Numata Ayumi, Sakai Rika, Kawachi Kae, Ishiyama Yasufumi, Hattori Yukako, Takasaki Hirotaka, Yokose Tomoyuki, Nakamura Naoya, Nakajima Hideaki
Department of Medical Oncology, Kanagawa Cancer Center, Yokohama, Japan.
Department of Pathology, Kanagawa Cancer Center, Yokohama, Japan.
J Clin Exp Hematop. 2024 Dec 25;64(4):286-291. doi: 10.3960/jslrt.24010. Epub 2024 Oct 28.
Primary thyroid lymphoma is a rare type of cancer. Most cases involve large B-cell lymphomas (LBCLs), which largely show good prognoses. However, the reasons for this have not been understood. To identify the factors influencing the favorable clinical outcomes of thyroid LBCLs, clinicopathological and genetic analyses of 21 cases of thyroid LBCLs were performed, including immunohistochemistry, fluorescence in situ hybridization (FISH), and analysis for MYD88 mutations based on the World Health Organization Classification of Tumors, 5th Edition. The median age of the patients was 70 years (range, 54-80 years). Fifteen patients (71%) had limited-stage disease. The 5-year overall survival rate was 83% (95% confidence interval: 56%-94%). No instances of central nervous system (CNS) recurrence was observed. The series included 15 cases with diffuse LBCL not otherwise specified (DLBCLnos) and 6 cases with transformation of indolent BCLs (T-IBCLs). Immunohistochemistry subdivided DLBCLs into 12 germinal center B-cell (GCB) and 9 non-GCB subtypes. FISH analysis revealed split signals of MYC in 2/17 cases, MALT1 in 0/15 cases, and BCL6 in 3/15 cases. No MYD88 mutations were detected in any of the cases (0/21). The factors contributing to the favorable clinical course in thyroid LBCLs were a higher proportion of GCB phenotypes and the lack of MYD88 mutations in DLBCLnos and T-IBCLs. Even MYC-R cases showed better prognosis. Further studies involving a large series of LBCLs in extranodal organs are needed to expand on the findings of this study.
原发性甲状腺淋巴瘤是一种罕见的癌症类型。大多数病例为大B细胞淋巴瘤（LBCL），其大多预后良好。然而，其原因尚不清楚。为了确定影响甲状腺LBCL良好临床结局的因素，对21例甲状腺LBCL进行了临床病理和基因分析，包括免疫组织化学、荧光原位杂交（FISH）以及基于《世界卫生组织肿瘤分类》第5版对MYD88突变的分析。患者的中位年龄为70岁（范围54 - 80岁）。15例患者（71%）为局限期疾病。5年总生存率为83%（95%置信区间：56% - 94%）。未观察到中枢神经系统（CNS）复发的病例。该系列包括15例未另行指定的弥漫性LBCL（DLBCLnos）和6例惰性B细胞淋巴瘤转化型（T - IBCL）。免疫组织化学将DLBCL细分为12例生发中心B细胞（GCB）和9例非GCB亚型。FISH分析显示17例中有2例存在MYC分裂信号，15例中有0例存在MALT1分裂信号，15例中有3例存在BCL6分裂信号。所有病例均未检测到MYD88突变（0/21）。甲状腺LBCL临床病程良好的因素是GCB表型比例较高以及DLBCLnos和T - IBCL中缺乏MYD88突变。即使是MYC - R病例也显示出较好的预后。需要进一步开展涉及大量结外器官LBCL的研究来拓展本研究的发现。
