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Ameloblastoma, a benign yet aggressive odontogenic tumor known for its recurrence and the severe morbidity from radical surgeries, may benefit from advancements in targeted therapy. We present a case of a 15-year-old girl with ameloblastoma successfully treated with targeted therapy and review the literature with this question: Is anti-MAPK targeted therapy safe and effective for treating ameloblastoma? This systematic review was registered in PROSPERO, adhered to PRISMA guidelines, and searched multiple databases up to December 2023, identifying 13 relevant studies out of 647 records, covering 23 patients treated with MAPK inhibitor therapies. The results were promising as nearly all patients showed a positive treatment response, with four achieving complete radiological remission and others showing substantial reductions in primary, recurrent, and metastatic ameloblastoma sizes. Side effects were mostly mild to moderate. This study presents anti-MAPK therapy as a significant shift from invasive surgical treatments, potentially enhancing life quality and clinical outcomes by offering a less invasive yet effective treatment alternative. This approach could signify a breakthrough in managing this challenging tumor, emphasizing the need for further research into molecular-targeted therapies.

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.

Salivary gland neoplasms account for 3% of all head and neck tumours. Pleomorphic adenoma (PA) is the most common salivary gland tumour that mainly occurs in the parotid gland, followed by minor salivary glands of the oral cavity, however, the occurrence of PA inside the jaw bones is exceedingly rare and very few cases have been reported in the literature. Inside jaw bones these lesions tend to imitate large osteolytic lesions encompass a diagnostic challenge. An exhaustive review of the literature revealed only 10 cases of central pleomorphic adenoma. We present a rare case of primary PA that occurred inside the mandible and was provisionally diagnosed as ameloblastoma.

There are plenty of benign lesions that can result in swelling of the mandible, and these can be classified as odontogenic and non-odontogenic lesions. Among the categories of odontogenic lesion, ameloblastoma is the most occurring lesion that takes origin from the epithelial cellular elements and dental tissues in their different stages of development. Ameloblastoma is the most serious odontogenic neoplasm due to its prevalence and clinical characteristics. Ameloblastoma is a broad class which encompasses 80% of solid multicystic type of ameloblastoma with unicystic ameloblastoma (UA) variant included as vital clinicopathological form claiming the rest 20% along with peripheral ameloblastoma variant. UA refers to cystic lesions that seem like jaw cysts clinically, radiographically, or grossly but are lined by typical ameloblastomatous epithelium, with or without luminal and/or mural tumor development, on histologic investigation. Around 5-15% of all ameloblastic lesions do not have a propensity to metastasis, and this is UA. Unicystic mural form, although slow growing overall, is very invasive locally and has a high recurrence rate. As UA tumors show very close features with dentigerous cyst, a very sharp differential diagnosis protocol need to be executed to exclude the other unicystic odontogenic lesions considering the clinical, radiological, and biological characteristics along with proper follow-up and seeing any recurrence of the lesion taking place. Here, we report the case of a twenty-one year male patient with UA of the mandible and review of the literature.

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.

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.

The objective of this study was to review the clinical, radiologic, and histologic aspects of cases of dentigerous cysts metamorphosing into different entities along with comments on the molecular factors involved in the process. A series comprising 8 cases of dentigerous cysts was performed retrospectively along with a comprehensive literature review. Two cases of dentigerous cyst converting into mucous metaplasia were included, out of which one case was transformed into ameloblastoma after a year. The other two cases were reported as acanthomatous and unicystic ameloblastoma, whereas four transitioned to AOT. The complete review of cases and literature with molecular profiling concluded that the lining of dentigerous cysts has the potential for transforming into benign odontogenic tumors. Therefore, a careful clinical and histopathological examination is crucial for the correct diagnosis. Also, a thorough molecular understanding of the cystic lining is required.

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.

Keratoameloblastoma (KA) and solid variant of odontogenic keratocyst (SOKC) are rare odontogenic lesions, and their relationship and differences are unclear. The present study described a case that started as an odontogenic keratocyst (OKC) and transformed to SOKC/KA upon recurrence. Briefly, a 26‑year‑old man presented with swelling in the right cheek and was referred to the Department of Oral and Maxillofacial surgery, Hiroshima University Hospital (Hiroshima, Japan). At the initial visit, unicystic bone permeation was observed extending from the right canine to the molar, maxillary sinus and nasal cavity. After the biopsy, the patient underwent excisional surgery and was diagnosed with OKC. Thereafter, the lesion recurred six times over a period of 13 years and showed different histopathological features from those of the primary lesion, all consisting of numerous cysts with keratinization, which were diagnosed as SOKC/KA. The Ki‑67 positivity rate was ~10%, which was higher than that of the primary lesion, but there was no atypia. Genetic analysis of the recurrent lesion revealed mutations in adenomatous polyposis coli and Kirsten rat sarcoma viral oncogene homolog. This case originated from OKC, and the morphological features of OKC and KA were mixed upon recurrence, supporting the commonality and association between the two. However, multiple mutations different from those of OKC and ameloblastoma were detected, suggesting an association of SOKC/KA with increased proliferative activity and a high recurrence rate.