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Ophthalmic Plastic and Reconstructive... May 2024Ameloblastic carcinomas are malignant tumors arising from the odontogenic epithelium and defined as having features of ameloblastic differentiation in addition to...
Ameloblastic carcinomas are malignant tumors arising from the odontogenic epithelium and defined as having features of ameloblastic differentiation in addition to cytological features of malignancy. Orbital involvement is rare and generally involves invasion of the orbital floor, apex, or soft tissue. This report describes an advanced presentation of ameloblastic carcinoma with orbital invasion and provides a review of the literature. A 58-year-old male presented with a 2-year history of a mid-facial mass, causing intracranial invasion and distortion of most skull foramina, nasopharynx, nasal cavity, and both orbits. Notably, there was an en-plaque pattern of circumferential tracking of the tumor along both orbital walls without invasion beyond the extraconal space, causing compression of the orbital apex and proptosis. Histology demonstrated nests of ameloblastic carcinoma and the advanced tumor was deemed nonresectable, with treatment being palliative.
PubMed: 38722761
DOI: 10.1097/IOP.0000000000002706 -
Journal of Oral Pathology & Medicine :... Mar 2024Ameloblastic carcinoma (AC) is the most common odontogenic malignancy, constituting approximately 30% of cases in this category. Literature is sparse on malignant... (Review)
Review
BACKGROUND
Ameloblastic carcinoma (AC) is the most common odontogenic malignancy, constituting approximately 30% of cases in this category. Literature is sparse on malignant odontogenic neoplasms, with a large proportion of current knowledge derived from case reports or small case series.
METHODS
A systematic review of case series/case reports of AC was conducted following the Preferred Reporting Items for Systematic Reviews and Meta-analyses (PRISMA) Statement guidelines. Demographic and clinical information, including duration of the lesion, location, clinical presentation and radiologic features, were analysed. Additionally, the origin of the lesion (primary/secondary), Ki-67 proliferation index, treatment performed, metastasis, tumour recurrence and prognosis were collected for analysis.
RESULTS
A total of 126 studies, including 285 individual cases of AC, were included in this review. Patients presented with a near-equal distribution of painless and painful swellings. ACs presented at a median age of 45 years, with a male-to-female ratio of 1:2. The mandible was most frequently involved, with rare cases extending to involve more than one region, including crossing the midline. Although most lesions presented with poorly-demarcated borders (52.6%), unilocular lesions with well-demarcated borders (47.4%) comprised a substantial number in the sample. The proliferation index was only reported in 27 cases, with a mean score of 42% and a wide range. The probability of tumour recurrence increased, and the survival probability decreased with prolonged follow-up duration.
CONCLUSION
This study provides more comprehensive, up-to-date descriptive data on these rare odontogenic malignancies, aiding clinicians and Pathologists with the diagnosis and surgeons in their management of cases.
Topics: Humans; Male; Female; Middle Aged; Neoplasm Recurrence, Local; Odontogenic Tumors; Mandible; Prognosis; Carcinoma
PubMed: 38368851
DOI: 10.1111/jop.13517 -
Journal of Oral Pathology & Medicine :... Aug 2022To investigate and compare the demographic data, occurrence of recurrence and metastasis, and survival prognosis between ameloblastic carcinoma (AC) and metastasizing... (Review)
Review
PURPOSE
To investigate and compare the demographic data, occurrence of recurrence and metastasis, and survival prognosis between ameloblastic carcinoma (AC) and metastasizing ameloblastoma (MA), based on appropriate and currently accepted eligible diagnostic criteria, in a systematic review of the literature.
METHODS
An electronic search was undertaken, last updated in December 2021. Eligibility criteria included publications having enough clinicopathological information to confirm the diagnosis of these tumors.
RESULTS
Seventy-seven publications reporting 85 ACs and 43 MAs were included. Both tumors were more frequent in mandible and showed different clinical profiles regarding patients' sex and age. There was no difference in the estimated cumulative survival between patients diagnosed with these tumors. Metastases mainly affected the lungs, followed by cervical lymph nodes. The mean time between the first metastasis and the last follow-up was higher for MA (p = 0.021). In addition, MA patients remained alive longer than AC patients after the first metastasis diagnosis (p = 0.041). Considering only the cases that metastasized, a higher ratio of AC patients died in comparison to MA patients (p = 0.003). The occurrence of recurrence was associated with a conservative primary treatment with both AC (p < 0.001) and MA tumors (p = 0.017). Multiple recurrent events were associated with conservative primary therapies with MA (p < 0.001) but not with AC (p = 0.121).
CONCLUSION
In addition to some demographic differences, ACs that metastasize present a worse prognosis than MA. As conservative procedures are associated with multiple recurrent events, this treatment modality should be avoided for both tumors.
