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BMC Oral Health Mar 2024Ameloblastoma (AM) is the most common benign odontogenic tumor, which is more often detected in the mandible than maxilla, especially the mandibular body and mandibular...
BACKGROUND
Ameloblastoma (AM) is the most common benign odontogenic tumor, which is more often detected in the mandible than maxilla, especially the mandibular body and mandibular angle. Pediatric AM is a rare disease, especially in patients aged 10 and younger. Compared with the mainstream osteotomy and reconstructive surgery for adult ameloblastoma, there is more room for discussion in the treatment of pediatric ameloblastoma. The postoperative functional and psychological influence can not be ignored. Especially for children in the period of growth and development, an osteotomy is often challenging to be accepted by their parents. We report two patients with ameloblastoma under 10 years old who are treated with curettage and fenestration, which is a beneficial method for children with ameloblastoma.
CASE PRESENTATION
We present two cases of classic ameloblastoma in children. We describe in detail the patients' characteristics, treatment processes, and follow-up result. The bone formation and reconstruction in the lesion area after fenestration decompression and curettage are recorded at every clinic review. The surgical details and principles of curettage and decompression are also described and discussed. The two patients have good bone shape recovery and no recurrence.
CONCLUSIONS
Children are in the growth and development period and possess an extremely strong ability of bone formation and reconstruction. Based on the principles of minimally invasive and functional preservation, we believe that curettage combined with decompression can be the first choice for treating AM in children, especially for mandibular lesions.
Topics: Child; Humans; Ameloblastoma; Curettage; Decompression; Mandible; Mandibular Neoplasms
PubMed: 38519948
DOI: 10.1186/s12903-024-04126-8 -
Cureus Feb 2024Dentigerous cysts are the second most common developmental odontogenic cysts that develop around the crown of unerrupted teeth with the maxillary canine region being...
Dentigerous cysts are the second most common developmental odontogenic cysts that develop around the crown of unerrupted teeth with the maxillary canine region being one of the common sites of occurrence. The cystic lining of this lesion has been shown to develop into ameloblastoma, Muco epidermoid carcinoma, and squamous cell carcinomas. However, the development of cholesterol granuloma (CG) in the cystic lining of a dentigerous cyst is extremely rare. CG is a histological observation distinguished by the presence of a conglomeration of connective tissue and granulation tissue. The condition is predominantly seen in the field of otolaryngology, with very few cases reported in the maxillofacial region, most of which are associated with the maxillary sinus. This article presents the findings of a CG in a 39-year-old male patient that developed within the dentigerous cyst and discusses the possible etiopathogenesis, surgical management, and histological presentation.
PubMed: 38516497
DOI: 10.7759/cureus.54545 -
Case Reports in Ophthalmology 2024Maxillary ameloblastoma is a rare, slow-growing odontogenic tumor that can recur after surgical excision, be locally aggressive, and rarely develop systemic metastases....
INTRODUCTION
Maxillary ameloblastoma is a rare, slow-growing odontogenic tumor that can recur after surgical excision, be locally aggressive, and rarely develop systemic metastases. We describe the course and management of a patient with recurrent maxillary ameloblastoma with orbital invasion and systemic metastases, the fourth case of its kind to be described in the literature.
CASE PRESENTATION
A 50-year-old female presented with left hyperglobus. A diagnosis of maxillary ameloblastoma was made based on biopsy and neuroimaging with MRI and CT. Surgical management included partial maxillectomy with orbital floor reconstruction, given the orbital invasion. Three years later, left hyperglobus recurred, and the patient was found to have orbital recurrence and lung metastases on PET imaging. The lung and orbital lesions have responded well to chemoradiation therapy without surgical intervention.
CONCLUSION
Maxillary ameloblastoma is a rare tumor that typically arises from odontogenic tissues. Though considered benign, they can recur and in the case of our patient, metastasize. Complete surgical excision with wide surgical margins is associated with a shorter average time to recurrence and a lower incidence of metastasis. Cases of metastasis are managed with chemotherapy with or without adjuvant radiotherapy. Precision medicine may play a role in managing this entity in the future, given the discovery of differing profiles of maxillary ameloblastoma compared to mandibular. Ophthalmologists should be aware of this tumor as it can invade the orbit, resulting in significant ocular morbidity and mortality.
