Multilocular Radiolucent Pathology in the Body and Ramus of the Mandible: A Case Report.

Odontogenic keratocyst (OKC), a type of epithelial developmental cyst, is frequently found in the jaw region. It has invasive characteristics such as satellite cysts, rapid progression, and tissue expansion. The OKC often favors the mandibular angle and ascending ramus. OKC symptoms include pain, swelling, displacement or malpositioning of adjacent teeth, and erosion or thinning of the limited or no bucco-lingual cortical expansion. There is radiographic evidence of a distinct, often scalloped, radiolucent lesion with a characteristic "soap bubble" or "honeycomb" appearance. This article reports a female patient, aged 40 years, with the main concern of unilateral pain and swelling of the mandibular left side and the provisional diagnosis of ameloblastoma. After histopathological examination, the final diagnosis of the patient was OKC. This article also includes previously published literature on OKC with differential diagnosis and relevant clinical and radiologic findings of the case.

Concordance of clinician, Chat-GPT4, and ORAD diagnoses against histopathology in Odontogenic Keratocysts and tumours: a 15-Year New Zealand retrospective study.

BACKGROUND: This research aimed to investigate the concordance between clinical impressions and histopathologic diagnoses made by clinicians and artificial intelligence tools for odontogenic keratocyst (OKC) and Odontogenic tumours (OT) in a New Zealand population from 2008 to 2023. METHODS: Histopathological records from the Oral Pathology Centre, University of Otago (2008-2023) were examined to identify OKCs and OT. Specimen referral details, histopathologic reports, and clinician differential diagnoses, as well as those provided by ORAD and Chat-GPT4, were documented. Data were analyzed using SPSS, and concordance between provisional and histopathologic diagnoses was ascertained. RESULTS: Of the 34,225 biopsies, 302 and 321 samples were identified as OTs and OKCs. Concordance rates were 43.2% for clinicians, 45.6% for ORAD, and 41.4% for Chat-GPT4. Corresponding Kappa value against histological diagnosis were 0.23, 0.13 and 0.14. Surgeons achieved a higher concordance rate (47.7%) compared to non-surgeons (29.82%). Odds ratio of having concordant diagnosis using Chat-GPT4 and ORAD were between 1.4 and 2.8 (p < 0.05). ROC-AUC and PR-AUC were similar between the groups (Clinician 0.62/0.42, ORAD 0.58/0.28, Char-GPT4 0.63/0.37) for ameloblastoma and for OKC (Clinician 0.64/0.78, ORAD 0.66/0.77, Char-GPT4 0.60/0.71). CONCLUSION: Clinicians with surgical training achieved higher concordance rate when it comes to OT and OKC. Chat-GPT4 and Bayesian approach (ORAD) have shown potential in enhancing diagnostic capabilities.

Endodontic microsurgery and prosthodontic treatment of a permanent anterior concrescence: A case report.

Concrescence is a rare dental anomaly in which two adjacent teeth are united only by their cementum. Concrescence most frequently occurs in molars, especially a third mandibular molar and a supernumerary tooth. It is rarely seen in the maxillary anterior teeth. This case report is the first in the literature which details the successful treatment of a concrescence between the maxillary central incisor and a supernumerary tooth through multidisciplinary therapy. The treatment plan included root canal treatment, endodontic microsurgery, and prosthodontic treatment.

Lymphangiogenesis in Odontogenic Keratocysts Compared with Dentigerous Cysts.

