Service d'ORL et de chirurgie cervico-faciale, hôpital Hôtel-Dieu, CHU, 1, place A. Ricordeau, BP 1005, 44093 Nantes cedex 01, France.
Eur Ann Otorhinolaryngol Head Neck Dis. 2013 Nov;130(5):257-62. doi: 10.1016/j.anorl.2012.09.010. Epub 2013 Jun 6.
Adenoid cystic carcinomas (ACC) are rare malignant tumours arising in the major and minor salivary glands. Involvement of the nasal cavity and paranasal sinuses is rare and poorly described. The purpose of this study was to define the clinical and prognostic criteria of ACC of the paranasal sinuses based on the review of a series of 25 cases.
Single-centre retrospective study of 25 cases of ACC of the paranasal sinuses managed between 1998 and 2011, evaluating epidemiological, clinical, diagnostic and prognostic criteria. Factors influencing survival (Kaplan-Meier/Log Rank test) and the patient's quality of life (EORTC QLQ-C30 questionnaire) were also analysed.
Most patients (72%) had a locally-advanced tumour (stage T3 or T4) at diagnosis. Tumour sites, in decreasing order of frequency, were the maxillary sinus, nasal cavities and ethmoid sinus. The most common presenting complaints were maxillary pain or heaviness, unilateral blocked nose, and repeated epistaxis. When the tumour was resectable, treatment comprised a combination of surgery and adjuvant radiotherapy. The 5-year overall survival rate was 63% and the 5-year disease-free survival rate was 43%. The TNM stage at diagnosis (P=0.03), the histological subtype (P=0.023), the possibility of combined surgery and radiotherapy (P=0.03), and local control (P=0.05) were significant factors of improved 5-year overall survival. Positive surgical margins were associated with a trend towards poorer 5-year disease-free survival (ns).
ACC are rare malignant tumours associated with a poor prognosis, characterized by a high recurrence rate. Recommended treatment is a combination of surgery and adjuvant radiotherapy whenever possible. Five-year survival varies as a function of TNM stage, histological subtype, treatment options and local control.
腺样囊性癌（ACC）是一种罕见的恶性肿瘤，主要发生于大、小涎腺。鼻腔和副鼻窦受累少见且描述不佳。本研究的目的是通过回顾 25 例鼻窦腺样囊性癌的系列病例，确定鼻窦腺样囊性癌的临床和预后标准。
对 1998 年至 2011 年间治疗的 25 例鼻窦腺样囊性癌的单中心回顾性研究，评估了流行病学、临床、诊断和预后标准。还分析了影响生存的因素（Kaplan-Meier/Log Rank 检验）和患者的生活质量（EORTC QLQ-C30 问卷）。
大多数患者（72%）在诊断时为局部晚期肿瘤（T3 或 T4 期）。肿瘤部位依次为上颌窦、鼻腔和筛窦。最常见的表现症状为上颌疼痛或沉重感、单侧鼻塞和反复鼻出血。当肿瘤可切除时，治疗包括手术和辅助放疗相结合。5 年总生存率为 63%，5 年无病生存率为 43%。诊断时的 TNM 分期（P=0.03）、组织学亚型（P=0.023）、联合手术和放疗的可能性（P=0.03）和局部控制（P=0.05）是 5 年总生存率提高的显著因素。切缘阳性与 5 年无病生存率较差（ns）趋势相关。
ACC 是一种预后较差的罕见恶性肿瘤，其特点是复发率高。建议的治疗方法是尽可能采用手术和辅助放疗相结合。5 年生存率取决于 TNM 分期、组织学亚型、治疗选择和局部控制。
