腺样囊性恶性肿瘤（CXPA）是唾液腺的一种肿瘤，占唾液腺肿瘤的3.6%和唾液腺恶性肿瘤的12%。其预后由组织学在腺瘤包膜之外的进展决定。CXPA被认为是一种始发性或复发性多形性腺瘤的恶性转化，与良性和恶性病变相关。唾液腺癌是一种罕见的异质性肿瘤组，具有复杂的临床病理特征和独特的生物学行为。本病例报告总结了一名左腮腺CXPA患者的治疗情况，携带HER2扩增且预后不良。患者总生存期超过3.5年。本文讨论了免疫检查点抑制剂和靶向治疗联合方案在CXPA癌治疗中的应用和结果。靶向治疗与免疫治疗的联合长期临床受益，并且具有高临床响应率的靶向治疗（免疫治疗+双靶向三药联合方案）可能是治疗罕见和/或难治性肿瘤患者的理想选择，而不损害患者的安全性。© 2023 Elsevier Ltd. 保留所有权利。

Carcinoma ex pleomorphic adenoma (CXPA) is a neoplasm of the salivary gland that causes 3.6% of salivary gland tumors and 12% of salivary gland malignancies. Its prognosis is determined by the histological progression beyond the adenoma capsule. CXPA is thought to be a malignant transformation of a primary or recurrent pleomorphic adenoma and is associated with both benign and malignant lesions. Salivary gland cancers represent a rare heterogeneous group of neoplasms with complex clinicopathological characteristics and distinct biological behavior.This case report summarizes the treatment of a 57-year-old male patient with CXPA of the left parotid gland, harboring HER2 amplification with poor prognosis. The overall survival of the patient has been > 3.5 years. The application and outcome of an immune checkpoint inhibitor and targeted therapy combination regimens in the treatment of CXPA carcinoma are discussed.Targeted therapy combined with immunotherapy has long-term clinical benefits and targeted therapy which has a high clinical response rate (immunotherapy + dual-targeting three-drug regimens) may present an ideal choice for the treatment of patients with rare and/or refractory tumors without compromising patient safety.Copyright © 2023 Elsevier Ltd. All rights reserved.