Department of Pathology, Central Hospital of Wuhan, Tongji Medical College, Huazhong University of Science and Technology, Wuhan, China.
Department of Pathology, Union Hospital, Tongji Medical College, Huazhong University of Science and Technology, Wuhan, China.
Cancer Med. 2021 Feb;10(3):1120-1127. doi: 10.1002/cam4.3707. Epub 2021 Jan 15.
Adenoid cystic carcinoma (ACC) is a distinctive tumour. Limited studies involving a large population have reported multicentre systematic analyses of the clinical, pathological and immunohistochemical (IHC) features of ACC as well as the potential role of IHC markers in the prognosis of ACC.
The clinical, histopathological and IHC data of 296 cases obtained from two tertiary hospitals were analysed. The age at onset ranged from 12 to 87 years with a median age of 52 years. The male-to-female ratio was 1:1.3. Patients with ACC arising from the lacrimal gland were younger than those with tumours arising from other sites. Patients with tumours in the extra auditory canal and nasopharynx were older than those with tumours in other locations. Histopathologically, solid type ACC was the most frequent in the nasal cavity and paranasal sinus (6/51) group. Tumours arising from the oral cavity most commonly showed perineural invasion (10/60) and margin positivity (11/60). IHC analyses showed that CK8/18, CK7, CK14, epithelial membrane antigen and CD117 were expressed in 35/35 (100%), 87/88 (98.8%), 26/27 (96.2%), 42/43 (97.6%) and 113/120 (94.1%) patients, respectively. CK5/6, P63, smooth muscle actin, calponin and S100 were positively expressed in 73/73 (100%), 111/124 (89.5%), 38/43 (88.3%), 41/50 (82.0%) and 61/92 (66.3%) cases, respectively. S100 proteins were expressed in 54 (54/77) primary cases and two (2/9) metastatic cases (p = 0.013).
ACC is a distinctive tumour that mainly affects middle-aged and elderly individuals, with a mild female predominance. Loss of expression of S100 proteins may be a poor prognostic factor associated with metastasis.
腺样囊性癌（ACC）是一种具有独特特征的肿瘤。少数涉及大样本量的研究报告了多中心系统分析 ACC 的临床、病理和免疫组织化学（IHC）特征，以及 IHC 标志物在 ACC 预后中的潜在作用。
对来自两家三级医院的 296 例病例的临床、组织病理学和 IHC 数据进行分析。发病年龄为 12 至 87 岁，中位年龄为 52 岁。男女比例为 1:1.3。起源于泪腺的 ACC 患者比起源于其他部位的患者更年轻。发生在外耳道和鼻咽部的肿瘤患者比发生在其他部位的患者年龄更大。组织病理学上，鼻腔和鼻窦（6/51）组最常见的是实性型 ACC。口腔部位的肿瘤最常见神经周围侵犯（10/60）和边缘阳性（11/60）。免疫组化分析显示，35/35（100%）、87/88（98.8%）、26/27（96.2%）、42/43（97.6%）和 113/120（94.1%）患者分别表达 CK8/18、CK7、CK14、上皮膜抗原和 CD117。73/73（100%）、111/124（89.5%）、38/43（88.3%）、41/50（82.0%）和 61/92（66.3%）患者分别表达 CK5/6、P63、平滑肌肌动蛋白、钙调蛋白和 S100。54 例（54/77）原发病例和 2 例（2/9）转移病例中表达 S100 蛋白（p=0.013）。
ACC 是一种主要影响中老年人的独特肿瘤，女性略多见。S100 蛋白表达缺失可能是与转移相关的不良预后因素。
