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BMC Oral Health Jun 2024Ameloblastic fibrosarcoma (AFS) is a rare malignant odontogenic tumor, commonly occurring in young adults and typically affecting the mandibular region. We report an... (Review)
Review
BACKGROUND
Ameloblastic fibrosarcoma (AFS) is a rare malignant odontogenic tumor, commonly occurring in young adults and typically affecting the mandibular region. We report an exceptionally rare and highly atypical case of AFS in an elderly female patient originating from the maxillary bone.
CASE PRESENTATION
A 66-year-old woman was admitted with a two-week history of a lump in her left upper molar. CT scans suggested a cyst in the maxillary bone. An incisional biopsy revealed a spindle cell neoplasm. MRI showed abnormalities in the left maxilla, indicating a possible tumorous lesion. The patient underwent a subtotal maxillectomy, wide tumor excision, intraoral epithelial flap transplantation, and dental extraction. Histology identified atypical tumor cells with visible mitotic figures. Immunohistochemistry showed negative for PCK and CD34 expression, but positive for Vimentin and SMA expression. The Ki-67 proliferation index ranged from 30 to 50%. These findings suggested a potentially malignant soft tissue tumor in the left maxilla, leaning towards a diagnosis of AFS. The patient received postoperative radiotherapy. There was no recurrence during the six-month follow-up.
CONCLUSION
Based on repeated pathological evidence, we report a rare case of an elderly female with AFS originating from the maxillary bone. Surgery and postoperative radiotherapy resulted in a favorable outcome.
Topics: Humans; Female; Aged; Maxillary Neoplasms; Odontogenic Tumors; Fibrosarcoma; Tomography, X-Ray Computed; Vimentin; Magnetic Resonance Imaging
PubMed: 38937725
DOI: 10.1186/s12903-024-04509-x -
Oral and Maxillofacial Surgery Jun 2024This study aims to report clinicopathologic and imaging features of odontogenic myxomas (OM), highlighting uncommon findings.
PURPOSE
This study aims to report clinicopathologic and imaging features of odontogenic myxomas (OM), highlighting uncommon findings.
METHODS
Clinicopathologic and imaging data of OMs diagnosed in the five Brazilian diagnostic pathology centers were collected and analyzed.
RESULTS
The series comprised 42 females (68.9%) and 19 males (31.1%), with a 2.2:1 female-to-male ratio and a mean age of 34.5±15.4 years (range: 4-80). Clinically, most OMs presented as painless intraoral swelling (n = 36; 70.6%) in the mandible (n=37; 59.7%). Multilocular lesions (n=30; 83.3%) were more common than unilocular lesions (n=6; 16.7%). There was no statistically significant difference between the average size of unilocular and multilocular OMs (p=0.2431). The borders of OMs were mainly well-defined (n=24; 66.7%) with different degrees of cortication. Only seven tumors caused tooth resorption (15.9%), while 24 (54.5%) caused tooth displacement. Cortical bone perforation was observed in 12 (38.7%) cases. Morphologically, OMs were characterized mainly by stellate or spindle-shaped cells in a myxoid background (n=53; 85.5%). Surgical resection was the most common treatment modality (n=15; 65.2%), followed by conservative surgery (n=8; 34.8%). Outcomes were available in 20 cases (32.3%). Seven of these patients had local recurrence (35%). Enucleation was the treatment with the highest recurrence rate (4/7; 57.1%).
CONCLUSIONS
OM has a predilection for the posterior region of the jaws of female adults. Despite their bland morphological appearance, they displayed diverse imaging features. Clinicians must include the OM in the differential diagnosis of osteolytic lesions of the jaws. A long follow-up is needed to monitor possible recurrences.
PubMed: 38922493
DOI: 10.1007/s10006-024-01271-w -
International Journal of Applied &... 2024Dentinogenic ghost cell tumor (DGCT), a variant of the calcifying odontogenic cyst, is considered to be a benign epithelial and mesenchymal neoplasm containing aberrant...
Peripheral Dentinogenic Ghost Cell Tumor- Diagnostic Challenge in a Gingival Epulis: Report of a Case with Update of all Dentinogenic Ghost Cell Cases Reported in English Literature.
Dentinogenic ghost cell tumor (DGCT), a variant of the calcifying odontogenic cyst, is considered to be a benign epithelial and mesenchymal neoplasm containing aberrant epithelial keratinization, ghost cells, and spherical calcifications. While there can be peripheral and central variants of this entity, the extraosseous type is rarer and usually innocuous in its clinical presentation. The aim of this article is to report a case of peripheral DGCT on the maxillary anterior region in a 14-year-old female evolving for 5 years and to emphasize the importance of histopathologic examination of gingival growths to avoid diagnostic pitfalls. A compilation of all reported cases in the English literature till date with details on the site, size, age sex, symptoms radiographic features, treatment follow-up, and recurrences has been attempted for better understanding of the biologic nature of this rare neoplasm.
