Gnathic Bones Research Articles

Desmoplastic Fibroma of the Gnathic Bones-A Systematic Review.

To summarize published information about the desmoplastic fibroma of the gnathic bones into a descriptive analysis of the main features of this condition. A systematic review according to the PRISMA guidelines was conducted. Electronic search was performed in four databases and in the gray literature. Case reports and case series were included. Frequencies were obtained for descriptive analysis. We identified 66 articles, for a total of 96 cases. Female patients (55.8%) in the first decade of life (40.6%) with a mean age of 18.2 years were more affected. The mandible was the most affected bone with 81.2% of the cases. The main clinical feature was painless swelling (54.2%). Most of the imaging examinations (radiological, computed tomography, and magnetic resonance) showed well-defined radiolucencies (65.4%) lesions. The treatment was surgical removal in all cases. The recurrence rate was 10.8% and all in the posterior mandible. Spindle cell fibroblasts in a collagenized stroma were often described in the histopathological features. Vimentin, smooth muscle actin, and β-catenin were common immunohistochemical markers. Desmoplastic fibroma is a locally aggressive lesion that commonly affects the jaws in children. Histopathology is essential for diagnosis, and the pathogenesis of this tumor should be further investigated.

Deep learning in the diagnosis for cystic lesions of the jaws: a review of recent progress.

Cystic lesions of the gnathic bones present challenges in differential diagnosis. In recent years, artificial intelligence (AI) represented by deep learning (DL) has rapidly developed and emerged in the field of dental and maxillofacial radiology (DMFR) Dental radiography provides a rich resource for the study of diagnostic analysis methods for cystic lesions of the jaws and has attracted many researchers. The aim of the current study was to investigate the diagnostic performance of DL for cystic lesions of the jaws. Online searches on Google Scholar, PubMed, and IEEE Xplore databases, up to September 2023, with subsequent manual screening for confirmation. The initial search yielded 1862 titles, and 44 studies were ultimately included. All studies used DL methods or tools for the identification of a variable number of maxillofacial cysts. The performance of algorithms with different models varies. Although most of the reviewed stu dies demonstrated that DL methods have better discriminative performance than clinicians, further development is still needed before routine clinical implementation due to several challenges and limitations such as lack of model interpretability, multicenter data validation, etc Considering the current limitations and challenges, future studies for the differential diagnosis of cystic lesions of the jaws should follow actual clinical diagnostic scenarios to coordinate study design and enhance the impact of artificial intelligence in the diagnosis of oral and maxillofacial diseases.

Challenges in the Surgical Management of Large Osteoma in the Maxillary Sinus: Case Report

Osteoma is a benign neoplasm characterized by slow growth and can be composed of spongy or compact bone. Its symptomatology is directly related to the location, size, and proximity to adjacent structures. Histologically, it is classified as compact, cancellous, or mixed. Although rarely found in the gnathic bones, they are more frequently observed in the paranasal sinuses. This study aims to report a case of osteoma located in the maxillary sinus of a male patient, describing the surgical management performed in a hospital setting. The patient, a 49-year-old male with no systemic diseases, underwent surgical procedure under general anesthesia, including intraoral access, excision of the lesion, and subsequent fixation of the bone fragment. After six months of postoperative follow-up, no recurrences were observed. It is concluded that the definitive treatment of osteoma requires adequate correlation of the clinical picture, selection of the best type of surgical access, consideration of the location, and choice of the most appropriate synthesis method to promote a good prognosis.

