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BMC Oral Health Mar 2021Malignant odontogenic tumours are extremely rare tumours occurring within the jaws. Our study was performed to determine the demographic and clinico-pathological...
BACKGROUND
Malignant odontogenic tumours are extremely rare tumours occurring within the jaws. Our study was performed to determine the demographic and clinico-pathological features of malignant odontogenic tumours amongst a multi-ethnic Asian population.
METHODS
This was a retrospective cross-sectional study of malignant odontogenic tumours diagnosed at the Institute for Medical Research, Malaysia, from 2009 to 2019. All cases were independently reviewed and reclassified following the criteria set out in the latest edition of the World Health Organization 2017 reference text. Demographic and clinico-pathological data were recorded for each case.
RESULTS
Twenty-four cases of malignant odontogenic tumours were identified. The patients' age ranged from 16 to 79 years with the mean age at diagnosis being 50.8 years (SD = 16.18). There was a male predominance (66.7%) in this cohort of patients. The ethnic distribution appeared to reflect the Malaysian population with most cases seen amongst the Malay ethnic group (66.7%). Ameloblastic carcinoma was the most frequently diagnosed malignant odontogenic tumour (45.8%) and was also predominantly seen in males (90.9%). All patients with clear cell odontogenic carcinoma were females. There was no obvious sex predilection in primary odontogenic carcinoma not otherwise specified (NOS). The mandible (79.2%) was more frequently involved compared to the maxilla.
CONCLUSIONS
Diagnosis and management of malignant odontogenic tumours are challenging due to the rarity of these tumours. Our study has elucidated the clinico-pathological features of malignant odontogenic tumours seen in a multi-ethnic Asian population.
Topics: Adolescent; Adult; Aged; Cross-Sectional Studies; Female; Humans; Malaysia; Male; Middle Aged; Odontogenic Tumors; Referral and Consultation; Retrospective Studies; Young Adult
PubMed: 33736630
DOI: 10.1186/s12903-020-01365-3 -
Ear, Nose, & Throat Journal May 2023Ameloblastic carcinoma (AC) is a rare and aggressive malignant epithelial odontogenic tumor making up less than 1% of malignant head and neck tumors. The majority of...
Ameloblastic carcinoma (AC) is a rare and aggressive malignant epithelial odontogenic tumor making up less than 1% of malignant head and neck tumors. The majority of cases occur in the mandible with a minority occurring in the maxilla. Most occur de novo, while rare cases of AC have resulted from transformation from ameloblastoma. Here, we present a case in which a 30-year-old man presented with proptosis and a recurrent right temporal mass, which had been previously diagnosed as ameloblastoma on surgical pathology. CT findings demonstrated local invasion, and he was subsequently taken to the operating room for right craniotomy, infratemporal and middle cranial fossa tumor resection, and right modified radical neck dissection with reconstruction. Final pathology, which included areas of early focal necrosis, loss of peripheral palisading, and hyperchromatism, confirmed the diagnosis of ameloblastoma with transformation to AC. We further discuss radiologic and histopathological signs of this rare tumor, as well as recommended treatment modalities.
PubMed: 37158333
DOI: 10.1177/01455613231172857 -
Head and Neck Pathology Sep 2023Odontogenic carcinosarcoma (OCS) is a rare odontogenic malignancy with limited characterization and unexplored molecular features. We report clinicopathologic and... (Review)
Review
BACKGROUND
Odontogenic carcinosarcoma (OCS) is a rare odontogenic malignancy with limited characterization and unexplored molecular features. We report clinicopathologic and molecular findings in 3 additional OCS and review the literature.
METHODS
3 OCS (5.1%) were identified among 59 malignant odontogenic tumors (in our archives from 1992 to 2022). Clinical, radiologic, histopathologic, immunophenotypic, and molecular findings were reviewed. Data from prior case reports and systematic or non-systematic reviews were extracted for analysis.
