Department of Otolaryngology & Head and Neck Surgery, Chang Gung Memorial Hospital at Linkou, Taoyuan, Taiwan; College of Medicine, Chang Gung University, Taoyuan, Taiwan.
Division of Head and Neck, Plastic and Reconstructive Surgery, Department of Surgery, Queen Mary Hospital, Hong Kong, China.
Biomed J. 2020 Apr;43(2):189-194. doi: 10.1016/j.bj.2019.07.005. Epub 2020 May 7.
This study reviewed the clinical manifestations, pathological findings, and treatment outcomes of adenoid cystic carcinoma (ACC) in the external auditory canal (EAC).
This was a retrospective review of 12 patients with a diagnosis of ACC in the EAC seen in a single institution over a 30-year period. Data on the demographics, clinical presentation, treatment strategy, and outcome, as well as the pathological features of ACC, were reviewed and analyzed.
The male-to-female ratio was 1:3 and the mean patient age was 55.9 years. The most common clinical presentation was otalgia (75%). Ten patients underwent surgical interventions, including radical mastoidectomy in five patients, wide excision in three, and lateral temporal bone resection in two. Adjuvant radiotherapy or concurrent chemoradiotherapy (CCRT) was performed in case of incomplete resection. Two patients underwent non-surgical treatments: radiotherapy in one and CCRT in the other. Microscopic perineural invasion was not associated with otalgia or histological subtype. The mean follow-up period was 84.6 months. Local recurrence occurred in 33% of patients. One-quarter of patients had distant metastasis, and all had lung metastasis. The 5-year overall survival rate for these patients was 82.5%.
EAC ACC should be included in the differential diagnosis when a patient presents with otalgia and a mass in EAC for more than 6 months, particularly if the patient is a middle-aged female. Otalgia might not be associated with perineural invasion or histological subtype. The lung is the most common site of distant metastasis in patients with EAC ACC. Further studies should determine the optimal treatment protocol for this rare malignancy.
本研究回顾了外耳道腺样囊性癌(ACC)的临床表现、病理发现和治疗结果。
这是对单一机构 30 年来诊断为外耳道 ACC 的 12 例患者的回顾性研究。回顾和分析了人口统计学、临床表现、治疗策略和结果以及 ACC 的病理特征。
男女比例为 1:3,平均患者年龄为 55.9 岁。最常见的临床表现是耳痛(75%)。10 例患者接受了手术干预,包括 5 例根治性乳突切除术、3 例广泛切除术和 2 例外侧颞骨切除术。对于不完全切除的患者,进行辅助放疗或同步放化疗(CCRT)。2 例患者接受了非手术治疗:1 例放疗和 1 例 CCRT。显微镜下神经周围侵犯与耳痛或组织学亚型无关。平均随访时间为 84.6 个月。33%的患者发生局部复发。1/4的患者发生远处转移,且均为肺转移。这些患者的 5 年总生存率为 82.5%。
当患者出现外耳道疼痛和外耳道肿块超过 6 个月时,应将 EAC ACC 纳入鉴别诊断,特别是如果患者为中年女性。耳痛可能与神经周围侵犯或组织学亚型无关。肺是 EAC ACC 患者远处转移的最常见部位。进一步的研究应确定这种罕见恶性肿瘤的最佳治疗方案。