Manifestações clínicas e imaginológicas do querubismo: Um relato de caso

Introduction: Kerubism is a rare hereditary disorder characterized by bilateral enlargement of the gnathic bones in children, with a tendency to spontaneous remission at puberty. In some cases, it can develop without a family history. Aim: This report presents a case of non-hereditary cherubism affecting the dentition in a 7-year-old patient. Case report: A 7-year-old male patient complained of a bilateral increase in volume on the face in the mandibular body and ramus, which had been developing for 3 years, asymptomatically and with slow growth, with no history of similar cases in the family. Intraorally, the increase in volume extended from the canine to the molar, bilaterally, with anomalies in the teeth involved. Hematological tests were within limits. Panoramic radiography and facial CT revealed multiple multiloculated lesions in the mandibular body and ramus bilaterally. An incisional biopsy was performed under local anesthesia, which revealed the presence of multinucleated giant cells. Based on the clinical, imaging and histopathological findings, the final diagnosis was kerubism. Due to the self-limiting behavior of the pathology, surgical interventions were not necessary and the case will be followed up. Discussion: Although the lesions tend to be symmetrical, here they were more prominent on the right side. The radiographic features were typical of the disease. The lesions can cause malocclusions due to ectopic eruptions, as seen in this case. Despite the hereditary characteristic, there was no family history of the disease. Histological and laboratory analyses are important to rule out other fibro-osseous lesions, but clinical and radiographic aspects are paramount in the diagnosis. Once the bone matures, the lesion tends to regress. However, cosmetic surgery may still be necessary afterwards. Conclusion: The diagnosis of the disease should be based mainly on clinical and imaging findings, since histological analysis is of limited value. Once confirmed, the case should be followed up and surgical interventions postponed if necessary at a later date.

Lesion Expansion in Gnathic Fibrous Dysplasia: Natural History, Indicators of Progression, and Response to Bisphosphonates.

Fibrous dysplasia (FD) is characterized by expansile fibro-osseous lesions that may occur in association with endocrinopathies as part of McCune-Albright syndrome (MAS). Craniofacial FD is a significant source of morbidity and most commonly involves the gnathic bones. There is a critical need to understand the natural history and risk factors for gnathic FD progression to develop preventative trials and identify candidates for intervention. The purpose of this study was to characterize gnathic FD lesion expansion and to identify risk factors associated with lesion growth. Patients with gnathic FD and serial CT imaging were evaluated. Volumetric analyses of CT scans were performed using MIM Encore software. Generalized mixed model analysis was used to account for intra-subject correlation, with FD lesion volume as the dependent variable. In addition to age, effects of MAS-associated endocrinopathies, sex, disease severity, and bisphosphonate treatment were evaluated. A total of 104 total lesions in 52 patients were characterized longitudinally. Median age at initial scan was 8.8 years (range 3.4-18.8), and median age at final scan was 16.8 years (range 6.9-33.4 years). The median number of scans per subject was 4 (range 2-14). FD lesion volume increased with age (2.50 cm3 /yr, 95% confidence interval [CI] 1.95-3.04, p < 0.001). However, lesion expansion rate decreased over time (-0.05 cm3 /yr, 95% CI -0.07 to 0.04, p < 0.001). Mandibular lesions tended to expand at a greater rate than maxillary lesions (p < 0.001). Growth hormone excess was associated with accelerated expansion rate (p = 0.002). Other MAS-associated endocrinopathies, pubertal status, sex, weight, lesion density, disease severity, and bisphosphonate treatment were not associated with lesion volume or expansion. Gnathic FD lesion expansion is most rapid in younger children and declines as patients approach adulthood. The availability of quantitative natural history data will guide clinicians in identifying patients who are candidates for medical and surgical interventions and clinical trials for preventative therapies. Published 2023. This article is a U.S. Government work and is in the public domain in the USA.

Non‐ossifying fibroma of the mandible: A case report and review of the literature

AbstractThe case reported here is a large non‐ossifying fibroma (NOF) of the mandible presenting in a 24‐year‐old male patient. The lesion was diagnosed through radiological and histopathological assessment. It remains under observation without surgical intervention, despite its large size, as the extensive surgery to remove it is not currently justifiable. Whilst common at the metaphysis of the long bones in paediatric patients, NOFs are rarely observed in the jaw bones. These benign fibrous lesions are often asymptomatic and may be an incidental radiological finding. Most NOFs of the limbs presenting in children will not require any treatment, and spontaneously resolve when growth ceases. However, regarding mandibular lesions, the majority of historical cases have been successfully treated with curettage and do not recur. Due to their scarcity, the outcomes for untreated NOFs of the gnathic bones are not reliably known. We discuss the clinical, radiological and histopathological findings for this case. A thorough literature review of previous reports of this rare entity reveals the typical characteristics and behaviour of this lesion.

