UNASSIGNED: Since 1964, there has been a scarcity of reported cases of primary ameloblastoma (AM) or ameloblastic carcinoma (AMCa) of the skull. The clinical presentation and distinctive features of this uncommon condition at specific anatomical sites remain unclear. We report a case of malignant transformation of a primary AM of the skull situated in the frontal-temporal-parietal region and highlight its similarities to other cases reported in the literature.
UNASSIGNED: A 53-year-old female patient presented with a 20-day history of headaches and bilateral lower limb weakness for 10 days. Physical examination revealed slow and unsteady gait. An occupying lesion was observed in the right frontal-temporal-parietal region of the skull on the Cranial imaging. A right cranial bone tumor margin expansion resection was performed. The patient\'s motor functions recovered normally after surgery. Postoperative imaging examinations showed10 tumor resection. Follow-up imaging examinations showed tumor recurrence. The patient underwent resection of the recurrent tumor. Postoperative pathological analysis revealed malignant transformation of the AM.Follow-up imaging examinations showed tumor recurrence again. The patient was admitted for stereotactic radiotherapy. Follow-up imaging examinations demonstrated no evidence of tumor recurrence and subsequent chest CT revealed no signs of metastasis.
UNASSIGNED: Primary AM or AMCa of the skull is increasingly being described in the literature, but detailed reports on the malignant transformation of primary AM of the skull are lacking. The pathogenesis of this condition remains unclear. Aggressive treatment and close follow-up may be crucial for preventing disease recurrence and malignant transformation.

Ameloblastic carcinoma is a rare malignant neoplasm with characteristic histopathological features that are directed towards an aggressive surgical approach than benign odontogenic lesions. It affects people of all ages, mostly in the posterior mandible, without a preference for race or gender. De novo cancer is one of its primary types, while the second type is defined as a malignant change from an antecedent case of benign ameloblastoma. The rapid progression of molecular biology led to the revelation that ameloblastoma contains a BRAF-V600E genetic mutation over 60%. Besides conventional ameloblastic carcinomas, rare histologic variants have also been described in the literature, including clear and spindle cells. These variants pose diagnostic challenges as to whether it is a dedifferentiation or a distinct entity. The dearth of data lends credence to the notion that these histologic variations are related to high-grade neoplasms and more aggressive outcomes. As a result, the current report intends to analyze a series of patients diagnosed with conventional ameloblastic carcinoma of the head and neck region with spindle and clear cell types along with a brief assessment of the literature.

UNASSIGNED: 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.
UNASSIGNED: The patient presented with a painful swelling associated with restricted mouth opening.
UNASSIGNED: This is the clinical profile of a patient who has presented with a recurrent ameloblastic carcinoma.
UNASSIGNED: Histological features of the excised specimen resemble ameloblastic carcinoma constituting cytological atypia with tall columnar cells.
UNASSIGNED: Excision of tumour was done. Reconstruction was done using pectoralis major myocutaneous flap.
UNASSIGNED: 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.

Ameloblastoma is a benign yet locally aggressive tumor of the jaw bones and is most commonly found in the lower mandibular region. Histologically, it shows benign characteristics. However, ameloblastoma can turn malignant to show a more aggressive clinical course. Carcinoma ex ameloblastoma is an extremely rare malignancy arising from a pre-existing long-standing ameloblastoma or a recurrence of an ameloblastoma. According to the literature search, six to seven cases have so far been documented, and the majority of the lesions had a propensity to metastasize. Here, we present a case of carcinoma ex ameloblastoma implicating a 19-year-old male patient manifesting in the mandible, which arises from pre-existing ameloblastoma.

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.
CONCLUSIONS: Stem cells might be linked to the pathogenesis and clinical behavior of odontogenic pathologies and represent a potential target for future individualized therapies.

Ameloblastic carcinoma is an odontogenic neoplasm with combined features of ameloblastoma and carcinoma on histopathological examination. Its prognosis is dominated by risk of local recurrence and distant metastasis. We report our patient because of the rare site and to highlight the importance of early, aggressive surgical treatment and regular follow-up.

Ameloblastic carcinoma (AC) is a rare, primary epithelial odontogenic malignant neoplasm. It is the malignant counterpart of ameloblastoma. It comprises 1% of all cysts and tumours occurring in the jaws, arising from tissues associated with odontogenic epithelium. The objective of the present study was to describe a clinical case of a 63-year-old male with an enlargement in the mandible on the left side. Panoramic radiography revealed a radiolucent area with poorly defined borders, and an incisional biopsy was performed for the histopathological study using immunomarkers such as SOX2 and Ki-67. Ki-67 is considered a marker of cell proliferation, and SOX2 reportedly participates in the development of the ameloblastic epithelium lineage and is associated with a more aggressive clinical course. A final histopathological diagnosis of AC was given. Unfortunately, the patient died one week before surgical resection (the surgical treatment of choice for AC).

Ameloblastic carcinoma is a locally aggressive odontogenic tumor that most commonly affects young and middle-aged adults. Metastatic disease may develop insidiously and manifest months or years after the initial diagnosis. Herein, we describe the clinical, imaging, and pathologic findings of a 31-year-old male who presented to the emergency department with headache and vision loss of 3 months duration and was subsequently found to have ameloblastic carcinoma with hepatic metastases. Initial computed tomography (CT) and magnetic resonance imaging revealed a multilocular cystic mass with avidly-enhancing nodular soft-tissue components associated with the right temporal fossa. Histologic examination of a tissue sample showed findings consistent with ameloblastic carcinoma. An initial staging CT scan showed several small hepatic cystic lesions. Follow-up surveillance imaging showed interval growth. A subsequent biopsy of a hepatic lesion showed findings compatible with metastatic ameloblastic carcinoma. The patient was started on systemic chemotherapy with evidence of disease progression at 1-year follow-up.

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.
METHODS: 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.

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.