Statment of the Problem: Podoplanin can indicate the lymphangiogenesis. On the other hand, lymphangiogenesis affects the biological behavior of lesions. The clinical behavior of odontogenic keratocysts (OKC) and dentigerous cysts (DC) is different. Purpose: This study aimed to evaluate the immunohistochemical expression of podoplanin and to investigate lymphangiogenesis in OKCs as compared to DCs. Materials and Method: In this experimental laboratory study, sixty paraffined blocks, including 30 OKC and 30 DC samples, were examined in this study, all of which were histopathologically non-inflamed. To evaluate lymphangiogenesis, the immunohistochemical reaction of D2-40 was evaluated via cytoplasmic and membrane staining of lymphatic endothelial cells. The expression of podoplanin in the epithelial cells of two cyst groups was also examined. To analyze the collected data and compare the results between the two groups of cysts, independent samples t-test, Mann-Whitney U test, and Chi-square test were performed in SPSS version 22. The significance level was set at 0.05. Results: The mean lymph node count and podoplanin expression were significantly higher in the OKC epithelium as compared to DC (p< 0.001). Based on the results, 90% of OKC samples and 43.3% of DC samples showed grade 3 staining. Conclusion: The rate of lymphangiogenesis and podoplanin expression in the epithelium were higher in OKCs compared to DCs. According to the results, the expression of podoplanin may be a useful marker for determining the invasiveness and proliferation of OKC.

Evaluation of Paxillin Expression in Odontogenic Cysts and Tumors.

Statement of the Problem: Paxillin (PXN) is one of the proteins involved in cell adhesion. PXN and integrins constitute a key site for the focal adhesion between the cell and extracellular matrix. Several studies have shown that PXN is a factor in tumor formation, progression, invasion, and metastasis. Purpose: This study evaluated PXN expression in four types of odontogenic lesions with different aggressive behaviors. Materials and Method: In this retrospective cross-sectional study, PXN expression was immunohistochemically assessed in 68 paraffin-embedded tissue samples from patients with the confirmed diagnosis of four types of odontogenic lesions, including 14 dentigerous cysts (DC), 20 odontogenic keratocyst (OKC), 16 unicystic ameloblastoma, and 18 solid ameloblastoma. The PXN expression in these samples were scored based on the percentage and intensity of immunoreactivity, and compared among the groups by Chi-square test. Results: The PXN marker was detected in the cytoplasm of tumor cells (unicystic and solid ameloblastoma) and the epithelial layer of cysts (DC and OKC). The intensively stained marker of PXN was observed in 9 cases (64.3%) of the DC, 14 cases (70%) of OKC, 12 cases (75%) of unicystic ameloblastoma, and 13 cases (72.2%) of solid ameloblastoma. However, there was not statistical difference of PXN protein expression between DC and OKC (p Value = 0.51) and unicystic and solid ameloblastoma (p = 0.58), also the same was true for cysts and tumors (p = 0.37). Conclusion: The expression of PXN is not related to the biological behaviors of odontogenic lesions.

Management and recurrence of the odontogenic keratocyst: an overview of systematic reviews.

MATERIALS AND METHODS: An extensive literature search among six eletronic databases and Grey Literature was used to identify systematic reviews (S) that could respond if: in individuals diagnosed with OKC (P), is there any influence of the treatment method (I/C) on the recurrence rate of the lesion (O)? After evaluating all titles and abstracts and then applying the eligibility criteria, the included studies were read in full, and data were extracted based on a standardized sheet ordered in the PICO sequence. The assessment of the quality of the systematic reviews included, was determined by AMSTAR2, and final synthesis were descriptively made based on the results and quality of the systematic reviews. RESULTS: From a total of 19 included systematic reviews, it was observed that the most used treatment for OKC was enucleation, followed by enucleation with adjuvant techniques and marsupialization. The mean percentage of recurrence was 16,2%, and the highest OKC recurrence rate was 43.2% after simple enucleation. The use of adjuvant techniques promoted reductions in OKC recurrence rates. The overall methodological quality of systematic reviews was critically low, and this parameter demonstrate the need for more studies to facilitate the choose of the treatment. CONCLUSION: Despite being the most used treatment, simple enucleation is related to the highest rate of recurrence, except when performed after marsupialization/decompression. In addition, the use of adjuvant techniques has a strong impact on reducing the likelihood of recurrence. However, these findings are not conclusive because of the critically low quality of the systematic reviews.

Characterization of stromal calcifications in odontogenic keratocyst: a multicentric study.