ANALYSIS OF THE RADIOGRAPHIC ASPECTS OF LYMPHOMAS AND LEUKEMIAS AFFECTING GNATHIC BONES

Lymphomas and leukemias are malignant neoplasms that uncommonly affect the jaws and their radiographic manifestations may contribute to the diagnosis. Objective: To investigate the radiographic features of lymphomas and leukemias in the jaw bones. Study Design: systematic review in the electronic databases Pubmed, Web of Science, and Scopus using the key words "lymphoma, leukemia, mandible, maxilla, jaw, gnathic bones", between 2008 and 202. Inclusion criteria: Periapical, panoramic, or computed tomography (CT) images for individual cases and diagnosis of lymphoma/leukemia confirmed by immunohistochemistry. Three authors described the images and evaluated the quality of the radiographic exams. Results: From 1079 reports screened, 130 cases were selected containing 88 tomographic, 78 panoramic, and 26 periapical exams, most of them classified as excellent (54%). Diffuse large B-cell lymphomas NOS (DLBCL), Burkitt lymphoma, leukemic infiltration, and plasmablastic lymphoma were the more common subtypes. Involved teeth presented mobility in 36% and a provisional diagnosis of inflammatory/infectious tooth disease was considered 48% of the cases. Most cases were radiolucent and conventional CT exams were available for 76% of the cases, with hypodense aspect, ill-defined borders, and periosteal reactions were uncommon. Conclusion: Lymphomas with gnathic bone destruction are usually of high-grade subtypes and rarely present periosteal reaction. Lymphomas and leukemias are malignant neoplasms that uncommonly affect the jaws and their radiographic manifestations may contribute to the diagnosis. Objective: To investigate the radiographic features of lymphomas and leukemias in the jaw bones. Study Design: systematic review in the electronic databases Pubmed, Web of Science, and Scopus using the key words "lymphoma, leukemia, mandible, maxilla, jaw, gnathic bones", between 2008 and 202. Inclusion criteria: Periapical, panoramic, or computed tomography (CT) images for individual cases and diagnosis of lymphoma/leukemia confirmed by immunohistochemistry. Three authors described the images and evaluated the quality of the radiographic exams. Results: From 1079 reports screened, 130 cases were selected containing 88 tomographic, 78 panoramic, and 26 periapical exams, most of them classified as excellent (54%). Diffuse large B-cell lymphomas NOS (DLBCL), Burkitt lymphoma, leukemic infiltration, and plasmablastic lymphoma were the more common subtypes. Involved teeth presented mobility in 36% and a provisional diagnosis of inflammatory/infectious tooth disease was considered 48% of the cases. Most cases were radiolucent and conventional CT exams were available for 76% of the cases, with hypodense aspect, ill-defined borders, and periosteal reactions were uncommon. Conclusion: Lymphomas with gnathic bone destruction are usually of high-grade subtypes and rarely present periosteal reaction.

