Search Results (79)

Background: Dental eruption pathways could be influenced by several factors, both general and local, with different prevalence and morbidity. This study aims to report our experience of pediatric impacted teeth due to local factors, with the exclusion of the third molars, illustrating their prevalence, diagnostic and therapeutic pathways, and treatment outcomes. Methods: The inclusion criteria were minor age (<18 years) and the presence of impacted teeth due to a local cause, excluding wisdom teeth. The complete diagnostic and therapeutic procedures and their outcomes were described. The relationship between the treatment and the outcomes was assessed with a chi-square test. Results: One hundred twelve patients with a single impaction were included in the study. The local causes of single impaction were: 63 odontogenic cysts (57%), 24 supernumerary teeth (21%), 17 odontogenic tumors (15%), and eight primary bone lesions (7%). During the follow-up period, 83 teeth erupted spontaneously 12–36 months following surgery (74%), 12 were extracted during surgery (11%), and 17 needed orthodontic traction to achieve their aesthetic and functional position (15%). The relationship between mini-invasive surgery and spontaneous eruption was significant (p < 0.00001). Conclusions: Within the limitations of this study, mini-invasive surgical treatment preceded by a correct diagnosis may lead to a spontaneous eruption of permanent teeth, avoiding further orthodontic intervention and premature loss of permanent teeth. Full article

Background: Ameloblastic carcinoma (AC) is a rare malignant odontogenic tumor with limited knowledge surrounding its pathogenesis, molecular pathways, clinical behavior, treatment, and prognosis. This 40-year literature scoping review aims to enhance the comprehension of this complex condition, looking closely at how AC works at molecular and pathophysiological levels and what causes it to develop. Methods: The PUBMED, Medline, Scopus, and Cochrane central databases were searched, including articles from 1984 to date. Articles reporting epidemiological, clinical, instrumental, and histopathological data were included. Results: Out of the 375 articles examined, 52 met the inclusion criteria, yielding a total of 80 cases of AC. All cases before 1984 were excluded from the analysis, as were all that did not provide information on patient survival. Several molecular mechanisms associated with its development and progression were identified; these help in early diagnosis. Moreover, AC can spread locally, making a radical surgical approach necessary. There is still no agreement on how to manage neck dissection. Surgical removal followed by monitoring is an important part of managing AC. Conclusions: Advancements in biological and molecular insights have the potential to facilitate earlier diagnosis and treatment. These could lead to improvements in patients’ quality of life and long-term survival. Full article

Odontogenic myxoma (OM) is a rare, benign intraosseous tumor that arises from the dental follicle, dental papilla, or periodontal ligament. It typically affects the mandible, maxilla, and sinuses, more frequently in women than men. The histopathology of OM is characterized by a myxoid stroma with varying degrees of collagenization and spindle cells. Treatment is surgical, but there are no standardized guidelines. Available options include enucleation and curettage, with conservative approaches offering better aesthetics and functionality but a higher recurrence risk. This case report aims to discuss a rare presentation of OM in the anterior part of the mandible of a 22-year-old female patient with Hashimoto’s thyroiditis and idiopathic thrombocytopenic purpura. The patient presented with painless swelling and tooth mobility in the symphysis region. Radiographic evaluation revealed a unilocular lesion extending from the left second incisor to the right first incisor, with no root resorption. Histopathological analysis confirmed the OM diagnosis. Due to the patient’s young age and the size of the lesion, a conservative treatment approach was chosen, with the potential for future implant-prosthodontics rehabilitation. The case underscores the importance of thorough clinical, radiological, and histopathological assessment to ensure accurate diagnosis and management of OM. Long-term follow-up is essential due to the increased risk of recurrence in younger patients. Full article

Gorlin–Goltz syndrome, also known as nevoid basal cell carcinoma syndrome (NBCCS), is a rare, inherited autosomal dominant disorder primarily caused by mutations in the PTCH1 gene, which regulates the Hedgehog signaling pathway. This genetic defect leads to the uncontrolled proliferation of basal cells, resulting in the formation of multiple basal cell carcinomas (BCCs) and odontogenic keratocysts (OKCs). This study aims to present a complex clinical case of a patient with Gorlin–Goltz syndrome who developed multiple recurrent metastatic basal cell carcinomas on the facial region, detailing the multidisciplinary treatment strategies employed and the challenges encountered during the management of the disease. The patient, diagnosed with a pathogenic PTCH1 gene mutation, underwent a series of treatment interventions over several years. These included multiple surgical excisions aimed at tumor removal, diverse radiotherapy approaches for residual or inoperable lesions, and systemic targeted therapy with Hedgehog pathway inhibitors to control tumor progression. The recurrent and aggressive nature of the basal cell carcinomas resulted in extensive facial tissue loss, posing significant challenges for radical tumor excision and subsequent reconstructive procedures. Multimodal therapeutic strategies, including Mohs micrographic surgery for precise tumor clearance and targeted systemic therapy with vismodegib, were implemented. However, the aggressive progression of lesions required ongoing surgical interventions, highlighting the limitations of current treatment modalities in achieving long-term disease control. This case underscores the critical need for a comprehensive, multidisciplinary approach to managing Gorlin–Goltz syndrome. Successful management requires close collaboration between dermatologists, oncologists, maxillofacial surgeons, and plastic surgeons to balance effective tumor control with optimal functional and aesthetic outcomes. The integration of advanced surgical techniques and targeted molecular therapies shows promise in improving patient outcomes. Nonetheless, early diagnosis, rigorous follow-up, and patient education remain essential components in minimizing disease progression and enhancing the quality of life for affected individuals. Full article

