Abstract
Regional odontodysplasia (RO) is a rare dental anomaly of unknown etiology that can affect both deciduous and permanent dentition. We report a case of a 10-year-old female child with a chief complaint of painless swelling in the right maxilla, extending from the region of the canine to the tuberosity, covered by normal-colored mucosa, and approximately 2.5 cm in diameter. A panoramic radiograph showed malformation of the mineralized tissues of teeth of this region, with quite broad pulp chambers, suggesting the appearance of ghost teeth. An incisional biopsy of the soft tissue lesion was performed. Microscopic examination revealed the presence of small calcified enameloid conglomerates associated with spindle cells arranged in a storiform pattern, small islands of odontogenic epithelium, and hyalinized dentin-like material. The diagnosis was RO. The soft tissue lesion was surgically removed, and the patient was referred to a dentistry service for further functional rehabilitation of the affected teeth.
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