RESULTS
Three mandibular OCS (age range: 66 to 72 years; 1 male, 2 females) were identified. Case 1 had novel clear-cell morphology, multiple recurrences, and a lethal outcome 28 months after resection. EWSR1 rearrangements were negative, but the tumor showed focal nuclear β-catenin and strong LEF-1 immunoreactivity. Case 2 demonstrated ameloblastic and sclerosing features and encased the inferior alveolar nerve; the patient was disease-free 22 months after resection with adjuvant chemoradiation therapy. LEF-1 was again strongly positive, and next-generation sequencing demonstrated 9p region-(CDKN2A, CDKN2B) copy number loss, and 12q region-(MDM2, CDK4) copy number gain. Case 3 showed clear-cell and markedly sclerosing features; no follow-up information was available. Literature review along with the current cases yielded 20 cases. OCS showed a male predilection (1.5:1), mandibular predominance (80%, typically posterior), and a bimodal age distribution (modes: 27.7 years, 62.7 years). OCS presented as masses (100%), often with pain (55%), and paresthesia (45%). Tumors were typically radiolucent (88.9%), with bone destruction (61.1%), and/or tooth effacement (27.8%). Preoperative biopsy was sensitive for malignancy (85.7%). At least 45% show evidence for a precursor lesion. 3-year DSS and DFS were 58% and 35%, respectively. Regional and distant (usually lung) metastatic rates were 25% and 31.3%, respectively. Increased mitotic rates and presence of tumor necrosis trended toward worse DSS and DFS.
CONCLUSION
OCS is a rare but aggressive malignancy, often arising from precursor tumors and may represent a terminal phenotype rather than a distinct entity. We describe novel clear-cell and sclerosing morphologies. Wnt pathway alterations appear important. Mitotic rates and necrosis may be adverse prognosticators. In keeping with nomenclature trends in other sites, OCS may be more appropriately designated as "biphasic sarcomatoid odontogenic carcinomas."
Topics: Female; Humans; Male; Aged; Adult; Odontogenic Tumors; Mouth Neoplasms; Carcinosarcoma; Carcinoma; Biopsy
PubMed: 37486533
DOI: 10.1007/s12105-023-01569-3 -
Neurosurgery May 2021Ameloblastic carcinoma (AC) is a malignant neoplasm of epithelial origin that typically arises from the mandible or maxilla. It represents approximately 2% of all...
BACKGROUND AND IMPORTANCE
Ameloblastic carcinoma (AC) is a malignant neoplasm of epithelial origin that typically arises from the mandible or maxilla. It represents approximately 2% of all odontogenic tumors. Gross total resection is the surgical goal given AC's aggressiveness and propensity for recurrence. We present the first reported AC metastasis to the cervical spine.
CLINICAL PRESENTATION
A 61-yr-old African American female with a history of AC of bilateral mandibles and lung metastases presented with neck pain and right arm weakness progressive over several months. Cervical spine imaging demonstrated a cervical 3 pathological fracture with severe anterior vertebral body compression and resultant cervical 2-3 kyphotic deformity and bony retropulsion causing severe cord compression. The patient underwent a cervical 3 corpectomy and cervical 2-4 anterior fixation followed by a cervical 3 laminectomy and cervical 2-5 dorsal internal fixation and fusion. Postoperatively, the patient's neurological exam remained stable and imaging showed improved spinal alignment and appropriate anterior and posterior instrumentation. Unfortunately, the patient thereafter suffered a decline in performance status and progression of lung metastatic disease. Her oncology team is considering chemotherapy and stereotactic radiosurgery, but her prognosis remains grim.
CONCLUSION
AC is a rare and aggressive pathology with a poor prognosis despite multimodal therapy. We present the first case of AC metastatic spread to the spine. We aim to bring this pathology to the attention of our worldwide neurosurgical colleagues and share our surgical approach and multidisciplinary management to assist those who may encounter this pathology in the future.
Topics: Cervical Vertebrae; Female; Fracture Fixation, Internal; Humans; Laminectomy; Middle Aged; Odontogenic Tumors; Spinal Cord Compression; Spinal Fusion; Spinal Neoplasms
PubMed: 33611519
DOI: 10.1093/neuros/nyab044 -
International Journal of Surgical... Sep 2023Ameloblastic carcinoma (AC) is an uncommon malignant odontogenic tumor, with about 200 cases published in the literature. Here, we report a case of AC occurring in the...
