtained at 2-year follow-up. CONCLUSION: Early diagnosis and treatment are essential to prevent greater loss of periodontal attachment.

Maxillary Giant Cell Granuloma: A Long-Term Follow-Up.

This is a case of a 32-year-old female with a known diagnosis of Turner syndrome who presented with complaints of chronic progressive right-sided facial pain and sinus pressure, and who was afebrile. On physical examination, there was eye proptosis on the right and significant increased fullness in the right infraorbital and maxillary regions. Computed tomography and magnetic resonance imaging demonstrated a large expansile space-occupying lesion in the right maxillary area, that histologically turned out to be a giant cell reparative granuloma. The lesion was completely removed and of interest, the patient was followed up both clinically and on imaging for 10 years with no signs of recurrence. A discussion on this entity, as well the clinical and imaging differential diagnoses, is carried out.

Giant cell lesion of the jaw as a presenting feature of Noonan syndrome.

This is a case of a 20-year-old woman who presented with a left jaw mass which was resected and found to be a giant cell granuloma of the mandible. Her history and physical examination were suggestive for Noonan syndrome which was confirmed with genetic testing and the finding of a PTPN11 gene mutation which has rarely been associated with giant cell lesions of the jaw. Given her particular genetic mutation and the presence of a giant cell lesion, we present a case of Noonan-like/multiple giant cell lesion syndrome.

Central Giant Cell Granuloma with Aneurysmal Bone Cyst in a 28-Year Male Patient: A Rare Concurrence.

Central Giant Cell Granuloma (CGCG) is a benign tumor of jaw. As compared to males, females are more commonly affected. Mandible is the common site of occurrence. Aneurysmal bone cysts (ABCs) are cystic lesions which are expansile osteolytic blood-filled lesions. These are commonly seen in the mandible, and they also show gender-predilection towards females. Concurrence of both the lesions is rarely reported. The treatment of both CGCG and ABC depends upon the extent of the lesions as well as on their nature. Due to aggressive nature of the lesions, these usually cause disfigurement of the patients' face. Wide excision is the treatment modality for aggressive lesions to avoid recurrence. We present a case of 28-year male who showed CGCG along with ABC in the same lesion, a rare concurrence.

Fibroma de Células Gigantes en Lactante Mayor / Giant Cell Fibroma in Older Infant. A Case Report

RESUMEN: El fibroma de células gigantes es considerado un tumor benigno no neoplásico de la mucosa oral. Este aparece en las primeras tres décadas de la vida, siendo relativamente raro en pacientes pediátricos. Puede encontrarse principalmente en la encía mandibular, mostrando predilección por el sexo femenino. Clínicamente se presenta como un crecimiento indoloro, de base sésil o pediculado, que generalmente se confunde con otras lesiones de tipo fibrosas como los fibromas de irritación. Histológicamente, se distingue por presentar fibroblastos estrellados con la presencia de células gigantes multinucleadas cerca de la lámina del epitelio. Presentamos el caso de una paciente femenino de un año de edad la cual presenta crecimiento nodular indoloro en relación con una superficie del paladar de 51 y 61. Teniendo en cuenta el tamaño y la ubicación de la lesión, se realizó escisión, biopsia y se envió para análisis histopatológico que confirmó la lesión como fibroma de células gigantes.

ABSTRACT: The giant cell fibroma is a benign nonneoplastic fibrous tumor of the oral mucosa. It occurs in the first three decades of life and is relatively rare in pediatric patients. It can be found predominantly in the mandibular gingiva, showing predilection for females. Clinically it presents as a painless, sessile, or pedunculated growth which is usually mistaken for other fibrous lesions like irritation fibroids. Histologically it is distinguished by the presence of stellated fibroblasts along with multinucleated giant cells near the epithelial sheet. We present a case where a one-year-old female patient presented with a painless nodular growth in relation to a palatesurface of 51 and 61. Considering the size and location of the lesion, excision and biopsy were performed and sent for histopathological analysis which confirmed the lesion as giant cell fibroma.

Central giant cell lesion of the jaws: An updated analysis of 2270 cases reported in the literature.

