Lazzarino M, Orlandi E, Paulli M, Sträter J, Klersy C, Gianelli U, Gargantini L, Rousset M T, Gambacorta M, Marra E, Lavabre-Bertrand T, Magrini U, Manegold C, Bernasconi C, Möller P
Department of Pathology, University of Pavia, Italy.
J Clin Oncol. 1997 Apr;15(4):1646-53. doi: 10.1200/JCO.1997.15.4.1646.
To define clinicopathologic features, response to treatment, and prognostic factors of primary mediastinal B-cell lymphoma (MBL), a CD20+ tumor recognized as a distinct entity among non-Hodgkin's lymphomas (NHL).
One hundred six patients presented with disease confined to thorax (86%), bulky mediastinum (73%), superior vena cava syndrome (47%), and contiguous infiltration (57%). Ninety-nine received doxorubicin-containing chemotherapy (CHT).
Thirty-five of 99 patients were primarily CHT-resistant, and 64 responded: 23 achieved complete response (CR) and 41 achieved response with residual mediastinal abnormality. Seventy-seven percent of responders received mediastinal radiotherapy (RT). Of 64 responders, 18 (28%) relapsed: none of 23 CR patients and 18 of 41 (44%) with residual mediastinal abnormality. Relapse-free survival rate of responders was 71% at 3 years. Actuarial 3-year survival rate was 52% for all patients and 82% for responders. Predictive factors of poor outcome were identified by logistic regression; Cox survival analysis was performed on death and relapse. Pericardial effusion (P < .001) and Eastern Cooperative Oncology Group (ECOG) performance status > or = 2 (P = .009) predicted nonresponse (NR) and affected survival. Less than partial midway response to CHT predicted NR to subsequent therapies. Bulky disease was related to persistent mediastinal abnormality and risk of relapse (P = .025).
MBL is an aggressive NHL with unique clinicopathologic aspects, often refractory to current CHT designed for high-grade NHL. Poor performance status and pericardial effusion predict NR and poor survival. Inadequate response after the first courses of front-line CHT predicts failure of subsequent treatment. Responders with bulky mediastinum or residual mediastinal abnormality after CHT are at risk of relapse. These factors should help to select high-risk patients for intensive treatments.
明确原发性纵隔B细胞淋巴瘤（MBL）的临床病理特征、对治疗的反应及预后因素。MBL是一种CD20+肿瘤，在非霍奇金淋巴瘤（NHL）中被视为一种独特的实体。
106例患者的疾病局限于胸部（86%），有巨大纵隔肿物（73%），上腔静脉综合征（47%），以及连续性浸润（57%）。99例接受了含阿霉素的化疗（CHT）。
99例患者中35例对CHT原发性耐药，64例有反应：23例达到完全缓解（CR），41例有反应但纵隔仍有异常。77%有反应的患者接受了纵隔放疗（RT）。64例有反应的患者中，18例（28%）复发：23例CR患者均未复发，41例纵隔仍有异常的患者中有18例（44%）复发。有反应患者的3年无复发生存率为71%。所有患者的3年精算生存率为52%，有反应患者为82%。通过逻辑回归确定预后不良的预测因素；对死亡和复发进行Cox生存分析。心包积液（P<0.001）和东部肿瘤协作组（ECOG）体能状态≥2（P = 0.009）预测无反应（NR）并影响生存。对CHT的反应小于部分中期反应预测对后续治疗无反应。巨大肿物与纵隔持续异常及复发风险相关（P = 0.025）。
MBL是一种侵袭性NHL，具有独特的临床病理特征，对目前用于高级别NHL的CHT往往耐药。体能状态差和心包积液预测无反应及生存不良。一线CHT的首个疗程后反应不足预测后续治疗失败。CHT后有巨大纵隔肿物或纵隔仍有异常的有反应患者有复发风险。这些因素应有助于选择高危患者进行强化治疗。
