Abstract
Adenoid cystic carcinoma (ACC) of the submandibular gland is a rare and highly aggressive malignancy, distinguished by its tendency for perineural invasion and distant metastasis, particularly to the lungs and bones. The management of ACC is challenging due to its biological variability and the absence of specific randomized controlled trials to guide treatment. This report aims to encapsulate the clinical features, histological profile, diagnostic workup, and management options for submandibular gland ACC, with an emphasis on recent advancements in understanding and treatment. We present the case of a 42-year-old woman who presented with a progressively enlarging mass in the left submandibular region, subsequently diagnosed as ACC post-surgery. The patient received adjuvant radiotherapy following a multidisciplinary team decision, highlighting the importance of a tailored, multi-modal approach in the management of submandibular gland ACC. Ongoing research and collaborative efforts are essential for developing effective, personalized treatment strategies for this rare malignancy.