Odontogenic keratocysts (OKCs) are locally aggressive cysts that exhibit typical histopathological features and have a propensity for recurrence. Though histological variations are observed in OKCs, hard tissue formation and metaplastic changes are rare, and the underlying pathogenesis is not well understood. This study aimed to characterize stromal calcifications and analyze their association with odontogenic components in non-syndromic and syndrome-associated cases of OKCs. We analyzed 153 cases of OKCs from healthcare institutes in India and Japan. The epithelial and stromal features were evaluated, and the relationship of calcifications with odontogenic rests was determined. Immunohistochemistry for cytokeratin-19 and special stains including Masson Trichrome and Van Gieson, were used for identification of odontogenic rests and calcifications respectively. Stromal calcifications were observed in 29.41% OKCs. The calcification patterns included irregular dystrophic, dentinoid with linear or calcospherite-type mineralization, and psammoma calcifications. Psammoma and dentinoid calcifications were found in the proximity of cytokeratin-19-positive odontogenic rests or satellite cysts, whereas majority cases with dystrophic calcifications did not exhibit co-localization with stromal odontogenic components. Distinct patterns of calcifications were observed in OKCs. Calcifications found in proximity of the odontogenic rests were possibly indicative of an inductive or host-mediated response.

Wnt/ß-catenin-YAP axis in the pathogenesis of primary intraosseous carcinoma NOS, deriving from odontogenic keratocyst.

Odontogenic tumors (OGTs), which originate from cells of odontogenic apparatus and their remnants, are rare entities. Primary intraosseous carcinoma NOS (PIOC), is one of the OGTs, but it is even rarer and has a worse prognosis. The precise characteristics of PIOC, especially in immunohistochemical features and its pathogenesis, remain unclear. We characterized a case of PIOC arising from the left mandible, in which histopathological findings showed a transition from the odontogenic keratocyst to the carcinoma. Remarkably, the tumor lesion of this PIOC prominently exhibits malignant attributes, including invasive growth of carcinoma cell infiltration into the bone tissue, an elevated Ki-67 index, and lower signal for CK13 and higher signal for CK17 compared with the non-tumor region, histopathologically and immunohistopathologically. Further immunohistochemical analyses demonstrated increased expression of ADP-ribosylation factor (ARF)-like 4c (ARL4C) (accompanying expression of ß-catenin in the nucleus) and yes-associated protein (YAP) in the tumor lesion. On the other hand, YAP was expressed and the expression of ARL4C was hardly detected in the non-tumor region. In addition, quantitative RT-PCR analysis using RNAs and dot blot analysis using genomic DNA showed the activation of Wnt/ß-catenin signaling and epigenetic alterations, such as an increase of 5mC levels and a decrease of 5hmC levels, in the tumor lesion. A DNA microarray and a gene set enrichment analysis demonstrated that various types of intracellular signaling would be activated and several kinds of cellular functions would be altered in the pathogenesis of PIOC. Experiments with the GSK-3 inhibitor revealed that ß-catenin pathway increased not only mRNA levels of ankyrin repeat domain1 (ANKRD1) but also protein levels of YAP and transcriptional co-activator with PDZ-binding motif (TAZ) in oral squamous cell carcinoma cell lines. These results suggested that further activation of YAP signaling by Wnt/ß-catenin signaling may be associated with the pathogenesis of PIOC deriving from odontogenic keratocyst in which YAP signaling is activated.

Differential Diagnosis of OKC and SBC on Panoramic Radiographs: Leveraging Deep Learning Algorithms.

This study aims to determine whether it can distinguish odontogenic keratocyst (OKC) and simple bone cyst (SBC) based solely on preoperative panoramic radiographs through a deep learning algorithm. (1) Methods: We conducted a retrospective analysis of patient data from January 2018 to December 2022 at Pusan National University Dental Hospital. This study included 63 cases of OKC confirmed by histological examination after surgical excision and 125 cases of SBC that underwent surgical curettage. All panoramic radiographs were obtained utilizing the Proline XC system (Planmeca Co., Helsinki, Finland), which already had diagnostic data on them. The panoramic images were cut into 299 × 299 cropped sizes and divided into 80% training and 20% validation data sets for 5-fold cross-validation. Inception-ResNet-V2 system was adopted to train for OKC and SBC discrimination. (2) Results: The classification network for diagnostic performance evaluation achieved 0.829 accuracy, 0.800 precision, 0.615 recall, and a 0.695 F1 score. (4) Conclusions: The deep learning algorithm demonstrated notable accuracy in distinguishing OKC from SBC, facilitated by CAM visualization. This progress is expected to become an essential resource for clinicians, improving diagnostic and treatment outcomes.