PURPOSE: To review all available data published on central giant cell lesion (CGCL) of the jaws into a comprehensive analysis of its clinical/radiological features, with emphasis on the predictive factors associated with its recurrence. METHODS: An electronic search was undertaken in 5 databases (February/2018), looking for reporting cases of CGCLs. RESULTS: A total of 365 publications were included, comprising 2270 lesions. CGCLs were more prevalent in women and the mandible. Cortical bone perforation occurred in 50% of the cases. Marginal/segmental resection was more often performed in larger lesions, and drug therapy was more frequent in small lesions. Recurrence was reported in 232 of 1316 cases (17.6%). The recurrence rate of the aggressive lesions (22.8%) after surgical treatment was higher than non-aggressive lesions (7.8%). Four of 5 CGCLs showed partial/total regression with pharmacological treatment. Aggressive lesions showed a worse response to corticosteroids than non-aggressive lesions. For the lesions submitted to surgery as the first treatment, curettage, enucleation, or marginal resection in relation to segmental resection, aggressive lesions, cortical bone perforation, and tooth root resorption were associated with increased recurrence rate. Recurrence related to a combination of surgical/pharmacological treatment could not be evaluated due to the variety of protocols. CONCLUSIONS: Aggressive CGCLs recur more often than the non-aggressive ones. Despite sometimes showing poor response to corticosteroid injection or surgical curettage, a combination of both treatment strategies should be considered in aggressive cases to reduce morbidities associated with radical surgery. The best protocol to manage aggressive and non-aggressive lesions remains to be determined.

Hybrid Central Odontogenic Fibroma with Giant Cell Granuloma like Lesion: A Report of Three Additional Cases and Review of the Literature.

Central odontogenic fibroma (COF) is an uncommon intraosseous neoplasm of the gnathic bones which is composed of fibrous connective tissue, with or without calcifications, and variable amounts of inactive odontogenic epithelium. It makes up less than 5% of odontogenic tumors and is more commonly seen in females. Central giant cell granuloma (CGCG) is a locally destructive but benign lesion of the jaws containing osteoclast-like multinucleated giant cells in a fibrovascular stroma. CGCG makes up approximately 10% of all benign jaw tumors and typically occurs in females younger than 30 years of age. A hybrid lesion with histologic features of both COF and CGCG is very rare and was first described in 1992. To date, fewer than 50 cases of this lesion have been reported. In this study, we present three additional cases of COF developing in conjunction with giant cell granuloma-like lesion, as well as provide a comprehensive literature review. Two of the lesions presented in our study were located in the posterior mandible and one occurred in the anterior mandible. Buccal and/or lingual expansion was noted in two patients and no recurrence was reported. Histologically, all three lesions demonstrated a blend of odontogenic epithelial islands with numerous multinucleated giant cells in a highly cellular connective tissue stroma. Immunohistochemical staining with CK19 and CD68 highlighted the odontogenic epithelium and multinucleated giant cells respectively. The precise nature of these hybrid lesions remains obscure and additional molecular studies may be of help in understanding their pathogenesis.

Florid cemento-osseous dysplasia and peripheral giant cell granuloma in a patient with neurofibromatosis 1.

We report a 35-year-old mulatto female patient with neurofibromatosis Type 1 who presented with facial asymmetry. The patient had two lesions: florid cemento-osseous dysplasia associated with peripheral giant cell granuloma. She was referred for surgical treatment of the peripheral giant cell granuloma and the florid cemento-osseous dysplasia was treated conservatively by a multidisciplinary team. So far, no changes have been observed in the patient's clinical status. We observed no recurrence of peripheral giant cell granuloma. To the best of our knowledge, the present case is the first report of a patient with neurofibromatosis Type 1 associated with a giant cell lesion and florid cemento-osseous dysplasia.

Florid cemento-osseous dysplasia and peripheral giant cell granuloma in a patient with neurofibromatosis 1

Abstract: We report a 35-year-old mulatto female patient with neurofibromatosis Type 1 who presented with facial asymmetry. The patient had two lesions: florid cemento-osseous dysplasia associated with peripheral giant cell granuloma. She was referred for surgical treatment of the peripheral giant cell granuloma and the florid cemento-osseous dysplasia was treated conservatively by a multidisciplinary team. So far, no changes have been observed in the patient's clinical status. We observed no recurrence of peripheral giant cell granuloma. To the best of our knowledge, the present case is the first report of a patient with neurofibromatosis Type 1 associated with a giant cell lesion and florid cemento-osseous dysplasia.