CHALLENGES OF MANDIBLE CENTRAL GIANT CELL GRANULOMA TREATMENT: A CASE REPORT

Central giant cell granuloma (CGCG) is considered to be a rare benign intraosseous lesion of unknown etiology, that is non-neoplastic but locally aggressive, and is present in gnathic bones. This study aimed to present the challenges of a CGCG treatment. A 27-year-old woman was referred for treatment of CGCG in the mandible. Clinical examination, panoramic X-ray, and cone-beam tomography revealed a large lesion involving the right posterior region of the mandible, causing facial asymmetry. The lesion was initial treated with 18 sessions of intralesional triamcinolone acetonide infiltrations. There was a partial response in forming the basal cortical bone. After, a surgical procedure fully enucleated the lesion. After 6 years, radiographic exams showed adequate bone formation, and a third approach was performed for rehabilitation with implants and prothesis. After 9 years of follow-up, the patient is asymptomatic, showing satisfactory functional and esthetic conditions. Central giant cell granuloma (CGCG) is considered to be a rare benign intraosseous lesion of unknown etiology, that is non-neoplastic but locally aggressive, and is present in gnathic bones. This study aimed to present the challenges of a CGCG treatment. A 27-year-old woman was referred for treatment of CGCG in the mandible. Clinical examination, panoramic X-ray, and cone-beam tomography revealed a large lesion involving the right posterior region of the mandible, causing facial asymmetry. The lesion was initial treated with 18 sessions of intralesional triamcinolone acetonide infiltrations. There was a partial response in forming the basal cortical bone. After, a surgical procedure fully enucleated the lesion. After 6 years, radiographic exams showed adequate bone formation, and a third approach was performed for rehabilitation with implants and prothesis. After 9 years of follow-up, the patient is asymptomatic, showing satisfactory functional and esthetic conditions.

GLANDULAR ODONTOGENIC CYST MIMICKING RADICULAR CYST IN POSTERIOR MAXILLA: CASE REPORT

Glandular odontogenic cyst is a rare developmental odontogenic cyst that may exhibit aggressive behavior and may be prone to recurrence. It is frequently found in middle-aged adults, preferentially affecting the anterior region of the gnathic bones, especially the mandible. It presents nonspecific clinical and radiographic findings that may resemble other lesions, but has specific histopathological characteristics that aid in the diagnosis. This study aimed to report an unusual presentation of a glandular odontogenic cyst diagnosed in the posterior maxillary region of a 47-year-old female patient, in which the first diagnostic hypothesis was of a radicular cyst. Glandular odontogenic cyst is a rare developmental odontogenic cyst that may exhibit aggressive behavior and may be prone to recurrence. It is frequently found in middle-aged adults, preferentially affecting the anterior region of the gnathic bones, especially the mandible. It presents nonspecific clinical and radiographic findings that may resemble other lesions, but has specific histopathological characteristics that aid in the diagnosis. This study aimed to report an unusual presentation of a glandular odontogenic cyst diagnosed in the posterior maxillary region of a 47-year-old female patient, in which the first diagnostic hypothesis was of a radicular cyst.

ANAPLASTIC PLASMACYTOMA OF THE MANDIBLE: A CASE REPORT

Anaplastic plasmacytomas (AP) of the gnathic bones are uncommon tumors. An otherwise healthy 67-year-old male patient presented with a 2-year history of an asymptomatic swelling affecting the anterior mandible causing tooth mobility. Imaging exams revealed a destructive well-defined multilocular radiolucency causing cortical expansion and disruption. An incisional biopsy was performed and microscopic examination revealed sheets of medium to large-sized anaplastic cells with abundant cytoplasm, eccentric nuclei, and marked pleomorphism. Immunohistochemical markers showed positivity for CD138 and kappa, and negativity for LCA, AE1AE3, desmin, and lambda. The Ki-67 labeling index was 90%. The final diagnosis was AP of the mandible. The patient's clinical checkup ruled out other bone lesions, confirming the diagnosis of a solitary plasmacytoma. The patient is currently undergoing radiotherapy. Oral pathologists should be aware of AP to avoid erroneous diagnoses when evaluating anaplastic cell proliferations of the gnathic bones in elderly patients. Anaplastic plasmacytomas (AP) of the gnathic bones are uncommon tumors. An otherwise healthy 67-year-old male patient presented with a 2-year history of an asymptomatic swelling affecting the anterior mandible causing tooth mobility. Imaging exams revealed a destructive well-defined multilocular radiolucency causing cortical expansion and disruption. An incisional biopsy was performed and microscopic examination revealed sheets of medium to large-sized anaplastic cells with abundant cytoplasm, eccentric nuclei, and marked pleomorphism. Immunohistochemical markers showed positivity for CD138 and kappa, and negativity for LCA, AE1AE3, desmin, and lambda. The Ki-67 labeling index was 90%. The final diagnosis was AP of the mandible. The patient's clinical checkup ruled out other bone lesions, confirming the diagnosis of a solitary plasmacytoma. The patient is currently undergoing radiotherapy. Oral pathologists should be aware of AP to avoid erroneous diagnoses when evaluating anaplastic cell proliferations of the gnathic bones in elderly patients.

