Primary aggressive oral lymphomas (PAOL) are a rare subset of extranodal non-Hodgkin lymphomas arising in the oral cavity without evidence of other systemic involvement at diagnosis. PAOL accounts for only about 2–3% of all lymphomas. They most commonly belong to aggressive B-cell subtypes such as Diffuse large B-cell lymphoma (DLBCL) and plasmablastic lymphoma (PBL), with occasional cases of Burkitt lymphoma and T-cell/NK-cell lymphomas. Clinically, these malignancies often present with non-specific symptoms (e.g., swelling, pain, ulceration, tooth mobility) that mimic benign dental conditions, leading to diagnostic delays. An integrated diagnostic approach—combining thorough oral examination, imaging (CT, MRI, PET), and definitive biopsy with immunohistochemistry and genetic studies—is critical for accurate diagnosis and staging. Treatment typically involves systemic chemotherapy, often combined with rituximab for CD20+ tumors and adjunctive radiotherapy for localized disease. Ongoing research into the genomic and microenvironmental landscape of PAOL is paving the way for novel targeted therapies to improve outcomes. In HIV+ or transplant patients, PAOL are often driven by viral co-infections (EBV, HHV-8) and may require tailored therapy, including optimization of immune status. The dentist’s role encompasses not only diagnosis but also active participation in cancer therapy through preventive and supportive dental care, and persists thereafter by monitoring for recurrence and treating chronic treatment sequelae. This review provides a comprehensive overview of PAOL‘s epidemiology, clinical-pathologic and molecular features, current and emerging treatments, and the essential collaborative role of dentists and hematologists in patient care.
原发性侵袭性口腔淋巴瘤（PAOL）是一种罕见的结外非霍奇金淋巴瘤亚型，起源于口腔，诊断时无其他系统性受累证据。PAOL仅占所有淋巴瘤的约2-3%。其最常见类型为侵袭性B细胞亚型，如弥漫性大B细胞淋巴瘤（DLBCL）和浆母细胞淋巴瘤（PBL），偶见伯基特淋巴瘤及T细胞/NK细胞淋巴瘤病例。临床上，这类恶性肿瘤常表现为非特异性症状（如肿胀、疼痛、溃疡、牙齿松动），易与良性牙科疾病混淆，导致诊断延误。综合诊断方法——结合详细口腔检查、影像学检查（CT、MRI、PET）以及包含免疫组化与遗传学检测的确诊性活检——对准确诊断和分期至关重要。治疗通常采用全身化疗，CD20阳性肿瘤常联合利妥昔单抗，局部病变辅以放疗。针对PAOL基因组学及微环境特征的持续研究，正为改善预后的新型靶向治疗开辟道路。在HIV阳性或移植患者中，PAOL常由病毒共感染（EB病毒、HHV-8病毒）驱动，可能需要个体化治疗，包括免疫状态优化。牙科医生的职责不仅涵盖诊断，还需通过预防性和支持性牙科护理积极参与癌症治疗，并在治疗后持续监测复发及处理慢性治疗后遗症。本综述全面阐述了PAOL的流行病学、临床病理与分子特征、当前及新兴治疗方法，以及牙科医生与血液科医生在患者照护中不可或缺的协作角色。
肿瘤(癌症)患者之家