病例报告：原发性下颌腺样囊性癌肺转移的免疫治疗长期疾病控制
Case Report: Long-term disease control with immunotherapy in primary mandibular adenoid cystic carcinoma presenting with pulmonary metastasis.
摘要(中文译文)
下颌骨原发性骨内腺样囊性癌（PIACC）是一种极为罕见的恶性肿瘤。尽管根治性手术和放疗可实现局部区域控制，但由于缺乏标准化全身治疗方案以及腺样囊性癌（ACC）特征性的“冷”免疫微环境，远处转移的管理仍然具有挑战性。我们报告一例62岁男性下颌骨PIACC患者，初诊时即伴发同时性双肺转移（IVC期）。在接受根治性切除及辅助放疗后，患者接受口服替吉奥（S-1）一线维持治疗，获得疾病稳定36个月。肺部进展后，在短暂且耐受性较差的信迪利单抗联合S-1及后续多西他赛尝试之后，治疗方案优化为PD-1抑制剂信迪利单抗联合口服卡培他滨的节拍化疗方案。该免疫节拍治疗方案实现了超过47个月的持久疾病稳定，且毒性轻微。因经济原因停用免疫治疗后，疾病迅速进展，凸显了该联合方案的关键作用。患者随后接受安罗替尼挽救治疗，获得短暂稳定，但最终死于重症肺炎，总生存期为101个月（约8.4年）。本病例挑战了ACC对免疫治疗固有难治的范式。我们推测，全身性干预措施——可能包括 intervening chemotherapy 诱导的免疫原性细胞死亡以及氟尿嘧啶类药物对微环境的重塑——可能使ACC对PD-1阻断敏感。尽管受限于缺乏存档组织进行转化验证，这一假设生成性病例值得进一步研究转移性ACC的联合免疫治疗策略。
Abstract
Primary intraosseous adenoid cystic carcinoma (PIACC) of the mandible is an exceptionally rare malignancy. While radical surgery and radiotherapy provide locoregional control, managing distant metastases remains challenging due to the lack of standard systemic therapies and the characteristically "cold" immune microenvironment of adenoid cystic carcinoma (ACC). We report the case of a 62-year-old male diagnosed with PIACC of the mandible presenting with synchronous bilateral pulmonary metastases (Stage IVC). Following radical resection and adjuvant radiotherapy, the patient received first-line maintenance with oral S-1, achieving stable disease for 36 months. Upon pulmonary progression, after a brief and poorly tolerated trial of sintilimab plus S-1 and subsequent docetaxel, therapy was optimized to a metronomic combination of the PD-1 inhibitor sintilimab and oral capecitabine. This immuno-metronomic regimen achieved durable disease stabilization exceeding 47 months with minimal toxicity. Upon discontinuing immunotherapy due to financial constraints, rapid disease progression ensued, underscoring the critical role of the combination. The patient subsequently received salvage anlotinib, achieving brief stabilization, but passed away from severe pneumonia, yielding a total overall survival of 101 months (~8.4 years). This case challenges the paradigm that ACC is intrinsically refractory to immunotherapy. We hypothesize that systemic interventions, potentially including immunogenic cell death induced by intervening chemotherapy and microenvironment remodeling by fluoropyrimidines, may sensitize ACC to PD-1 blockade. While limited by the lack of archival tissue for translational validation, this hypothesis-generating case warrants further investigation into combination immunotherapy strategies for metastatic ACC.
如何引用
AMA
Hongcheng Yang, Yanzhen Lai, Qingqing Li, Haijin Yang, Meichen Ji, Xuqiang Luo, et al. Case Report: Long-term disease control with immunotherapy in primary mandibular adenoid cystic carcinoma presenting with pulmonary metastasis.. Frontiers in immunology. 2026; doi:10.3389/fimmu.2026.1811044.
APA
Hongcheng Yang, Yanzhen Lai, Qingqing Li, Haijin Yang, Meichen Ji, Xuqiang Luo, et al (2026). Case Report: Long-term disease control with immunotherapy in primary mandibular adenoid cystic carcinoma presenting with pulmonary metastasis.. Frontiers in immunology. https://doi.org/10.3389/fimmu.2026.1811044
GB/T 7714
Hongcheng Yang, Yanzhen Lai, Qingqing Li, Haijin Yang, Meichen Ji, Xuqiang Luo, et al. Case Report: Long-term disease control with immunotherapy in primary mandibular adenoid cystic carcinoma presenting with pulmonary metastasis.[J]. Frontiers in immunology, 2026 doi:10.3389/fimmu.2026.1811044.
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