Background and Clinical Significance: Basal cell nevoid carcinoma syndrome, or Gorlin–Goltz Syndrome (GGS), is a genetic disease caused by germline mutations in genes involved in the Sonic HedgeHog (SHH) signaling pathway, mainly in the PTCH1 gene. PTCH1 is a receptor for SHH, and the activation of SHH signaling exerts a direct effect on the proliferation and maintenance of stem cells; alteration of its signaling could promote a favorable microenvironment for the maintenance of tumor viability. The main clinical manifestations of patients with GGS include multiple basal cell carcinomas, odontogenic keratocysts, calcification of the falx cerebri, palmoplantar fossae, hypertelorism, prognathism, fused or bifid ribs, and macrocephaly, which occur at different stages of life. Case Presentation: Here, the case of a 48-year-old woman is described, for whom a clinical and histopathological diagnosis of GGS was made due to the presence of two major criteria (multiple odontogenic keratocysts and calcification of the falx cerebri) and one minor criterion (congenital anomalies), according to Kimonis. Additionally, an end-point RT-PCR assay showed a decrease in PTCH1 gene expression. A conservative therapy was established, and satisfactory results were obtained in a follow-up period of 18 months. Conclusions: Kimonis' clinical criteria are important for establishing the diagnosis of Gorlin syndrome. Full article

Background/Objectives: Odontomas are the most common mixed odontogenic tumors and may cause impaction of adjacent teeth and masticatory disorders. Treatment of tooth impaction caused by the presence of odontomas is related to their stages of root development and their positions in the alveolar bone. The aim of this case report is to present the combined surgical and orthodontic treatment in growing patients with odontomas and to discuss the treatment outcomes. Methods: Two growing patients, an 8-year-old boy and a 17-year-old girl, with large odontomas in the posterior maxillary region of the maxilla were presented. The tumors were found during a radiological examination, and the first molars on the affected sides were impacted due to the presence of odontomas. Results: The treatment plans included the surgical removal of the tumors. In the case of the younger patient, the impacted developing molar erupted spontaneously in the oral cavity one year and two months after surgery. An orthodontic traction of the impacted molar was successfully applied in the older patient. Conclusions: The presence of a large odontoma in the posterior segments may lead to a displacement and impaction of neighboring molars and malformation of their roots. Spontaneous eruption of the affected molar can be expected if the tumor is diagnosed and removed early before its root formation is completed; otherwise, an orthodontic extrusion is needed. Interdisciplinary cooperation is important to diagnose and plan the dental treatment in young patients with odontomas. Full article

The aim of this study was to verify whether the expression of proteins related to the formation of invadopodia, MT1-MMP, cortactin, Tks-4 and Tks-5 is associated with the degree of tumor invasiveness of different types of unicystic ameloblastomas. An immunohistochemical study was performed on 29 unicystic ameloblastoma (UA) samples, 9 conventional ameloblastoma (CAM) samples and 9 dental follicle (DF) samples. The potential for tumor invasiveness was assessed based on the immunoexpression of the following invadopodia-forming proteins: MT1-MMP, cortactin, Tks-4 and Tks5. Mural unicystic ameloblastoma (MUA) showed higher MT1-MMP, cortactin, Tks-4, and Tks-5 immunoexpression than luminal and intra-luminal types. Conventional ameloblastoma exhibited lower MT1-MMP, cortactin, and Tks-5 expression compared to MUA. MUA’s cystic capsule neoplastic cells had significantly higher MT1-MMP, cortactin, Tks-4, and Tks-5 expression than lumen cells. Dental follicles showed minimal expression. Neoplastic cells in the cystic capsule of mural unicystic ameloblastomas showed higher invadopodia-related protein expression than lumen and luminal/intraluminal cells, suggesting that proximity to the bone region influences the aggressive behavior of mural unicystic ameloblastomas more compared to other subtypes. Full article

