Abstract: Objective To explore the clinicopathological features, diagnosis, treatment and prognosis of sclerosing polycystic adenosis (SPA) and provide a reference for clinics. Methods A case of sclerosing polycystic adenosis of the parotid glands was retrospectively analyzed, and the relevant literature was reviewed. Results A 57-year-old female patient presented with a tumor, which she had noticed for half a month, on the left side of the lower ear, with occasional paroxysmal numbness and no complaint of other discomfort. Resection of the left submandibular area tumor was performed, and the tumor specimen pathological results showed sclerosing polycystic adenosis of the left parotid gland, with no recurrence after six-months follow-up. Sclerosing polycystic adenosis is rare, occurs in the parotid gland and is characterized by a frequently painless, slow-growing mass of the parotid gland. Imaging examination and fine needle aspiration biopsy can only be used as a reference; the diagnosis must include a pathology examination. Histological manifestations showed that abundant sclerotic collagenous stroma was permeated by ductal and acinar lobules, and cystic dilatation of the duct was accompanied by epithelial hyperplasia and diverse ductal cells. Immunohistochemistry of the ductal and acinar cells showed positive expression of cytokeratin (AE1-3 and CAM5.2) and S100 protein. The ducts filled with hyperplastic and dysplastic epithelium were surrounded by an intact myoepithelial layer that was positive for SMA, p63, and calponin, with a Ki-67 index less than 3%. Treatment comprised mainly surgical resection, with a good prognosis. However, one-third of cases relapse: low-grade malignant tumors may occur, with at least one report of invasive cancer. Conclusion Sclerosing polycystic adenosis of the salivary gland is rare and has a good prognosis, but patients may relapse easily after surgery. The diagnosis depends primarily on pathological examination. The main treatment is surgical resection, the prognosis is good, and follow-up should be strengthened after surgery.

Key words: sclerosing polycystic adenopathy, parotid gland, salivary gland tumor, chronic sclerosing sialadenitis, prognosis, recurrence, cancer

摘要： 目的 探讨腮腺硬化性多囊性腺病的临床病理特点、诊 断、治疗及预后,为临床提供参考。方法 对1例腮腺涎腺硬化性多囊性腺病的病例资料及文献进行回顾性分析。结果 患者,女性,57岁,发现左侧耳后下区肿物半个月,偶有阵发性麻木,无其他不适,行“左侧腮腺区肿物切除术”,术后病理检查结果示左腮腺硬化性多囊性腺病,术后6个月随访未见复发。文献复习结果显示：涎腺硬化性多囊性腺病在临床上罕见,好发于腮腺,一般表现为腮腺区缓慢发展的无痛性肿块,偶有疼痛。影像学和细针吸取细胞学检查只能作为诊断参考,需结合组织病理学检查以明确诊断。涎腺硬化性多囊性腺病的组织病理学表现为不规则的导管和腺泡分布于丰富的硬化性间质,导管囊状扩张,可伴上皮增生,导管细胞形态多样。导管和腺泡细胞的免疫组织化学表征为细胞角蛋白（AE1-3 和 CAM5.2）阳性,S100蛋白呈阳性。充满增生和发育不良上皮的导管被肌上皮层包围,SMA、p63和calponin 呈阳性,Ki-67 指数通常小于3%。该病治疗方法主要为手术切除,预后较好,但有1/3的病例复发,且有低度恶性肿瘤的倾向,已有发展为浸润癌的报道。结论 腮腺硬化性多囊性腺病罕见,诊断主要依靠病理学检查,治疗主要为手术切除,预后较好,术后应加强随访。

关键词: 硬化性多囊性腺病, 腮腺, 涎腺肿瘤, 慢性硬化性涎腺炎, 预后, 复发, 癌变