PubMed: 38912354
DOI: 10.4103/ijabmr.ijabmr_506_23 -
Journal of Maxillofacial and Oral... Jun 2024The Cementoblastoma is a benign ectomesenchymal odontogenic neoplasm of the jaw, arising from an exuberant production of cemental tissue continuous with the roots of...
INTRODUCTION
The Cementoblastoma is a benign ectomesenchymal odontogenic neoplasm of the jaw, arising from an exuberant production of cemental tissue continuous with the roots of teeth (usually mandibular posteriors), resulting in a calcified mass adherent with the root apices.
CASE PRESENTATION
A curious case of a large Cementoblastoma, the size and shape of a ping pong ball, is presented, the first of its kind ever reported to involve the internal / medial / lingual aspect of the mandible, and presenting with unusual and deviant features.
DISCUSSION & CONCLUSION
The Cementoblastoma is usually associated with certain inherent and pathognomonic features, making it rather straightforward to identify and diagnose. Strikingly uncharacteristic clinical, radiographic and histologic attributes presented in this case, made this 'out of the ordinary' and 'Maverick'-like Cementoblastoma, arduous to diagnose, nevertheless, making it an interesting, informative and curious Case Study. This report has brought to light for the first time, several new facets of the Cementoblastoma.
PubMed: 38911399
DOI: 10.1007/s12663-024-02181-w -
Pathology, Research and Practice Jun 2024Odontogenic tumors (OGTs), which originate from cells of odontogenic apparatus and their remnants, are rare entities. Primary intraosseous carcinoma NOS (PIOC), is one...
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 β-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/β-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 β-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/β-catenin signaling may be associated with the pathogenesis of PIOC deriving from odontogenic keratocyst in which YAP signaling is activated.
PubMed: 38908335
DOI: 10.1016/j.prp.2024.155420 -
Indian Journal of Pathology &... Jun 2024Odontomes are the most common benign odontogenic tumors of the jaw which are usually slow-growing and non-aggressive. They are generally asymptomatic and diagnosed...
Odontomes are the most common benign odontogenic tumors of the jaw which are usually slow-growing and non-aggressive. They are generally asymptomatic and diagnosed during routine radiographic investigations and are mostly associated with tooth eruption disturbances. The present case series report covers seven cases of odontomes discussing on clinical, radiographic, and histopathologic features (ground section and decalcification) along with a brief review of the literature.
PubMed: 38904470
DOI: 10.4103/ijpm.ijpm_628_23 -
Oral Oncology Aug 2024Ghost cell odontogenic carcinoma (GCOC) is defined as a rare type of odontogenic carcinoma that is characterized by ghost cells and occasional dentinoid. However, the... (Review)
Review
β-catenin nuclear translocation and WNT pathway mutations in ghost cell odontogenic carcinoma: A literature review and proposal of a new molecular-based classification "WNT pathway-altered malignant odontogenic tumor".
Ghost cell odontogenic carcinoma (GCOC) is defined as a rare type of odontogenic carcinoma that is characterized by ghost cells and occasional dentinoid. However, the current classification system based primarily on the presence of ghost cells has limitations in the diagnosis of GCOC and its histologic mimics including odontogenic carcinoma with dentinoid (OCD). This study reviewed previous studies on β-catenin nuclear translocation and WNT pathway mutations in GCOC and OCD and discussed the potential utility of a new molecular-based classification "WNT pathway-altered malignant odontogenic tumor" for these rare odontogenic tumors.
Topics: Humans; beta Catenin; Odontogenic Tumors; Wnt Signaling Pathway; Mutation; Cell Nucleus
PubMed: 38901368
DOI: 10.1016/j.oraloncology.2024.106907 -
F1000Research 2022Various stemness markers (SOX2, OCT4, and NANOG) have been studied in odontogenic cysts and tumors. However, studies on SALL4 having similar properties of stemness has...
BACKGROUND
Various stemness markers (SOX2, OCT4, and NANOG) have been studied in odontogenic cysts and tumors. However, studies on SALL4 having similar properties of stemness has not been documented. Additionally, insight into fascin as a migratory molecule is less explored. In this study, the expression of SALL4 and fascin were evaluated in ameloblastoma, adenomatoid odontogenic tumor (AOT), odontogenic keratocyst (OKC), dentigerous cyst (DC), radicular cyst (RC), and calcifying odontogenic cyst (COC).
METHODS
Semi-quantitative analysis of fascin and SALL4 immuno-positive cells was done in a total of 40 cases of ameloblastoma (11 plexiform, 12 follicular, 12 unicystic, and 5 desmoplastic) variants, 6 cases of AOT, 15 each of OKC, DC, RC and 5 of COC. Chi-square test was applied to evaluate the association between SALL4 and fascin expression in odontogenic cysts and tumors.
