Sinonasal, Nasopharyngeal, Upper Oral, and Oropharyngeal Cavity Adenoid Cystic Carcinoma: An International Multicenter Retrospective Study on 457 Patients

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Introduction: The study has the following aims: (1) define the best treatment strategy for patients suffering from adenoid cystic carcinoma (ACC) of the sinonasal tract, nasopharynx, and upper oral and upper oropharyngeal cavity and (2) identify a new definition of recovery for ACC, given its unique behavior. Materials and Methods: Primary or secondary sinonasal, nasopharyngeal, and upper oral and oropharyngeal cavity ACCs treated at the hospitals of Padua, Brescia, Paris, Varese, and Houston were included. A pseudonymized database was created to collect patient-, treatment-, and follow-up-related data. A univariate survival analysis was performed in terms of overall survival (OS), disease-specific, progression-, and recurrence-free survival (DSS, PFS, RFS). Local treatment strategies were compared to each other and to the most relevant prognostic variables. Symptoms burden change after retreatment and cumulative incidence of adverse effects were calculated. Results: The study included 457 patients. The survival analysis demonstrated the prognostic validity of disease stage, grade, treatment intent, residue, response after treatment, and margin status. The multivariate analysis demonstrated that cT, cM, and Perzin/Szanto grade are independent prognostic factors. The analysis of symptoms burden change after retreatment demonstrated that, mostly, is higher. The cumulative incidence analysis showed that 40% of patients experienced at least one G3/G4 event at 120 months. Conclusion: The study demonstrated that: gross total resection without adjuvant radiotherapy provides a higher DSS; patients who undergo treatment with a noncurative intent are associated with a worst prognosis; burden of symptoms and toxicity caused by retreatments are considerable despite a limited prognostic gain; local and distant metastases can occur many years after the primary treatment. Publication History Article published online: 07 February 2025 © 2025. Thieme. All rights reserved. Georg Thieme Verlag KG Oswald-Hesse-Straße 50, 70469 Stuttgart, Germany

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