Topics: Ameloblastoma; Carcinoma; Humans; Mandible; Mandibular Neoplasms; Odontogenic Tumors
PubMed: 35822408
DOI: 10.1111/jop.13334 -
Journal of Oral Pathology & Medicine :... Apr 2023The advances in molecular technologies have allowed a better understanding of the molecular basis of odontogenic cysts and tumours. PTCH1 mutations have been reported in... (Review)
Review
The advances in molecular technologies have allowed a better understanding of the molecular basis of odontogenic cysts and tumours. PTCH1 mutations have been reported in a high proportion of odontogenic keratocyst. BRAF p.V600E are recurrent in ameloblastoma and KRAS p.G12V/R in adenomatoid odontogenic tumour, dysregulating the MAPK/ERK pathway. Notably, BRAF p.V600E is also detected in ameloblastic carcinoma, but at a lower frequency than in its benign counterpart ameloblastoma. Recently, adenoid ameloblastoma has been shown to be BRAF wild-type and to harbour CTNNB1 (β-catenin gene) mutations, further suggesting that it is not an ameloblastoma subtype. CTNNB1 mutations also occur in other ghost-cell-containing tumours, including calcifying odontogenic cysts, dentinogenic ghost cell tumours and odontogenic carcinoma with dentinoid, but the link between CTNNB1 mutations and ghost cell formation in these lesions remains unclear. Regarding mixed tumours, BRAF p.V600E has been reported in a subset of ameloblastic fibromas, ameloblastic-fibrodentinomas and fibro-odontomas, in addition to ameloblastic fibrosarcoma. Such mutation-positivity in a subset of samples can be helpful in differentiating some of these lesions from odontoma, which is BRAF-wild-type. Recently, FOS rearrangements have been reported in cementoblastoma, supporting its relationship with osteoblastoma. Collectively, the identification of recurrent mutations in these aforementioned lesions has helped to clarify their molecular basis and to better understand the interrelationships between some tumours, but none of these genetic abnormalities is diagnostic. Since the functional effect of pathogenic mutations is context and tissue-dependent, a clear role for the reported mutations in odontogenic cysts and tumours in their pathogenesis remains to be elucidated.
Topics: Humans; Ameloblastoma; Proto-Oncogene Proteins B-raf; Odontogenic Tumors; Odontogenic Cysts; Odontoma; Mouth Neoplasms; Carcinoma
PubMed: 36629457
DOI: 10.1111/jop.13401 -
Frontiers in Oral Health 2021DNA methyltransferases (DNMTs) and the histone modification H3K9ac are epigenetic markers. This study aimed to describe the immunohistochemical expression of DNMT1,...
DNA methyltransferases (DNMTs) and the histone modification H3K9ac are epigenetic markers. This study aimed to describe the immunohistochemical expression of DNMT1, DNMT3A, DNMT3B, and H3K9ac in the dental follicle (DF), ameloblastoma (AME), and ameloblastic carcinoma (AC), correlating these expressions with the recurrence and aggressive behavior in ameloblastoma. Immunohistochemical reactions were performed in 10 human DFs, 38 ameloblastomas, and 6 AC samples. Another 59 ameloblastomas assembled in a tissue microarray were used to compare the immunoexpression with the clinical, radiographic, and histopathological characteristics and the presence of BRAFv600e mutation. Each slide was digitized as a high-resolution image and quantified by Aperio ScanScope Nuclear V9 software. All statistical analyzes were performed using GraphPad Prism statistical software. DNMT3B expression was higher in ameloblastomas than in the DFs, while the AC overexpressed all proteins. The ameloblastomas with BRAFv600e mutation, vestibular/lingual, or vestibular/palatine bone cortical disruption and maxilla involvement showed DNMT1 overexpression, while recurrent cases had high DNMT3B levels. DNA methylation and histone modification might play a role in the development, clinical aggressiveness, and recurrence rates of ameloblastoma, such as the progression to AC. Further investigation about gene methylations in ameloblastomas is needed to better understand its relationship with aggressiveness and recurrence.
PubMed: 35048062
DOI: 10.3389/froh.2021.751162 -
Head and Neck Pathology Mar 2023Homeobox genes play crucial roles in tooth morphogenesis and development and thus mutations in homeobox genes cause developmental disorders such as odontogenic lesions.... (Review)
Review
BACKGROUND
Homeobox genes play crucial roles in tooth morphogenesis and development and thus mutations in homeobox genes cause developmental disorders such as odontogenic lesions. The aim of this scoping review is to identify and compile available data from the literatures on the topic of homeobox gene expression in odontogenic lesions.
METHOD
An electronic search to collate all the information on studies on homeobox gene expression in odontogenic lesions was carried out in four databases (PubMed, EBSCO host, Web of Science and Cochrane Library) with selected keywords. All papers which reported expression of homeobox genes in odontogenic lesions were considered.
