原发性肠淋巴瘤(primary intestinal lymphoma, PIL)是一种罕见且复杂的非霍奇金淋巴瘤,其发病率低,病理类型复杂,诊断困难,治疗方案尚未完善。近年来,现代影像技术显著提高了疾病的早期检出率和分期准确性,内镜检查结合实时活检技术增强了病理诊断的精确性,免疫组化和分子诊断手段的综合应用进一步提升了对亚型的鉴别能力,尽管化疗和放疗仍为标准治疗手段,但新型疗法如免疫治疗和靶向药物正在改写治疗格局。本文旨在深入探讨PIL的流行病学、病因学、临床特征、诊断标准、最佳治疗方案以及预后因素,以期为PIL患者的临床诊疗提供借鉴与参考。Primary intestinal lymphoma (PIL) is a rare and complex subtype of non-Hodgkin lymphoma, characterized by low incidence, diverse histopathology, diagnostic challenges, and lacking of standardized treatment protocols. Recent advancements in imaging, endoscopic techniques, and molecular diagnostics have significantly improved early detection and subtyping of PIL. Modern imaging modalities, such as CT and MRI, enhance disease identification and staging, while endoscopic techniques combined with real-time biopsy facilitate accurate pathological diagnosis. The integration of immunohistochemistry and molecular diagnostics further refines subtyping and provides valuable prognostic information. While chemotherapy and radiotherapy remain the main treatment for PIL, novel therapies, including immunotherapy and targeted agents, are increasingly contributing to improved outcomes. These advancements, alongside diagnostic innovations significantly alter the treatment landscape for patients with PIL. This review aims to provide a comprehensive overview of PIL, encompassing its epidemiology, etiology, clinical presentation, diagnostic criteria, optimal treatment strategies, and prognostic factors. By synthesizing current knowledge, this review seeks to offer valuable insights into the clinical management of patients w
