关键词: 喉肿瘤, 腺样囊性癌, 全喉切除术

Abstract: Objective To analyze the clinical characteristics of laryngeal adenoid cystic carcinoma. Methods We performed a retrospective study of 4 patients with laryngeal adenoid cystic carcinoma(LACC)treated in the Department of Otolaryngology, Head and Neck surgery of Peking University First Hospital from 2018-2021. The clinical manifestations, diagnosis, treatment, and prognosis were summarized and analyzed in combination with a literature review. Results The 4 patients comprised 3 women and 1 man. The most common symptoms were dyspnea(3 cases)and hoarseness(2 cases). The involved laryngeal subsites included the subglottis(2 cases)and the supraglottis(2 cases). Lung metastasis was suspected in 1 patient, while no distant metastases were observed in the rest. Total laryngectomy was performed in 2 patients, partial laryngectomy was performed in 1 patient, and palliative tumor resection combined with radiotherapy was performed in 1 patient. The range of follow-up time varied from 8-42 months(median 20.5 months). No local recurrence or distant metastasis were observed. Conclusion LACC is a rare malignancy with a female predilection. This neoplasm is characterized by slow growth kinetics, submucosal spread, and perineural invasion. The main symptoms are dyspnea and hoarseness, and the lesions most likely to occur in the subglottic and supraglottic areas. Diagnosis is based on pathology, for which the characteristic biomarker is CD117, while molecular genetic examination reveals MYB gene translocation. The mainstay treatment of LACC is radical surgery, of which total laryngectomy is the most commonly used surgical modality, which may be supplemented with postoperative radiotherapy. Surgical patients have a better prognosis than non-surgical patients. Distant metastases may occur late and require long-term follow-up.

Key words: Laryngeal carcinoma, Adenoid cystic carcinoma, Total laryngectomy