2-Staged Surgical Protocol for Management of Large Odontogenic Keratocyst.

The World Health Organisation classification and the treatment protocol for the odontogenic keratocyst (OKC), previously referred to as the keratocystic odontogenic tumour, were examined based on a study of the literature. Because not all OKCs have an identifiable protein patched homolog mutation, the idea of changing the management protocol for OKC in response to this shift in tumour category was met with scepticism and was not widely adopted. This study's objective was to outline a successful management plan for an odontogenic keratocyst in a patient who was 23 years old. The procedure for therapy involved marsupialisation, which was followed by enucleation, peripheral osteotomy, and the injection of 5 FFU. Following a 2-year observation period (clinical and radiological monitoring), it was found that bone regeneration was normal and there was no sign of a recurrence.

Further Expanding the Mutational Spectrum of Gorlin Syndrome in Three Unrelated Families.

Introduction: Gorlin syndrome is a rare, autosomal dominant multi-systemic disorder with a predisposition to the development of cancers such as medulloblastoma and nevoid basal cell carcinoma. Heterozygous pathogenic variants in PTCH1 are responsible for 90% of Gorlin syndrome cases. Pathogenic variants in PTCH1 cause overstimulation of the sonic hedgehog signaling pathway, which plays a role in the development of embryonic structures and tumorigenesis. Clinical major and minor diagnostic criteria for Gorlin syndrome have been determined. Odontogenic keratocyst (OKC) is the most common reason for medical admission in Gorlin syndrome. In this article, it is aimed to draw attention to the fact that patients with Gorlin syndrome are not very rare in our country and the variability in phenotypic and dysmorphic findings may be a clue for the diagnosis. Methods: Exome sequencing was performed on the Illumina NextSeq550 System platform by using the Ion Ampliseq exome RDY kit for Illumina. Sanger sequencing was performed accordingly for the other affected individuals in both families. Results: In this study, the clinical and molecular findings of 9 Gorlin syndrome patients from three unrelated families are presented. Macrocephaly, calcification of falx cerebri, palmar-plantar pits, rib anomalies, and OKC were detected in decreasing order in more than half of the patients. A novel heterozygous frameshift PTCH1 variant in family 1, a nonsense previously reported PTCH1 variant in family 2, and a novel heterozygous splice-site PTCH1 variant in family 3 were detected. Conclusion: Gorlin syndrome should be kept in mind in patients presenting with macrocephaly, palmoplantar pits, and OKC history. Careful examination of all family members is essential in the timely diagnosis of other affected individuals with minor phenotypic findings.

Assessing Conservative Treatment Options for Odontogenic Keratocyst in a 12-Year-Old Patient, with 1-Year Follow-up.

The odontogenic keratocyst (OKC) is a frequently developing odontogenic cyst that accounts for 10%-14% of all jaw cysts. Due to the high recurrence rate, aggressive therapeutic techniques such as jaw resection and marsupialization are indicated. Following a conservative marsupialization procedure in which Carnoy's solution and an iodoform packing were used, the clinical, radiological, and histological evaluation of OKC in a 12-year-old female patient revealed no evidence of recurrence over the subsequent year of follow-up.

Application of machine learning in the preoperative radiomic diagnosis of ameloblastoma and odontogenic keratocyst based on cone-beam CT.

