Insights into adenoid cystic carcinoma of the lower airway (ACCLA): a 10-year experience

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Background: ACCLA is a rare salivary gland tumor arising from the submucosal glands of the trachea and bronchus. Objectives: To characterize the clinical features, imaging findings, treatment approaches, disease progression, and survival outcomes of ACCLA. Methods: A retrospective review of ACCLA cases treated at a tertiary center between 2014-2024. Results: Six patients were identified, mostly female (83%) and non-smokers (83%), with a mean age at diagnosis of 50.5±19 years. Symptoms persisted for a mean of 354 days before diagnosis, most commonly cough (n=5) and dyspnea (n=2). The diagnosis was made using flexible (n=4) or rigid (n=2) bronchoscopy. PD-L1 expression and molecular profiling, assessed in 3 patients, were negative. Tumors were located in the trachea (n=2), main bronchus (n=2), or both (n=2) and staged as IB (33%) or IIIA (66%). Three patients underwent thoracic surgery, with stage IIIA (n=1) and stage IB (n=2). Two received adjuvant radiotherapy (RT) due to R1 (n=1) and cN1 involvement (n=1). Other treatments included RT alone (n=1) or RT combined with tracheal prosthesis after debulking (n=2). Median follow-up was 54.3 months. Disease recurrence occurred in all nonsurgical patients (mean 31.5 months), with pulmonary (n=2) or tracheal metastases (n=1). Two received best supportive care. One patient, initially treated with endoscopic intervention and RT, underwent right lower lobe wedge resection. After failure of chemotherapy for new nodules, nivolumab was initiated but discontinued due to progression with renal metastases. Two patients (33%) died during follow-up. Conclusions: Delayed diagnosis, limited treatments, and recurrence highlight the need for better ACCLA management.

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