Clear Cell Odontogenic Carcinoma of the Mandible Harboring EWSR1 Rearrangement: Report of a Massive Jaw Tumor and Review of Diagnostic Considerations.

Clear cell odontogenic carcinoma (CCOC) is a low-grade malignant neoplasm that affects the jaws. We report an 18 cm massive case of mandibular CCOC in a 43-year-old female. The tumor was composed of nests and cords of round to polygonal monomorphic clear cells separated by prominent stromal hyalinization. Immunohistochemically, the tumor cells showed focal cytokeratin 5/6 positivity and intracytoplasmic PAS-positive granules and were negative for S100 and after diastase treatment (PAS-D). Molecularly, this case was positive for EWSR1 rearrangement by FISH. The following should be included in the histopathological differential diagnosis: hyalinizing clear cell carcinoma of the salivary gland, clear cell variant of central mucoepidermoid carcinoma, clear cell variant of calcifying epithelial odontogenic tumor, and metastatic renal cell carcinoma. CCOC is a rare entity, with only 79 cases reported in the mandible. This case highlights the propensity for CCOC to exhibit invasiveness, destructive nature, and facial disfigurement if left untreated.

Ameloblastoma of the jaws in children: an evaluation of cases seen in a tertiary hospital in South-Eastern Nigeria.

BACKGROUND: Ameloblastoma is one of the most common benign odontogenic tumours in Nigeria. It is considered uncommon in children. MATERIALS AND METHODS: This is a retrospective study of pediatric patients with histopathological diagnosis of ameloblastoma seen over seven years at the Oral and Maxillofacial Surgery Department of the University of Nigeria Teaching Hospital, Enugu, Nigeria. Relevant information was retrieved from patients' records and data obtained were analysed using SPSS version 23, the chi-square test was used to compare qualitative variables, a p-value of <0.05 was considered as significant. RESULTS: One hundred and thirty-six cases of ameloblastoma in all age groups were seen within the period. Thirty of the cases met the requirement. The mean age of the patients was 14.4 STD 2.03 (range from 10-17) years. Fourteen (46.7%) patients were male while 16 (53.3%) were female giving a ratio of 1:1.1. The duration of the lesion ranged from 3 months to 72 months (mean 15.07 months). Histologically, the follicular type (n=20, 66.7%) constitute the majority, while the clinical types were solid-multicystic (n=18, 60%) and unicystic (n=12, 40%). Enucleation was the treatment of choice in most (n=18, (60%)) of the patients. CONCLUSION: Ameloblastoma is relatively uncommon in children, especially those less than ten years of age. The solid-multicystic variety was the predominant type in the children studied. Most patients presented long after the onset of the tumour and enucleation with mechanical curettage produced satisfactory results in these patients. FUNDING: None declared.