OBJECTIVES: Preoperative diagnosis of oral ameloblastoma (AME) and odontogenic keratocyst (OKC) has been a challenge in dentistry. This study uses radiomics approaches and machine learning (ML) algorithms to characterize cone-beam CT (CBCT) image features for the preoperative differential diagnosis of AME and OKC and compares ML algorithms to expert radiologists to validate performance. METHODS: We retrospectively collected the data of 326 patients with AME and OKC, where all diagnoses were confirmed by histopathologic tests. A total of 348 features were selected to train six ML models for differential diagnosis by a 5-fold cross-validation. We then compared the performance of ML-based diagnoses to those of radiologists. RESULTS: Among the six ML models, XGBoost was effective in distinguishing AME and OKC in CBCT images, with its classification performance outperforming the other models. The mean precision, recall, accuracy, F1-score, and area under the curve (AUC) were 0.900, 0.807, 0.843, 0.841, and 0.872, respectively. Compared to the diagnostics by radiologists, ML-based radiomic diagnostics performed better. CONCLUSIONS: Radiomic-based ML algorithms allow CBCT images of AME and OKC to be distinguished accurately, facilitating the preoperative differential diagnosis of AME and OKC. ADVANCES IN KNOWLEDGE: ML and radiomic approaches with high-resolution CBCT images provide new insights into the differential diagnosis of AME and OKC.

Management regulations for odontogenic keratocyst: a case report and review of the literature.

BACKGROUND: Reconstruction of the entire dentition with odontogenic keratocyst is a very challenging quandary. Most cases of odontogenic keratocyst are often reported to be benign, resulting in severe occlusal discrepancies with the maxillary and mandibular dentition. Dental radiographs occasionally reveal an uncommon, locally aggressive developing cyst termed as odontogenic keratocyst, which is typically located in the posterior jaw. When this cyst occurs in the anterior region, it is often misdiagnosed with other periapical lesions due to its lack of response to pulp vitality tests. CASE PRESENTATION: This clinical case scenario demarcates the endodontic management of a patient diagnosed with odontogenic keratocyst. A 37-year-old Indian male patient reported to the department with throbbing pain in the lower left posterior tooth requiring endodontic therapy. This patient also presented with odontogenic keratocyst in the anterior region of the jaw, for which he had undergone surgical rehabilitation. This case report highlights the clinical protocol for the endodontic therapy in patient diagnosed with ododntogenic keratocyst. Masticatory impairment was not visible after the follow-up period and the treatment outcome was successful. CONCLUSION: This case report details the presentation, characteristic radiographic findings, and endodontic management of a patient with an extremely rare condition of odontogenic keratocyst. The management involves multidisciplinary approach for the rehabilitation.

Odontogenic Keratocyst With Moderate Epithelial Dysplasia: A Rare Entity.

We present a rare case of odontogenic keratocyst (OKC) with moderate epithelial dysplasia in a 47-year-old male patient. He presented with a history of pain and swelling on the right side of his face for the past three months. The radiograph revealed multi-locular radiolucency involving the ramus, coronoid process, and condylar process of the right mandible. We have discussed the cytology, incisional biopsy, radiological investigations, surgical management, reconstruction, excisional biopsy report, and follow-up of this rare entity.

Gorlin-Goltz Syndrome: An Incidental Finding of a Rare Entity.

Gorlin-Goltz syndrome (GGS) is a rare hereditary disease characterized by multiple basal cell carcinomas, odontogenic keratocyst (OKCs) and musculoskeletal malformations. Pathogenesis of the syndrome is attributed to abnormalities in the long arm of chromosome 9 (q22.3-q31) and mutations in the human patched gene (PTCH1 gene). Here, we report a rare case of an incidental finding of GGS in an 18-year-old male patient presenting multiple OKCs, calcification of the falx cerebri, and bifid rib.

Gorlin-Goltz Syndrome - A Rare Case Entity in Young Child.

Gorlin-Goltz syndrome (GGS) is an infrequent multisystemic disease with an autosomal dominant trait, which depicted presence of numerous basal cell carcinoma in conjunction with multiorgan abnormalities. This syndrome may be diagnosed early by a dentist by routine radiographic exams in the first decade of life, since the keratocystic odontogenic tumour are usually one of the first manifestations of the syndrome. This article includes a case report of the GGS with regard to its history, incidence, etiology, features, investigations, diagnostic criteria, keratocystic odontogenic tumour and treatment modalities.

Immunohistochemical expression of SPARC in odontogenic keratocysts: a comparative study with other odontogenic cysts.

