Chen Huan, Pan Tao, He Yizi, Zeng Ruolan, Li Yajun, Yi Liming, Zang Hui, Chen Siwei, Duan Qintong, Xiao Ling, Zhou Hui
Department of Lymphoma and Hematology, Hunan Cancer Hospital, The Affiliated Cancer Hospital of Xiangya School of Medicine, Central South University, Changsha, China.
The Third Xiangya Hospital, Central South University, Changsha, China.
Front Oncol. 2021 Mar 22;11:654854. doi: 10.3389/fonc.2021.654854. eCollection 2021.
Primary mediastinal large B-cell lymphoma (PMBCL) is a distinct clinicopathologic disease from other types of diffuse large B-cell lymphoma (DLBCL) with unique prognostic features and limited availability of clinical data. The current standard treatment for newly diagnosed PMBCL has long been dependent on a dose-intensive, dose-adjusted multi-agent chemotherapy regimen of rituximab plus etoposide, prednisone, vincristine, cyclophosphamide, and doxorubicin (DA-R-EPOCH). Recent randomized trials have provided evidence that R-CHOP followed by consolidation radiotherapy (RT) is a valuable alternative option to first-line treatment. For recurrent/refractory PMBCL (rrPMBCL), new drugs such as pembrolizumab and CAR-T cell therapy have proven to be effective in a few studies. Positron emission tomography-computed tomography (PET-CT) is the preferred imaging modality of choice for the initial phase of lymphoma treatment and to assess response to treatment. In the future, baseline quantitative PET-CT can be used to predict prognosis in PMBCL. This review focuses on the pathology of PMBCL, underlying molecular basis, treatment options, radiotherapy, targeted therapies, and the potential role of PET-CT to guide treatment choices in this disease.
原发性纵隔大B细胞淋巴瘤（PMBCL）是一种与其他类型弥漫性大B细胞淋巴瘤（DLBCL）不同的临床病理疾病，具有独特的预后特征且临床数据有限。长期以来，新诊断PMBCL的当前标准治疗依赖于利妥昔单抗联合依托泊苷、泼尼松、长春新碱、环磷酰胺和多柔比星的剂量密集、剂量调整多药化疗方案（DA-R-EPOCH）。最近的随机试验提供了证据，表明R-CHOP方案序贯巩固放疗（RT）是一线治疗的一种有价值的替代选择。对于复发/难治性PMBCL（rrPMBCL），在一些研究中，诸如帕博利珠单抗和嵌合抗原受体T细胞（CAR-T）疗法等新药已被证明有效。正电子发射断层扫描-计算机断层扫描（PET-CT）是淋巴瘤治疗初始阶段以及评估治疗反应的首选成像方式。未来，基线定量PET-CT可用于预测PMBCL的预后。本综述重点关注PMBCL的病理学、潜在分子基础、治疗选择、放疗、靶向治疗以及PET-CT在指导该疾病治疗选择方面的潜在作用。
