MYC oncogene rearrangements (MYC-R) negatively affect survival in patients with Ann Arbor stage III–IV diffuse large B-cell lymphoma (DLBCL), but their impact in limited stage (LS) I–II is unclear. Therefore, we assessed the impact of MYC-R on progression-free survival (PFS) and overall survival (OS) in LS DLBCL patients at the population level. We identified 1,434 LS DLBCL patients with known MYC-R status diagnosed between 2014 and 2020, who received R-CHOP(-like) regimens using the Netherlands Cancer Registry, with survival follow-up until February 2022. Stage I patients with (n = 83, 11%) and without (n = 650, 89%) a MYC-R had similar 2-years PFS (89% and 93%, p = 0.63) and OS (both 95%, p = 0.22). Conversely, stage II DLBCL patients with a MYC-R (n = 90, 13%) had inferior survival outcomes compared to stage II patients without a MYC-R (n = 611, 87%) (PFS 70% vs. 89%, p = 0.001; OS 79% vs. 94%, p < 0.0001). Both single MYC-R (single hit, n = 36) and concurrent BCL2 and/or BCL6 rearrangements (double/triple hit, n = 39) were associated with increased mortality and relapse risk. In conclusion, in stage II DLBCL a MYC-R is negatively associated with survival. In stage I DLBCL, however, survival outcomes are excellent irrespective of MYC-R status. This challenges the diagnostic assessment of MYC-R in stage I DLBCL patients.
MYC癌基因重排（MYC-R）对安阿伯分期III–IV期弥漫性大B细胞淋巴瘤（DLBCL）患者的生存有负面影响，但其对局限期（LS）I–II期的影响尚不明确。为此，我们在人群层面评估了MYC-R对局限期DLBCL患者无进展生存期（PFS）和总生存期（OS）的影响。通过荷兰癌症登记处，我们确定了2014年至2020年间诊断的1,434例已知MYC-R状态的局限期DLBCL患者，这些患者均接受了R-CHOP（类似）方案治疗，生存随访持续至2022年2月。伴有MYC-R（n=83，11%）与不伴有MYC-R（n=650，89%）的I期患者具有相似的2年PFS（89% vs. 93%，p=0.63）和OS（均为95%，p=0.22）。相反，伴有MYC-R的II期DLBCL患者（n=90，13%）与不伴有MYC-R的II期患者（n=611，87%）相比，生存结局较差（PFS 70% vs. 89%，p=0.001；OS 79% vs. 94%，p<0.0001）。无论是单一的MYC-R（单打击，n=36）还是伴随BCL2和/或BCL6重排（双打击/三打击，n=39）均与死亡率和复发风险增加相关。总之，在II期DLBCL中，MYC-R与生存率呈负相关；然而在I期DLBCL中，无论MYC-R状态如何，生存结局均非常良好。这对I期DLBCL患者进行MYC-R的诊断评估提出了质疑。
肿瘤(癌症)患者之家