Atallah Sarah, Marc Morgane, Schernberg Antoine, Huguet Florence, Wagner Isabelle, Mäkitie Antti, Baujat Bertrand
Department of Otorhinolaryngology-Head and Neck Surgery, Sorbonne University, Tenon Hospital, AP-HP, Paris, France.
Doctoral School of Public Health, University of Paris Sud, CESP, INSERM U1018, University of Paris-Saclay, UVSQ, Villejuif, France.
Cancer Manag Res. 2022 Jun 4;14:1879-1890. doi: 10.2147/CMAR.S355663. eCollection 2022.
Adenoid cystic carcinoma (AdCC) is a rare tumour as it accounts for about 10% of all salivary gland neoplasms. It occurs in all age groups with a predominance of women, but no risk factors have been identified to date. Although AdCC behaves as a slow-growing tumour, it is characterized by multiple and late recurrences. Therefore, we aim to update the knowledge of the treatment options in advanced and recurrent cases.
We performed a systematic literature review to provide a synthesis of the practical knowledge required for AdCC non-surgical management. Altogether, 99 out of the 1208 available publications were selected for analysis.
AdCC is described as a basaloid tumour consisting of epithelial and myoepithelial cells. Immunohistochemistry is useful for diagnosis (PS100, Vimentin, CD117, CKit, muscle actin, p63) and for prognosis (Ki67). Identified mutations could lead to therapeutic opportunities (MYB-NFIB, Notch 1). The work-up is mainly based on neck and chest CT scan and MRI, and PET-CT with 18-FDG or PSMA can be considered. Surgical treatment remains the gold standard in resectable cases. Post-operative intensity modulated radiotherapy is the standard of care, but hadron therapy may be used in specific situations. Based on the available literature, no standard chemotherapy regimen can be recommended.
There is currently no consensus on the use of chemotherapy in AdCC, either concomitantly to RT in a postoperative setting or at a metastatic stage. Further, the available targeted therapies do not yet provide significant tumour response.
腺样囊性癌（AdCC）是一种罕见肿瘤，约占所有唾液腺肿瘤的10%。它可发生于各年龄组，女性居多，但迄今尚未发现危险因素。尽管腺样囊性癌生长缓慢，但其特点是多次复发且复发较晚。因此，我们旨在更新晚期和复发病例治疗选择的相关知识。
我们进行了一项系统的文献综述，以综合腺样囊性癌非手术治疗所需的实践知识。总共从1208篇可用出版物中筛选出99篇进行分析。
腺样囊性癌被描述为一种由上皮细胞和肌上皮细胞组成的基底样肿瘤。免疫组织化学对诊断（PS100、波形蛋白、CD117、CKit、肌动蛋白、p63）和预后评估（Ki67）有用。已识别的突变可能带来治疗机会（MYB-NFIB、Notch 1）。检查主要基于颈部和胸部CT扫描及MRI，可考虑使用18-FDG或PSMA的PET-CT。手术治疗仍是可切除病例的金标准。术后调强放疗是标准治疗方法，但在特定情况下可使用强子治疗。基于现有文献，无法推荐标准的化疗方案。
目前对于腺样囊性癌化疗的使用，无论是在术后与放疗联合使用还是在转移阶段使用，均未达成共识。此外，现有的靶向治疗尚未产生显著的肿瘤反应。