PubMed: 38504671
DOI: 10.1159/000537707 -
The American Journal of Case Reports Mar 2024BACKGROUND Adamantinoma is a rare low-grade malignant bone tumor, usually found in the tibial diaphysis and metaphysis, with histological similarities to mandibular...
BACKGROUND Adamantinoma is a rare low-grade malignant bone tumor, usually found in the tibial diaphysis and metaphysis, with histological similarities to mandibular ameloblastoma. The most effective treatment of recurrent adamantinoma is not yet clear. This report is of a 22-year-old woman with recurrent tibial adamantinoma treated with the tyrosine kinase inhibitor pazopanib. CASE REPORT We report the case of a 22-year-old woman who was referred to our center for a suspicious bone lesion in the right tibia. Bone biopsy findings were consistent with an adamantinoma. En bloc resection was completed successfully, with no postoperative complications. Five years later, a positive emission tomography scan revealed mildly increased tracer uptake near the area of the previous lesion and in the right inguinal lymph node. Biopsies of the lesion and inguinal lymph node confirmed recurrence of the adamantinoma. Due to abdominal and pelvic metastasis, the patient underwent surgical debulking, along with an appendectomy, right salpingo-oophorectomy, intraoperative radiation therapy, and hyperthermic intraperitoneal chemotherapy. Subsequently, the patient was placed on pazopanib for 4 months; however, her tumor continued to worsen after 4 months of chemotherapy. Currently, the patient is receiving gemcitabine and docetaxel as second-line medical therapy. CONCLUSIONS This report showed that pazopanib as standalone treatment does not appear to have promising role on patient outcomes. To the best of our knowledge, this is the second report of pazopanib in the treatment of adamantinoma.
Topics: Female; Humans; Young Adult; Adamantinoma; Ameloblastoma; Bone Neoplasms; Indazoles; Pyrimidines; Sulfonamides; Tibia
PubMed: 38486378
DOI: 10.12659/AJCR.941248 -
Biomedical Reports Apr 2024Ameloblastoma (AM) is a prominent benign odontogenic tumor characterized by aggressiveness, likely originating from tooth-generating tissue or the dental follicle (DF)....
Ameloblastoma (AM) is a prominent benign odontogenic tumor characterized by aggressiveness, likely originating from tooth-generating tissue or the dental follicle (DF). However, proteomic distinctions between AM and DF remain unclear. In the present study, the aim was to identify the distinction between AM and DF in terms of their proteome and to determine the associated hub genes. Shotgun proteomics was used to compare the proteomes of seven fresh-frozen AM tissues and five DF tissues. Differentially expressed proteins (DEPs) were quantified and subsequently analyzed through Gene Ontology-based functional analysis, protein-protein interaction (PPI) analysis and hub gene identification. Among 7,550 DEPs, 520 and 216 were exclusive to AM and DF, respectively. Significant biological pathways included histone H2A monoubiquitination and actin filament-based movement in AM, as well as pro-B cell differentiation in DF. According to PPI analysis, the top-ranked upregulated hub genes were ubiquitin C (UBC), breast cancer gene 1 (BRCA1), lymphocyte cell-specific protein-tyrosine kinase (LCK), Janus kinase 1 and ATR serine/threonine kinase, whereas the top-ranked downregulated hub genes were UBC, protein kinase, DNA-activated, catalytic subunit (PRKDC), V-Myc avian myelocytomatosis viral oncogene homolog (MYC), tumor protein P53 and P21 (RAC1) activated kinase 1. When combining upregulated and downregulated genes, UBC exhibited the highest degree and betweenness values, followed by MYC, BRCA1, PRKDC, embryonic lethal, abnormal vision, Drosophila, homolog-like 1, myosin heavy chain 9, amyloid beta precursor protein, telomeric repeat binding factor 2, LCK and filamin A. In summary, these findings contributed to the knowledge on AM protein profiles, potentially aiding future research regarding AM etiopathogenesis and leading to AM prevention and treatment.
PubMed: 38476605
DOI: 10.3892/br.2024.1752 -
Eplasty 2024Ameloblastoma is a rare odontogenic tumor most commonly located within the mandible. These tumors can grow to massive proportions and result in malocclusion. Segmental...
