Adenoid cystic carcinoma of the larynx: a report of six cases with review of the literature

Abstract

Conclusion: Adenoid cystic carcinoma (ACC) in the larynx is an extremely rare entity. Although the 5-year disease-specific survival rates for laryngeal ACC are high, distant metastasis might occur up to more than 5 years post treatment. Therefore patients with ACC require long-term follow-up. Objective: To summarize the characteristics of laryngeal ACC by analyzing six cases in a single center. Methods: This was a retrospective analysis of six patients with laryngeal ACC who were treated in Beijing Tongren Hospital between 1998 and 2013. Results: The mean age at diagnosis was 44 years, ranging from 15 to 61 years. Dyspnea was the most common complaint. All patients underwent combined-modality treatment with surgical resection and external beam radiation. The range of follow-up time varied from 1 to 7 years (median 5 years). Follow-up inspections indicated that none of them had local or regional recurrence. Distant metastasis occurred in 33.7% (2/6) of the patients, with one lung metastasis 5 years after operation and one liver metastasis 4 years after operation.

Introduction

Salivary gland carcinomas of the larynx are rare, and account for < 1% of laryngeal malignancies [1]. Adenoid cystic carcinomas (ACCs) are the most common malignant tumors that occur in minor salivary glands. It was Billroth in 1856 who coined the term ‘cylindroma,’ which became a widely accepted term for this tumor [2]. Laryngeal ACC is extremely rare because of the paucity of accessory salivary glands. This kind of tumor is characterized by multiple recurrences and late distant metastasis. Because of frequent early perineural and hematologic spread, local recurrences, and systemic metastases (especially to the lung), which sometimes arise years after diagnosis and treatment, patients with laryngeal ACC require long-term follow-up [3].

This study aimed to review our long-term experience of treating ACC of the larynx at Beijing Tongren Hospital.