Adenoid cystic carcinoma of the trachea: an uncommon presentation of a rare malignancy

Abstract Adenoid cystic carcinoma (ACC) is a rare salivary gland tumor characterized by an indolent yet aggressive course, facilitated by perineural invasion, a propensity for delayed distant metastases, and recurrence after resection. A 61-year-old female presented with a three-month history of intermittent hoarseness and resting dysphagia. Contrast-enhanced computed tomography demonstrated a subglottic mass with proximal tracheal invasion, and ultrasound-guided neck fine-needle aspiration cytology was consistent with ACC. Surgical management included total laryngectomy with proximal tracheal and left thyroid lobe resection, followed by immediate tracheostomy. ACC was confirmed on histopathology with negative margins, and adjuvant radiotherapy (RT) was initiated to reduce locoregional recurrence risk. At one-year follow-up, the patient remains in remission with no evidence of recurrence or metastasis. Early symptom recognition and surgery with adjuvant radiotherapy remain central to local disease control, and long-term surveillance is essential given the potential for late recurrence.