BACKGROUND: Secreted protein acidic and rich in cysteine (SPARC) has been shown to modulate aggressive behavior in several benign and malignant tumors. Little is known about SPARC expression in odontogenic keratocyst (OKC), an odontogenic cyst with an aggressive nature. To the best of our knowledge, only one study has been investigated the expression of this protein in OKCs. This study aimed to characterize SPARC expression in OKCs. Additionally, to determine whether SPARC is associated with aggressive behavior in OKCs, SPARC expression in OKCs was compared with radicular cysts (RCs), dentigerous cysts (DCs) and calcifying odontogenic cysts (COCs). These odontogenic cysts showed no or less aggressive behavior. METHODS: SPARC expression was evaluated in 38 OKCs, 39 RCs, 35 DCs and 14 COCs using immunohistochemistry. The percentages of positive cells and the intensities of immunostaining in the epithelial lining and the cystic wall were evaluated and scored. RESULTS: Generally, OKCs showed similar staining patterns to RCs, DCs and COCs. In the epithelial lining, SPARC was not detected, except for ghost cells in all COCs. In the cystic wall, the majority of positive cells were fibroblasts. Compared between 4 groups of odontogenic cysts, SPARC expression in OKCs was significantly higher than those of RCs (P < 0.001), DCs (P < 0.001) and COCs (P = 0.001). CONCLUSIONS: A significant increase of SPARC expression in OKCs compared with RCs, DCs and COCs suggests that SPARC may play a role in the aggressive behavior of OKCs.

Recurrence Patterns of Odontogenic Keratocysts in Syndromic and Non-Syndromic Patients.

Purpose: Odontogenic keratocysts (OKCs) have high recurrence rates. We aimed to identify recurrence patterns in OKCs and the onset of second primary OKCs in non-syndromic and syndromic patients. Material and Methods: Patients with OKCs reporting to our department from 1998 to 2021 (23 years) were retrospectively evaluated using demographic, clinical (age, sex, location, and size), histopathological, radiographic, and treatment data. All patients were followed-up for > 3 years and evaluated for OKC recurrence. Patients with naevoid basal cell carcinoma syndrome (NBCCS) were evaluated separately. Results: We included 38 and 13 patients in the non-syndromic and syndromic OKC groups, respectively. The recurrence rates were 15.8 and 21.4% in the non-syndromic and syndromic groups, respectively; 8.9% of patients exhibited a second recurrence and 1.8% a third recurrence. No second primary OKCs were observed in the non-syndromic group; 76.9% of patients in the syndromic group developed at least one. Conclusion: We found a higher recurrence rate in patients with NBCCS compared with patients with non-syndromic OKCs (21.4 versus 15.8%). The probability of developing a second primary OKC in patients with NBCCS was higher compared with that in patients with non-syndromic OKCs (76.9 versus 0%). No statistically significant risk factors for OKC recurrence were identified.

Comparison of Five Different Treatment Approaches of Mandibular Keratocystic Odontogenic Keratocyst (OKC): A Retrospective Recurrence Analysis of Clinical and Radiographic Parameters.

The odontogenic keratocyst (OKC) is a benign but locally aggressive growing lesion that infiltrates the bone and surrounding tissue. It is characterized by high rates of recurrence along with rapid growth. Different forms of partly successful treatment therapies are reported. The retrospective study at hand examined 114 patients with OKC treated over a period of 20 years. Data extracted includes gender, age, location, previous treatment for the lesion, surgery, outcome, recurrence rate and follow-up. 63.1% of the patients underwent cystectomy, 22.5% by cystectomy and carnoy solution, 7.2% by cystectomy, and curettage, 4.5% by cystostomy and 2.7% by partial resection. In this study, no significant differences could be observed regarding the surgical method. Most recurrences occurred with 91.9% in the mandible with an average size of 5.5 cm2 and increased in women. Within a mean follow-up time of 3.6 years the recurrence rate was 36.9%, on average after 36 months. Recurrences were most frequently diagnosed at the age of 31-50 (43.9%). Despite numerous studies, there is still no unanimous opinion on an effective therapy for OKC. However, precise resection of OKC can be facilitated by preoperative 3D-imaging and virtual planning.