Ameloblastic carcinoma (AC) is an uncommon malignant odontogenic tumor, with about 200 cases published in the literature. Here, we report a case of AC occurring in the maxilla - at the same anatomical location of an ameloblastoma 8 years earlier. A 23-year-old woman with maxillary swelling of 6-month duration was referred to our service. Extraoral examination revealed a swelling over the right side of the face, lifting her lip and nose. Intraorally, a firm, reddish, nodular swelling involving the right maxilla was observed. Computed tomography showed a hypodense image invading the right maxilla, maxillary sinus, and nasal fossa. An incisional biopsy was performed, followed by histopathologic examination and an immunohistochemical panel, which led to the diagnosis of AC. The lesion was treated with partial maxillectomy followed by immediate rehabilitation. Neither local recurrence of the tumor nor distant metastasis was observed during a 4-year follow-up. AC is a highly malignant lesion which requires aggressive therapy. Its diagnosis should be based on combined clinical, imaging, and pathological manifestations in order to improve diagnostic accuracy.
Topics: Female; Humans; Odontogenic Tumors; Ameloblastoma; Maxilla; Tomography, X-Ray Computed; Carcinoma
PubMed: 36031847
DOI: 10.1177/10668969221118323 -
Head and Neck Pathology Jun 2020Claudins are integral to the structure and function of tight junctions. Altered claudin expression has been shown to affect disease behavior and patient prognosis in...
Claudins are integral to the structure and function of tight junctions. Altered claudin expression has been shown to affect disease behavior and patient prognosis in various neoplasms. The objectives of this study were to analyze the claudin-1, -4 and -7 expression in odontogenic tumors and characterize their expression pattern in distinct tumor cell types in relation to the recurrence potential. Sixty-nine cases of odontogenic tumors, including 43 ameloblastomas (AM), 17 adenomatoid odontogenic tumors (AOT), 6 ameloblastic fibromas (AF) and 3 ameloblastic carcinomas (AC) were investigated for claudin-1, -4 and -7 expression immunohistochemically. The staining was analyzed semi-quantitatively and categorized into 4 levels, based on the percentage of positively stained neoplastic epithelial cells. Claudin-1 was expressed in all AOT and AF cases, whereas most AC (66.7%) showed no expression. The claudin-1 staining was moderate-to-intense in the odontogenic epithelium of AF. In contrast, its staining of ameloblast-like cells and stellate reticulum-like cells in AM was weak. Claudin-7 expression was noted in all tumor types studied, while the expression of claudin-4 was limited and mainly localized in the squamous differentiated cells of AM and AC. AM showed significantly higher claudin-4, but lower claudin-7 expression than AOT. In addition, AC showed diminished claudin-1 immunoreactivity, compared to AOT. Low claudin-1 expression in AM was significantly associated with the increased clinical recurrence. The loss of claudin-1 may underlie the locally invasive nature of AM.
Topics: Adolescent; Adult; Aged; Aged, 80 and over; Biomarkers, Tumor; Child; Claudins; Female; Humans; Male; Middle Aged; Odontogenic Tumors; Young Adult
PubMed: 31473941
DOI: 10.1007/s12105-019-01072-8 -
Genes Aug 2023Stem cells have been associated with self-renewing and plasticity and have been investigated in various odontogenic lesions in association with their pathogenesis and... (Review)
Review
BACKGROUND
Stem cells have been associated with self-renewing and plasticity and have been investigated in various odontogenic lesions in association with their pathogenesis and biological behavior. We aim to provide a systematic review of stem cell markers' expression in odontogenic tumors and cysts.
METHODS
The literature was searched through the MEDLINE/PubMed, EMBASE via OVID, Web of Science, and CINHAL via EBSCO databases for original studies evaluating stem cell markers' expression in different odontogenic tumors/cysts, or an odontogenic disease group and a control group. The studies' risk of bias (RoB) was assessed via a Joanna Briggs Institute Critical Appraisal Tool. Meta-analysis was conducted for markers evaluated in the same pair of odontogenic tumors/cysts in at least two studies.
RESULTS
29 studies reported the expression of stem cell markers, e.g., SOX2, OCT4, NANOG, CD44, ALDH1, BMI1, and CD105, in various odontogenic lesions, through immunohistochemistry/immunofluorescence, polymerase chain reaction, flow cytometry, microarrays, and RNA-sequencing. Low, moderate, and high RoBs were observed in seven, nine, and thirteen studies, respectively. Meta-analysis revealed a remarkable discriminative ability of SOX2 for ameloblastic carcinomas or odontogenic keratocysts over ameloblastomas.