Background/Objectives: Odontogenic tumors in pediatric patients are uncommon; among all, the intraosseous occurrence of odontogenic myxoma is very rare, accounting for almost 8.5–11.6% of all odontogenic tumors in children. The radiological appearance is highly variable and is often responsible for the diagnostic delay and treatment. Methods: We report a case of odontogenic myxoma occurring in the posterior mandible of a 12-year-old female, found on a panoramic radiograph performed for the delayed eruption of the second inferior molar, treated by conservative surgery. A comprehensive analysis of the literature was also carried out. Results: The radiological features of the presented case were unique, as the lesion was encompassed within the uncompleted (developing) dental crown still unerupted, as confirmed by the macroscopic appearance. Then, the differential diagnosis included odontogenic fibroma, immature dental pulp or follicle from a developing tooth, and ameloblastic fibrodontoma. The histological examination led to the final diagnosis of odontogenic myxoma. As for the literature analysis, after a PRISMA-based selection of the papers, a total of 23 articles (case reports and case series on odontogenic myxomas in pediatric patients, a total of 33 cases) were finally selected and studied; all the pertinent data are widely discussed within the paper. Conclusions: The current case highlights the importance of the radiological investigation in pediatric patients when a delayed eruption lasts for several months, leading to an early diagnosis necessary to avoid more aggressive surgical therapies and possible recurrence; data from the literature about site of occurrence, sex, age, kind of surgical procedure, and recurrence rate are discussed too. Full article

Objective: To determine the prevalence of oral and maxillofacial lesions among patients at King Abdulaziz University from January 2016 to December 2022. Methods: This cross-sectional observational study included patients diagnosed with oral and maxillofacial intra-bony lesions based on radiological findings and confirmed by histopathological examination. The lesions were classified according to the fourth edition of the World Health Organization Classification of Head and Neck Tumors. Results: This study included 237 patients with a mean age of 31.53 ± 14.97 years, of which 45.1% were female. Most patients (46.7%) had mandibular lesions, followed by maxillary lesions (35.9%). Only 2.95% of the tumors were malignant. Odontogenic cysts were the most prevalent (65.40%), with radicular cysts and keratocytes being the most common types. The most prevalent tumor types were odontoma and ameloblastoma. The most malignant lesion was multiple myeloma. Conclusions: Our findings reveal that mandibular cystic lesions predominated, and emphasize the low incidence of malignancy in the study population. They provide valuable insights into the oral and maxillofacial lesion landscape at a high-volume tertiary care center. Full article

Peripheral ameloblastoma (PA) is a rare benign tumor that can occur mostly in the mandibular gingiva of the premolar area, originating from the residual odontogenic epithelium. The patient is usually asymptomatic and the lesion can be an occasional finding during routine intraoral examination. Due to the lack of clinical and radiographic pathognomonic features, the diagnosis is based on histopathological analysis, associated with 3D computed tomography (CT) imaging. Here, we report the case of a middle-aged man showing an asymptomatic, sessile, normochromic papillomatous mass of the lingual alveolar mucosa, in correspondence of tooth 4.4, which was histologically diagnosed as peripheral ameloblastoma. After the complete excision of the lesion, there were no recurrence and no symptoms during the 3-year follow-up. The treatment of choice for PA is a conservative surgical excision, which usually results in a good prognosis, together with a long-term follow-up, necessary to intercept possible recurrence or, more rarely, malignant transformation. Full article

Background and Clinical Significance: Among the odontogenic tumors, ameloblastoma is one of the most notorious, although it remains relatively rare, accounting for approximately one percent of all oral tumors. This neoplasm, derived from odontogenic epithelium, may arise from the developing enamel organ, epithelial cell rests of dental lamina, epithelial lining of odontogenic cysts, and basal cells of oral epithelium. This is a case presentation of a mural unicystic ameloblastoma, the most aggressive subtype and the one with the highest chance of recurrence. Case Presentation: A patient was referred by his dentist for root canal treatment at the Emergency Dental Clinic of Boston University. The patient complained of mandibular numbness. A panoramic radiograph was acquired, revealing a radiolucent lesion in the right mandible. Clinical examination detected a soft swelling perforating the buccal cortex in the area of #27–#30. A Cone-Beam CT (CBCT) was acquired in the Oral and Maxillofacial Radiology Clinic revealing a well-defined, partially corticated entity in the periapical area of teeth #27 through #30, with evidence of scalloping borders. The internal structure was unilocular and uniformly low-density. The entity caused interruption of the lamina dura of the associated teeth and inferior displacement of the inferior alveolar canal. Differential diagnoses included unicystic ameloblastoma (UA) and central giant cell granuloma as a second less likely diagnosis. An incisional biopsy was performed for further evaluation. Biopsy confirmed UA with mural involvement. Conclusions: UAs typically exhibit less aggressive behavior. However, cases like this one, where mural involvement is noted and no associated impaction is detected, underline the possibility of variable radiographic presentation and the significance of a multidisciplinary approach in correct diagnosis and treatment. Histological subtyping is crucial for guiding treatment. Full article