BACKGROUND
Ameloblastoma is a rare odontogenic tumor most commonly located within the mandible. These tumors can grow to massive proportions and result in malocclusion. Segmental mandibulectomy and reconstruction with an osteocutaneous free flap are frequently required. Virtual surgical planning (VSP) aids the surgeon in creating precise anatomic reconstruction when there is preoperative malocclusion due to tumor size. In this study we seek to further examine reconstruction of posterior mandibulectomy defects inclusive of condylar resection.
METHODS
Retrospective review of patients treated for giant ameloblastoma (tumor >4 cm) was examined; 3 patients with posterior tumors requiring ramus and condylar resection were included. Reconstruction in all patients was performed using fibula free flaps and VSP custom-made mandibular reconstruction plates. In these patients the reconstructed ramus was shortened and precise contouring done with a burr to recreate the native condylar surface. Intermaxillary fixation was used to maintain occlusion for 1 month postoperatively. Inferior alveolar nerve repair with allograft and nerve connectors was performed for all 3 patients.
RESULTS
All patients underwent successful mandibular reconstruction with preservation of mandibular function and improved occlusion postoperatively. Inferior alveolar nerve repair using nerve allograft allowed for neurosensory recovery in the mandibular division of trigeminal nerve distribution in 2 of the 3 patients.
CONCLUSIONS
Giant ameloblastoma involving the mandibular condyle can be successfully treated with the fibula free flap utilizing mandible reconstruction plates and VSP. This technique allows for excellent restoration of occlusion and neurosensory recovery when paired with reconstruction of the inferior alveolar nerve at time of reconstruction.
PubMed: 38476514
DOI: No ID Found -
Turkish Archives of Pediatrics Mar 2024Although Gorlin syndrome has rich skin findings, there is limited information about their subtypes, features specific to this genodermatosis, and relationships with each...
OBJECTIVE
Although Gorlin syndrome has rich skin findings, there is limited information about their subtypes, features specific to this genodermatosis, and relationships with each other.
MATERIALS AND METHODS
The demographic characteristics as well as cutaneous and extracutaneous findings of consecutive Gorlin syndrome patients diagnosed during 23 years were evaluated retrospectively. The relationship between palmoplantar pitting and basal cell carcinoma (BCC) in this localization and the relationship between odontogenic keratocysts (OKCs) and epidermoid cysts were investigated.
RESULTS
A total of 30 patients were diagnosed with Gorlin syndrome of whom 36.7% were children. BCC was the most common finding (90%) followed by OKCs (83.3%), skeletal system anomalies (76.7%), and palmoplantar pitting (76.7%). While classical BCC (63.3%) lesions were the predominant clinical subtype among all patients, acrochordon-like or small-sized papular BCCs were seen in 45.4% of pediatric patients. Three patients, 2 of whom were children, had BCC lesions in the palmoplantar region in association with palmoplantar pitting. Epidermoid cysts presenting clinically as solitary (n = 12) or a few nodules (n = 4) without punctum, located more commonly in acral areas (n = 10) were seen in 16 (53.3%) patients of whom 7 were children. Epidermoid cysts were seen in 60% of patients with OKCs, and the relationship between epidermoid cysts and OKCs was not statistically significant (P = .15). Extracutaneous tumors such as medulloblastoma (n = 3), cardiac fibroma (n = 1), and ameloblastoma (n = 1) were also recorded.
CONCLUSION
The awareness of papular or acrochordon-like BCCs, palmoplantar BCCs, and acral epidermoid cysts without punctum may facilitate early diagnosis of Gorlin syndrome in children.
PubMed: 38454226
DOI: 10.5152/TurkArchPediatr.2024.23231 -
International Journal of Oral Science Feb 2024Ameloblastoma is a benign tumor characterized by locally invasive phenotypes, leading to facial bone destruction and a high recurrence rate. However, the mechanisms...