Clinical Features and Radiographic Aspects of Squamous Cell Carcinoma in the Gnathic Bones

This integrative review aimed to discuss the clinical features and imaging aspects of squamous cell carcinoma in the gnathic bones on panoramic radiographs and cone-beam computed tomography. The electronic search was conducted in PubMed, Embase, and Scopus using the keywords cone-beam computed tomography, panoramic radiography, dentomaxillofacial complex, and oral squamous cell carcinoma. Studies between 2012 and 2022, report imaging aspects of the oral squamous cell carcinoma in panoramic radiography and cone-beam computed tomography were selected. The initial search found 375 articles, leaving 171 after excluding duplicates. Eighteen studies met the inclusion criteria, bringing together a total of twenty cases. Swelling and pain are common clinical features. In most cases, the squamous cell carcinoma was in the mandible; the borders were poorly defined with invasive aspects; the internal structure was radiolucent/hypodense and some cases, had radiopaque flecks. The lesion causes structures destroyed like the adjacent bone, the alveolar process, border of the mandibular canal, ramus of the mandible. The image aspects raised in this review: as large areas of osteolysis interspersed with an irregular pattern of radiopaque/hyperdense flakes, with imprecise limits and invasive borders, causing significant destruction of adjacent structures, squamous cell carcinoma can be a diagnostic hypothesis. In these cases, we recommend urgency in completing the diagnosis. The panoramic radiography can provide information that leads to the suspicion of a malignant lesion, but cone-beam computed tomography provides the real dimension and repercussion of the lesion.

Genomic Profiling of the Craniofacial Ossifying Fibroma by Next-Generation Sequencing.

Ossifying fibroma (OF) of the craniofacial skeleton is a fibro-osseous lesion characterized by various patterns of bone formation in a cellular fibroblastic stroma. The molecular landscape of OF remains mostly unknown. There are a few known pathogenic abnormalities in OF, including HRPT2 mutations in conventional OF and SATB2 translocations in juvenile psammomatoid OF. On the other hand, conflicting reports exist regarding MDM2 gene amplification and chromosomal copy number alterations (CNA) in OF. Surgically removed biopsies and curettage specimens from OF patients were obtained. Clinical, radiographic, and pathologic features of tumors were reviewed. Genomic DNA was extracted from formalin-fixed, paraffin-embedded blocks of tumor tissue. Capture-based DNA next-generation sequencing targeting the coding regions 529 cancer genes and select introns was performed. We identified 17 OF cases from 8 male and 8 female patients with mean age of 22years (range 1-58years). Nine case occurred in the gnathic bones and 8 in the extragnathic craniofacial bones. These cases included 3 juvenile psammomatoid OF, 6 conventional OF and 8 juvenile trabecular OF. Large-scale CNAs were present in 6 of 17 cases. Seven cases (41%) had focal amplifications including FOSB (n = 2, 11%), FOS (n = 4, 23%), COL1A1 (n = 4, 23%) and TBX3 (n = 5, 29%). Three cases (17%) had pathogenic CDC73 mutations. No cases showed focal MDM2 amplification. Here, we provided a comprehensive molecular characterization of OF that reveals a heterogeneous genetic profile with occasional large-scale CNAs (n = 6, 35%). FOS, FOSB, and TBX3 genes that regulate AP-1 transcriptional complex are frequently altered in OF (n = 7, 41%), chiefly in juvenile trabecular OF. These genes encode transcription factors that act as downstream effectors of the MAP kinase signaling pathway. MDM2 amplification is an exceedingly rare event in OF, if present at all, so identification of this event should continue to raise concern for low-grade gnathic osteosarcoma. In summary, our findings suggest that OF represents a heterogeneous group of tumors at the genetic level but dysregulation of the AP-1 pathway may play a role in pathogenesis of juvenile trabecular OF.