RESULTS
A total of eleven (11) papers describing expression of homeobox genes in odontogenic lesions were identified. Methods of studies included next generation sequencing, microarray analysis, RT-PCR, Western blotting, in situ hybridization, and immunohistochemistry. The homeobox reported in odontogenic lesions includes LHX8 and DLX3 in odontoma; PITX2, MSX1, MSX2, DLX, DLX2, DLX3, DLX4, DLX5, DLX6, ISL1, OCT4 and HOX C in ameloblastoma; OCT4 in adenomatoid odontogenic tumour; PITX2 and MSX2 in primordial odontogenic tumour; PAX9 and BARX1 in odontogenic keratocyst; PITX2, ZEB1 and MEIS2 in ameloblastic carcinoma while there is absence of DLX2, DLX3 and MSX2 in clear cell odontogenic carcinoma.
CONCLUSIONS
This paper summarized and reviews the possible link between homeobox gene expression in odontogenic lesions. Based on the current available data, there are insufficient evidence to support any definite role of homeobox gene in odontogenic lesions.
Topics: Humans; Genes, Homeobox; Homeodomain Proteins; Transcription Factors; Ameloblastoma; Odontogenic Tumors; Carcinoma; Odontogenic Cysts
PubMed: 36344906
DOI: 10.1007/s12105-022-01481-2 -
Journal of Oral and Maxillofacial... Feb 2022Malignant odontogenic neoplasms are extremely challenging to study due to their rarity and variable clinical presentations. Ameloblastic carcinoma (AC) is one such...
Malignant odontogenic neoplasms are extremely challenging to study due to their rarity and variable clinical presentations. Ameloblastic carcinoma (AC) is one such odontogenic tumor which has been the subject of controversy, in part because of its scarcity, complicated by confusion in terminology along with complexity in classification. Histologic features of AC resemble tumor cells of ameloblastoma but exhibit cellular atypia. Surgical resection for this kind of lesion, leaving at least a 2 cm free margin coupled with neoadjuvant radiotherapy, might prove fruitful results. The current paper reports a case of an extraosseous variant of AC which posed a diagnostic challenge due to variable presentations histopathologically, suggesting the need for evidence-based case studies and molecular workup for a better therapeutic and prognostic insight.
PubMed: 35450254
DOI: 10.4103/jomfp.jomfp_378_21 -
Head & Neck Dec 2019Exploring the clinicopathological features of ameloblastic carcinoma (AC) and reviewing the literature to improve the diagnosis and treatment of the disease.
OBJECTIVES
Exploring the clinicopathological features of ameloblastic carcinoma (AC) and reviewing the literature to improve the diagnosis and treatment of the disease.
MATERIALS AND METHODS
Clinical data and pathological features of 18 cases of AC were retrospectively analyzed. A systematic review was carried out by searching PubMed and Medline databases using the MeSH terms "ameloblastic" and "carcinoma."
RESULTS
In the systematic analysis, 125 cases of AC from 81 eligible original studies and 18 cases of AC from this research were included. The male-to-female ratio was 2.58:1, and the mandible-to-maxilla ratio was 1.80:1. Mean age of patients was 45.3 years. Thirty-seven cases of recurrence and 27 cases of metastasis were recorded.
CONCLUSION
AC is a rare neoplasm of the odontogenic epithelium. A systematic review indicates that diagnoses at the early phase and a close periodic assessment for recurrence and metastasis are necessary.
Topics: Adult; Aged; Ameloblastoma; Carcinoma; Female; Humans; Male; Mandibular Neoplasms; Maxillary Neoplasms; Middle Aged; Neoplasm Metastasis; Neoplasm Recurrence, Local; Odontogenic Tumors; Retrospective Studies; Survival Rate
PubMed: 31444935
DOI: 10.1002/hed.25926 -
BMJ Case Reports Dec 2021Ameloblastic carcinoma is a rare malignant odontogenic neoplasm that exhibits diverse clinical and radiological presentations. In fact there are several differential...
Ameloblastic carcinoma is a rare malignant odontogenic neoplasm that exhibits diverse clinical and radiological presentations. In fact there are several differential diagnoses during histopathological evaluation too. Lack of adequate reports could not establish the predominant demographic, clinical and radiological presentations. For the same reasons, the role of adjuvant radiotherapy and chemotherapy is also unsubstantiated yet. This case discusses the innocuous clinical and radiological presentation of ameloblastic carcinoma in a 55-year-old man where the diagnostic confirmation was achieved through histopathological evaluation. The differential diagnoses, treatment and follow-up details of this case are discussed in light of the previous published case reports and systematic reviews of case reports in an attempt to increase the sensitisation among dentists towards ameloblastic carcinoma.
Topics: Ameloblastoma; Carcinoma; Diagnosis, Differential; Humans; Male; Mandibular Neoplasms; Middle Aged; Odontogenic Tumors
PubMed: 34906959
DOI: 10.1136/bcr-2021-246907