RESULTS
Fascin immunopositivity was observed in peripheral ameloblast-like cells, and weak or absent in stellate reticulum-like cells. A moderate to weak immune-reactivity to SALL4 was observed in the cytoplasm of ameloblastoma, epithelial cells of dentigerous and radicular cysts, having a marked inflammatory infiltrate, which is an interesting observation. COC and AOT had negative to weak expressions. No recurrence has been reported.
CONCLUSIONS
Expression of fascin in ameloblastomas elucidate their role in motility and localized invasion. Its expression in less aggressive lesions like DC, COC, AOT will incite to explore the other functional properties of fascin. SALL4 expression in the cytoplasm of odontogenic cysts and tumors may represent inactive or mutant forms which requires further validation.
Topics: Humans; Transcription Factors; Microfilament Proteins; Odontogenic Cysts; Carrier Proteins; Immunohistochemistry; Ameloblastoma; Odontogenic Tumors; Biomarkers, Tumor
PubMed: 38895097
DOI: 10.12688/f1000research.126091.3 -
Journal of Clinical Medicine May 2024The World Health Organization's (WHO) 2022 update on the classification of odontogenic and maxillofacial bone tumors has revolutionized diagnostic and treatment...
The World Health Organization's (WHO) 2022 update on the classification of odontogenic and maxillofacial bone tumors has revolutionized diagnostic and treatment paradigms by integrating novel molecular insights. Fibro-osseous lesions of the maxillo-facial bones constitute a heterogeneous group encompassing fibrous dysplasia, Psammomatoid Ossifying Fibroma (PSOF), Juvenile Trabecular Ossifying Fibroma (JTOF), and other variants. Despite histological similarities, their distinct clinical manifestations and prognostic implications mandate precise differentiation. The intricacies of diagnosing fibro-osseous lesions pose challenges for pathologists, maxillofacial surgeons, dentists and oral surgeons, underscoring the importance of a systematic approach to ensure optimal patient management. Herein, we present two cases, fibrous dysplasia and Cemento-Ossifying Fibroma, detailing their clinical encounters and management strategies. Both patients provided informed consent for publishing their data and images, adhering to ethical guidelines.
PubMed: 38892944
DOI: 10.3390/jcm13113233 -
BMC Oral Health Jun 2024Odontogenic carcinoma with dentinoid (OCD) is a rare and controversial entity, which has not yet been included in the current World Health Organization classification of... (Review)
Review
BACKGROUND
Odontogenic carcinoma with dentinoid (OCD) is a rare and controversial entity, which has not yet been included in the current World Health Organization classification of odontogenic lesions. Owing to the small number of reported cases, the clinicopathological characteristics, biological behavior, prognosis, and appropriate treatment strategies for OCD remain to be defined. Herein, we present an additional case of OCD with a focus on the differential diagnosis and review of the pertinent literature, in order to enable better recognition by oral clinicians and pathologists and further characterization of this entity.
CASE PRESENTATION
This paper reports a case of OCD in the posterior mandible of a 22-year-old female. Radiography showed a well-defined unilocular radiolucency with radiopaque materials. The intraoperative frozen section pathology gave a non-committed diagnosis of odontogenic neoplasm with uncertain malignant potential. Then a partial mandibulectomy with free iliac crest bone graft and titanium implants was performed. Microscopically, the tumor consisted of sheets, islands, and cords of round to polygonal epithelial cells associated with an abundant dentinoid matrix. Immunohistochemically, the tumor cells were diffusely positive for CK19, p63, and β-catenin (cytoplasmic and nuclear). No rearrangement of the EWSR1 gene was detected. The final diagnosis was OCD. There has been no evidence of recurrence or metastasis for 58 months after surgery. We also provide a literature review of OCD cases, including one case previously reported as ghost cell odontogenic carcinoma from our hospital.
CONCLUSIONS
OCD is a locally aggressive low grade malignancy without apparent metastatic potential. Wide surgical excision with clear margins and long-term period follow-up to identify any possible recurrence or metastases are recommended. Histopathological examination is essential to conclude the diagnosis. Special care must be taken to distinguish OCD from ghost cell odontogenic carcinoma and clear cell odontogenic carcinoma, as misdiagnosis might lead to unnecessary overtreatment. Study of additional cases is required to further characterize the clinicopathological features and clarify the nosologic status and biological behavior of this tumor.
Topics: Female; Humans; Young Adult; beta Catenin; Diagnosis, Differential; Keratin-19; Mandibular Neoplasms; Odontogenic Tumors; Transcription Factors; Tumor Suppressor Proteins
PubMed: 38890602
DOI: 10.1186/s12903-024-04471-8