Cystic Lesions of the Jaws: The Top 10 Differential Diagnoses to Ponder.

Cystic lesions of the gnathic bones present challenges in differential diagnosis. This category includes a smorgasbord of odontogenic and non-odontogenic entities that may be reactive or neoplastic in nature. While most cystic jaw lesions are benign, variability in biologic behavior makes distinction between these entities absolutely crucial. Review. Two clinical cases are presented in parallel and are followed by an illustrated discussion of the ten most likely differential diagnoses that should be considered when confronted with a cystic jaw lesion. Strong emphasis is placed on the histologic differences between these entities, empowering readers to diagnose them with confidence. Perhaps even more importantly, the more common diagnostic pitfalls in gnathic pathology are discussed, recognizing that a definitive diagnosis cannot be rendered in every situation. The histologic diagnoses for the two clinical cases are finally revealed. Cystic lesions of the maxilla and mandible may be odontogenic or non-odontogenic. The most common cystic lesions are the reactive periapical cyst, and the dentigerous cyst (which is developmental in nature). It is important to note that cystic neoplasms also occur in the jaws, and that the presence of inflammation may obscure the diagnostic histologic features of lesions like odontogenic keratocyst and unicystic ameloblastoma. Ancillary testing is of limited diagnostic value in most scenarios. However, both clinical and radiographic information (such as the location, size, duration, associated symptoms, and morphology of the lesion in its natural habitat) are significantly useful.

Isolated Intra-Oral Relapsed B-Acute Lymphoblastic Leukemia (B-ALL): A Case Report

B-Lymphoblastic Leukemia (B-ALL) is the second most common acute leukemia in adults. Its clinical manifestations include leukocytosis or leukopenia, anemia, and thrombocytopenia with an overall poor prognosis in elderly patients. If relapse occurs, it commonly involves the bone marrow but can also involve Extramedullary (EM) sites such as the brain, testis, liver, spleen and lymph nodes. Isolated extramedullary involvement of the oral cavity in relapse B-ALL is extremely rare with only a few reposted cases in the literature in pediatrics and Adolescent-and-Young-Adult (AYA) population. Here we describe the case of an elderly gentleman who presented with isolated EM B-ALL relapse involving the oral cavity and gnathic bones which was initially misdiagnosed as an odontogenic infection.

Keratocystic Odontogenic Tumor- A Rare Pediatric Case Report with an Impacted Premolar

Keratocystic Odontogenic Tumor which presently is also termed as Odontogenic keratocyst (OKC) this new title was given by WHO in 2005. It is a benign intraosseous cystic lesion of the gnathic bones which probably originates from the remnants of the dental lamina. We Present the case of a female child aged 12 years with aggressive KCOT and impacted maxillary second premolar.

Multiple complex odontomas of the mandible: a rare case report and literature review

Odontomas are the most common tumor-like (hamartoma) odontogenic lesions in the gnathic bones. There are two main types: complex odontoma which is composed mainly of amorphous calcification, and compound odontoma which is characterized by irregular tooth-like structures. Odontomas are generally asymptomatic and in most cases involve only one quadrant of the maxilla or mandible. The treatment of choice is surgical removal. The aim of the present study is to report a rare case of multiple complex odontomas affecting the mandible and to review the literature on the clinical and pathological features of these tumors.

