Adenoid cystic carcinoma (ACC) is a rare malignant neoplasm that primarily arises from the salivary glands. It accounts for approximately 10% of all salivary gland tumors and is the most common malignancy affecting the minor salivary glands. Nearly 50% of cases occur in intraoral sites, with a marked predilection for the hard palate. The parotid gland is the second most common location, followed by the submandibular gland. ACC typically affects individuals between the fourth and sixth decades of life, with a slight female predilection. Clinically, it is characterized by an indolent but aggressive progression, combining slow tumor growth with a high risk of local recurrence, distant metastasis, and perineural invasion, all contributing to a poor prognosis. Standard management involves wide surgical excision followed by adjuvant radiotherapy. Prognosis depends on factors such as histological grade, tumor location, size, and early diagnosis. This report aims to present a case of palatal ACC and an updated review of the literature on this rare pathology.
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