Reduction surgery using a combination of a stereolithographic model and navigation system for ossifying fibroma with secondary central giant cell granuloma.

Both central giant cell granuloma (CGCG) and ossifying fibroma (OF) are relatively common diseases. The synchronous presentation of CGCG and OF is, however, an extremely rare occurrence. We present an unusual case with the synchronous presentation of these two diseases in the maxilla and introduce a surgical strategy based on a combination of the stereolithographic model and navigation system for the treatment of gigantic OF with secondary CGCG.

Repigmentation of gray hair in lesions of annular elastolytic giant cell granuloma.

Hair pigmentation is a complex phenomenon that involves many hormones, neurotransmitters, cytokines, growth factors, eicosanoids, cyclic nucleotides, nutrients, and a physicochemical milieu. We report a case of repigmentation of gray hairs in lesions of annular elastolytic giant cell granuloma (AEGCG) on the scalp of a 67-year-old man.

Zoledronic Acid for the Treatment of Children With Refractory Central Giant Cell Granuloma.

There are no approved medical therapies for the treatment of pediatric central giant cell granuloma (CGCG), a benign but potentially aggressive tumor of the jaw. Zoledronic acid (ZA), a third-generation bisphosphonate, has been used in CGCG occurring in adults. We describe 4 patients with CGCG treated with ZA, 3 of whom achieved resolution of disease up to 4 years of follow-up. Our experience suggests that ZA may be considered as treatment for pediatric CGCG.

An usual elastophagocytic granuloma with involvement of the back: a possible variant of an annular elastolytic giant cell granuloma.

Annular elastolytic giant cell granuloma (AEGCG) is a very infrequent granulomatous dermatitis characterized by elastolysis and elastophagocytosis. It usually appears in middle-aged Caucasian women and is normally located in sun-exposed areas.We present a case of a 73-year-old woman with hypertension and type II diabetes, who was admitted to the hospital for an ischemic cerebrovascular accident. She presented with annular and serpiginous skin lesions on her back and arms that had appeared seven months earlier;  a clinical and histological diagnosis of elastophagocytic granuloma was made. Our patient exhibited a florid presentation and a self-limiting course.

Tumor-induced rickets in a child with a central giant cell granuloma: a case report.

Tumor-induced osteomalacia/rickets is a rare paraneoplastic disorder associated with a tumor-producing fibroblast growth factor 23 (FGF23). We present a child with symptoms of rickets as the first clinical sign of a central giant cell granuloma (CGCG) with high serum levels of FGF23, a hormone associated with decreased phosphate resorption. A 3-year-old boy presented with a limp and 6 months later with painless growth of the jaw. On examination gingival hypertrophy and genu varum were observed. Investigations revealed hypophosphatemia, normal 1,25 and 25 (OH) vitamin D, and high alkaline phosphatase. An MRI showed an osteolytic lesion of the maxilla. Radiographs revealed typical rachitic findings. Incisional biopsy of the tumor revealed a CGCG with mesenchymal matrix. The CGCG was initially treated with calcitonin, but the lesions continued to grow, making it necessary to perform tracheostomy and gastrostomy. One year after onset the hyperphosphaturia worsened, necessitating increasing oral phosphate supplements up to 100 mg/kg per day of elemental phosphorus. FGF23 levels were extremely high. Total removal of the tumor was impossible, and partial reduction was achieved after percutaneous computed tomography-guided radiofrequency, local instillation of triamcinolone, and oral propranolol. Compassionate use of cinacalcet was unsuccessful in preventing phosphaturia. The tumor slowly regressed after the third year of disease; phosphaturia improved, allowing the tapering of phosphate supplements, and FGF23 levels normalized. Tumor-induced osteomalacia/rickets is uncommon in children and is challenging for physicians to diagnose. It should be suspected in patients with intractable osteomalacia or rickets. A tumor should be ruled out if FGF23 levels are high.