MULTIPLE COMPLEX ODONTOMAS: A RARE CASE REPORT

Odontomas are the most common tumor-like (hamartoma) odontogenic lesions in the gnathic bones. Multiple odontomas are rare and characterized by numerous odontomas involving 1 to 4 quadrants of the gnathic bones. We report the case of a 16-year-old female patient without noteworthy extra- and intraoral physical examination alterations. Panoramic radiography revealed a well-delimited, unilocular, bilateral radiolucent lesion in the ascending ramus of the mandible. An incisional biopsy was performed, which revealed a developing complex odontoma at both sites. The treatment of choice was enucleation, followed by curettage of the bone defect. The removed material was sent for anatomopathologic analysis, which confirmed the diagnosis of complex odontoma. The patient has been in follow-up for 10 months and shows no signs or symptoms of recurrence.

CHALLENGES IN GARDNER'S SYNDROME: REPORT OF TWO CASES IN THE SAME FAMILY

A 38-year-old woman attended the dental clinic reporting painless facial asymmetry of unknown evolution. Extraoral exam revealed slight bilateral alteration in the mandibular contour. The patient had previously been diagnosed with Gardner's syndrome, and intestinal polyps and desmoid tumor were detected. The patient reported that this syndrome also affected some relatives. Imaging exams showed multiple radiopaque lesions compatible with osteomas, as well as impacted and supernumerary teeth. The patient's daughter (13 years old) presented a slight painless swelling bilaterally in the mandibular angle on clinical examination. Imaging exams revealed impacted and supernumerary teeth and multiple sclerotic masses, 2 of them in the mandibular angles on the surface of the cortical bone. A therapeutic approach to improve aesthetics has not been performed yet because of the pandemic. The clinical cases reinforce the need for proper anamnesis and genetic counseling of patients. Additionally, changes in the gnathic bones may favor the early diagnosis of intestinal polyps.

GORLIN-GOLTZ SYNDROME: A REPORT OF TWO CLINICAL CASES WITH DIAGNOSIS AND TREATMENT AT HOSPITAL LEVEL

Gorlin-Goltz syndrome, also known as nevoid basal cell carcinoma syndrome, is a rare hereditary autosomal dominant condition, with high penetrance and variable expressivity. The aim of this study is to report 2 cases diagnosed at emergency hospital units with the specialty of maxillofacial surgery and traumatology. The first refers to a 10-year-old patient who sought consultation due to an expressive increase in the maxilla volume with panoramic radiography evidencing multiple unilocular radiolucent images in the gnathic bones. The second is a 20-year-old woman presenting extraoral increased volume in the mandibular region with signs of infection; imaging exams showed 2 unilocular radiolucent images in the mandible. After clinical, radiological, and histopathologic evaluation, multiple keratocysts, skeletal changes, and other criteria that characterize the syndrome were identified. In both cases, surgical treatment of the odontogenic cysts was performed, and the patients remain in multiprofessional follow-up.

CENTRAL GIANT CELL LESION IN A PATIENT WITH HYPOPPHOSPHATEMIC OSTEOMALACIA ASSOCIATED WITH EPIDERMIC NEVUS SYNDROME

Epidermal nevus syndrome (ENS) is a rare congenital condition characterized by epidermal nevi associated with skeletal, neurological, cardiovascular, ocular, and genitourinary abnormalities. Epidermal nevi have been considered producers of phosphaturic factors capable of triggering hypophosphatemic osteomalacia. The association between the systemic factors of ENS and how they influence the behavior of central giant cell lesions (CGCL) in the gnathic bones is not yet established. A 19-year-old female patient with hypophosphatemic osteomalacia as a result of ENS (epidermal nevi in the hemiface, trunk, and right limbs) presented with facial asymmetry caused by increased volume due to aggressive and extensive mandibular CGCL. Due to the impossibility of mandibular reconstruction with a microsurgical flap and the patient's endocrine condition, which contraindicated drug treatment with glucocorticoids or calcitonin, conservative osteoplasty was chosen to avoid the sequelae of a hemimandibulectomy. At postoperative follow-up (less than 3 years), the lesion was stabilized and there was bone neoformation.