Ameloblastoma is a benign tumor characterized by locally invasive phenotypes, leading to facial bone destruction and a high recurrence rate. However, the mechanisms governing tumor initiation and recurrence are poorly understood. Here, we uncovered cellular landscapes and mechanisms that underlie tumor recurrence in ameloblastoma at single-cell resolution. Our results revealed that ameloblastoma exhibits five tumor subpopulations varying with respect to immune response (IR), bone remodeling (BR), tooth development (TD), epithelial development (ED), and cell cycle (CC) signatures. Of note, we found that CC ameloblastoma cells were endowed with stemness and contributed to tumor recurrence, which was dominated by the EZH2-mediated program. Targeting EZH2 effectively eliminated CC ameloblastoma cells and inhibited tumor growth in ameloblastoma patient-derived organoids. These data described the tumor subpopulation and clarified the identity, function, and regulatory mechanism of CC ameloblastoma cells, providing a potential therapeutic target for ameloblastoma.
Topics: Humans; Ameloblastoma; Neoplasm Recurrence, Local; Phenotype; Cell Transformation, Neoplastic; Gene Expression Profiling
PubMed: 38424060
DOI: 10.1038/s41368-024-00281-4 -
Annals of Maxillofacial Surgery 2023Ameloblastic carcinoma is defined as an ameloblastoma in which there is histological evidence of malignancy in primary tumour or recurrent tumour regardless if it has...
RATIONALE
Ameloblastic carcinoma is defined as an ameloblastoma in which there is histological evidence of malignancy in primary tumour or recurrent tumour regardless if it has metastasised or not. It is aggressive in nature.
PATIENT CONCERNS
The patient presented with a painful swelling associated with restricted mouth opening.
DIAGNOSIS
This is the clinical profile of a patient who has presented with a recurrent ameloblastic carcinoma.
TREATMENT
Histological features of the excised specimen resemble ameloblastic carcinoma constituting cytological atypia with tall columnar cells.
OUTCOME
Excision of tumour was done. Reconstruction was done using pectoralis major myocutaneous flap.
TAKE-AWAY LESSONS
Ameloblastic carcinoma is an aggressive tumour and constitutes destruction and distant metastatic spread. Hence, aggressive resection is the choice of treatment along with a long-term follow-up for better quality of life.
PubMed: 38405565
DOI: 10.4103/ams.ams_66_23 -
International Journal of Molecular... Feb 2024Ameloblastoma is a rare tumor but represents the most common odontogenic neoplasm. It is localized in the jaws and, although it is a benign, slow-growing tumor, it has...
Ameloblastoma is a rare tumor but represents the most common odontogenic neoplasm. It is localized in the jaws and, although it is a benign, slow-growing tumor, it has an aggressive local behavior and high recurrence rate. Therefore, alternative treatment options or complementary to surgery have been evaluated, with the most promising one among them being a targeted therapy with the murine sarcoma viral oncogene homologue B (), as in ameloblastoma the activating mutation V600E in BRAF is common. Studies in other tumors have shown that the synchronous inhibition of BRAF and human murine double minute 2 homologue (MDM2 or HDM2) protein is more effective than BRAF monotherapy, particularly in the presence of wild type p53 (WTp53). To investigate the MDM2 protein expression and gene amplification in ameloblastoma, in association with BRAF and p53 expression. Forty-four cases of ameloblastoma fixed in 10% buffered formalin and embedded in paraffin were examined for MDM2 overexpression and BRAF and p53 expression by immunohistochemistry, and for ploidy with fluorescence in situ hybridization. Sixteen of forty-four (36.36%) cases of ameloblastoma showed MDM2 overexpression. Seven of sixteen MDM2-positive ameloblastomas (43.75%) were BRAF positive and fifteen of sixteen MDM2-positive ameloblastomas (93.75%) were p53 negative. All MDM2 overexpressing tumors did not show copy number alterations for MDM2. Overexpression of MDM2 in ameloblastomas is not associated with MDM2 amplification, but most probably with MAPK activation and WTp53 expression. Further verification of those findings could form the basis for the use of MDM2 expression as a marker of MAPK activation in ameloblastomas and the trial of dual BRAF/MDM2 inhibition in the management of MDM2-overexpressing/BRAFV600E-positive/WTp53 ameloblastomas.
Topics: Animals; Humans; Mice; Ameloblastoma; In Situ Hybridization, Fluorescence; Mutation; Proto-Oncogene Proteins B-raf; Proto-Oncogene Proteins c-mdm2; Tumor Suppressor Protein p53
PubMed: 38396916
DOI: 10.3390/ijms25042238