CONCLUSION
Stem cells might be linked to the pathogenesis and clinical behavior of odontogenic pathologies and represent a potential target for future individualized therapies.
PubMed: 37761874
DOI: 10.3390/genes14091735 -
JPRAS Open Sep 2019Ameloblastic fibrosarcoma (AFS) is a rare malignant odontogenic tumour classified by the WHO (1992) as an odontogenic sarcoma and defined as "a neoplasm with a similar...
Ameloblastic fibrosarcoma (AFS) is a rare malignant odontogenic tumour classified by the WHO (1992) as an odontogenic sarcoma and defined as "a neoplasm with a similar structure to ameloblastic fibroma (AF) but in which the ectomesenchymal component shows the features of a sarcoma." The first report of AFS was published by Heath in 1887. AFS and related lesions are less frequently diagnosed than odontogenic carcinomas. Approximately two-thirds of AFSs seem to arise de novo, but others have developed in recurrent AF, in which the ectomesenchymal cells retain their embryonic appearance and develop malignant characteristics. We report a rare case of an aggressive odontogenic neoplasm, the incisional biopsy of which showed the features of AF, while the excisional biopsy revealed the features of malignancy, suggestive of AFS. The purpose of this report is to discuss the diagnostic difficulties, whether AFS is truly an extremely rare tumour as reported earlier and, lastly, should the treatment protocols of AFs be revised, as 44% of AFSs arise from recurrent AFs.
PubMed: 32158887
DOI: 10.1016/j.jpra.2019.05.001 -
Journal of Pharmacy & Bioallied Sciences Apr 2024Ameloblastic carcinoma is a rare malignant odontogenic tumor that is further classified into being primary or secondary arising from a preexisting benign ameloblastoma....
Ameloblastic carcinoma is a rare malignant odontogenic tumor that is further classified into being primary or secondary arising from a preexisting benign ameloblastoma. It affects the mandible in two thirds of the patients. There is no standard treatment protocol for this lesion but radicalsurgical excision with or without radiotherapy is reported in the majority of cases. In this paper, we present a case of a 22 year old male diagnosed with Ameloblastic carcinoma of the mandible with a clinical course of typical aggressiveness and extensive destruction. Histopathological examination of the incised biopsy showed a parakeratinized stratified squamous epithelium with underlying fibrous connective tissue stroma. The stroma is highly myxomatous and exhibits islands of odontogenic epithelium and chronic inflammatory cell infiltrates. Interlacing strands of odontogenic epithelium shows stellate reticulum-like cells and occasional areas of squamous metaplasia with cellular and nuclear pleomorphism. In addition, mitotic figures were noted. With the correlation of clinical, radiographic, and histological features, the lesion is diagnosed as ameloblastic carcinoma. The lesion was surgical excised and post-treatment follow-up for 6 months revealed no recurrence of the malignancy.
PubMed: 38882877
DOI: 10.4103/jpbs.jpbs_1204_23 -
Journal of Comparative Pathology Aug 2022A 2-year-old male Thoroughbred horse presented with a mass in the maxilla. The focally ulcerated mass, approximately 8 cm in diameter, covered the upper left...
A 2-year-old male Thoroughbred horse presented with a mass in the maxilla. The focally ulcerated mass, approximately 8 cm in diameter, covered the upper left intermediate and corner incisor teeth (nos. 602 and 603 according to the modified Triadan system) and radiographic examination revealed displacement and lysis of the incisors. Histologically, the tumour was composed of a dense proliferation of spindle-shaped cells and neoplastic odontogenic epithelial cells arranged in island, follicular, plexiform or sheetlike patterns. The spindle-shaped cells were immunopositive for cytokeratins AE1/AE3, 5/6, 14 and 19. The Ki-67 index was 32.6% in the spindle cell component. Based on the histological and immunohistochemical findings, the tumour was diagnosed as spindle cell ameloblastic carcinoma.
Topics: Ameloblastoma; Animals; Carcinoma; Horse Diseases; Horses; Male
PubMed: 36008038
DOI: 10.1016/j.jcpa.2022.05.006