Head and neck cancers (HNCs) arise from anatomically adjacent sites and subsites, with varying etiological factors, diagnostic strategies, prognoses, and treatment approaches. While conventional squamous cell carcinoma (SCC) is the most common histology in the head and neck district, HNCs encompass a variety of rare histopathological entities, categorized into epithelial tumors such as salivary gland cancers, sinonasal tumors, neuroendocrine tumors, malignant odontogenic tumors, and SCC variants versus non-epithelial tumors including soft tissue sarcomas, mucosal melanomas, and hematological malignancies. Rare HNCs (R-HNCs) represent a diagnostic and clinical challenge, requiring histopathological expertise, the availability of peculiar molecular analysis, and the personalization of local and systemic treatments, all guided by a multidisciplinary tumor board. Here, we provide a comprehensive literature review on R-HNCs, emphasizing key histopathological and molecular characteristics that are crucial for guiding treatment decisions. An insight about the latest developments in systemic treatments is also reported. Full article

Background and objectives: Histopathological similarities between craniopharyngioma (CP) and ameloblastoma (AB) have long been recognized, particularly the shared features of palisading columnar epithelium and stellate reticulum-like areas. This study aimed to investigate potential odontogenic differentiation in CP akin to AB using immunohistochemical odontogenic markers. Methods: We analyzed AMELX, ODAM, and CK19 expression in 44 cases (20 CP and 24 AB). Results: While AMELX and ODAM showed diffuse strong positive expression in both tumors with no significant statistical differences, CK19 expression was notably higher in CP. Conclusion: The markers AMELX and ODAM associated with odontogenic differentiation exhibited similar profiles in both tumors due to shared similar embryological origins. Notably, CK19, a biomarker of odontogenic epithelium, showed even higher expression, suggesting distinct pathways. These findings offer insights into tumor biology and may aid in diagnostic and therapeutic approaches. Full article

Ameloblastoma is a rare, benign, but locally aggressive odontogenic tumor that originates from the epithelial cells involved in tooth development. The surgical approach to treating an ameloblastoma depends on the type, size, location, and extent of the tumor, as well as the patient’s age and overall health. This umbrella review’s aim is to summarize the findings from systematic reviews (SRs) and meta-analyses on the effect of radical or conservative treatment of ameloblastoma on the recurrence rate and quality of life, to evaluate the methodological quality of the included SRs and discuss the clinical management. Three electronic databases (PubMed, Scopus, The Cochrane Library) were checked. The primary outcome was the recurrence rate after surgical treatment, while the secondary outcomes were the post-operative complications, quality of life, esthetic, and functional impairment. The methodological quality of the included SRs was assessed using the updated version of “A Measurement Tool to Assess Systematic Review” (AMSTAR-2). Eighteen SRs were included. The quality of the included reviews ranged from critically low (three studies) to high (eight studies). Four studies were included in meta-analysis, and they revealed that the recurrence rate is about three-times more likely in the conservative treatment group compared to the radical treatment group, and this result is statistically significant. Despite the high recurrence rate, the latter was more appropriate in the case of smaller lesions and younger patients, due to better post-operative quality of life and reduced functional and esthetic impairments. Based on the results of this overview, conservative treatment may be recommended as the first-line approach for intraosseous ameloblastoma not involving soft tissue. However, given the expectation of a higher recurrence rate, it is advisable to reduce the interval between follow-up visits. However, further prospective studies are needed to establish the best treatment choice and follow-up period. Full article

A squamous odontogenic tumor (SOT) is an epithelial locally benign neoplasia derived from the periodontium of the jaws. It is considered a lesion of low incidence. Predominantly, it affects the mandible, although both jaw bones may be involved. Here, we discuss the malignant clinical evolution of an SOT lesion in an 80-year-old female patient. The patient exhibited an expansive triangular lesion at the inferior right quadrant. Surgery was performed and an SOT was diagnosed (2019). Two years after, the lesion grew, and the analysis of the biopsy revealed SOT malignization with pleomorphic atypical squamous cells, characteristics of a squamous cell carcinoma (2021). Massive DNA sequencing of formalin-fixed–paraffin-embedded specimens of the initial and relapsed tumors indicated pathogenic mutations in RET and POLE genes in both tumors, loss of ALK, and gain of CDKN1B and MAP2K in the relapse. In addition, the clinical, radiographic, and microscopic features of this neoplasm are discussed and compared with those already published. The case presented contributes to the better understanding of this SOT tumor entity and to indicates its malignant evolution, together with its biological behavior and its histologic, clinical, and radiographic features. Also, it aims to stress the importance of deeper genetic analyses in rare diseases to uncover mutations that help to select a personalized treatment. Full article