Ma Jennifer, Daou Remy, Bou Eid Josiane, Fregonese Beatrice, El-Khoury Joe, Wijetunga N Ari, Imber Brandon S, Yahalom Joachim, Hajj Carla
Department of Radiation Oncology, Memorial Sloan Kettering Cancer Center, New York, NY, United States.
Department of Family Medicine, Saint Joseph University, Beirut, Lebanon.
Front Oncol. 2025 Mar 20;15:1475118. doi: 10.3389/fonc.2025.1475118. eCollection 2025.
PURPOSE/OBJECTIVE: Primary hepatic lymphomas (PHL) are an extremely rare form of non-Hodgkin Lymphoma (NHL) for which there are no established treatment guidelines, with available literature largely comprised of small case reports. Therefore, we evaluate our institutional experience treating PHL within the context of existing literature to better understand treatment modalities, role of radiotherapy (RT), and outcomes.
MATERIALS/METHODS: We conducted a single institutional retrospective study of all patients with PHL diagnosed from 2000-2021, defined as a biopsy-proven liver lesion in the absence of other lymphomatous solid organ involvement, except for concurrently diagnosed hepatosplenic lymphomas. Subgroup analysis was performed for diffuse large B-cell lymphoma (DLBCL) and indolent lymphomas, which included marginal zone lymphoma (MZL), Grade 1-2 follicular lymphoma (FL), and low-grade B-cell lymphoma (BCL), NOS. Univariable (UVA) and multivariable analysis (MVA) for overall survival (OS) were performed using the Cox proportional hazards model. A literature review was conducted using key words "liver", "lymphoma", and "treatment" to identify relevant literature.
We identified 30 patients with PHL within the institutional cohort and 192 patients from comprehensive literature review. Subgroup analysis of DLBCL included 15 patients. On MVA for OS, only ECOG score (p=0.02) and Lugano stage (p=0.04) remained significant. Subgroup analysis of the indolent lymphoma group included 9 patients. On MVA for OS, only age remained significant. Systemic therapy was the most common treatment modality overall (20 patients; 67%) with surgery, radiation and observation utilized in 4 patients (13%) each. Seventeen (57%) of patients were alive at the time of data collection, with 8 (27%) deceased and 5 (17%) lost to follow-up.
PHL are an extremely rare subtype of NHL for which there is no clear treatment consensus. Primary hepatic DLBCL appears to be treated mostly with chemotherapy with good disease control. For indolent PHL, low-dose RT appears to have good overall disease control with minimal toxicity. Our RT data is limited by the short duration of follow-up for patients receiving RT compared to those who received chemotherapy, surgery or observation. However, our results are encouraging for the use of RT for appropriate patients with indolent PHL.
目的/目标：原发性肝淋巴瘤（PHL）是一种极其罕见的非霍奇金淋巴瘤（NHL），目前尚无既定的治疗指南，现有文献主要由小型病例报告组成。因此，我们结合现有文献评估了我们机构治疗PHL的经验，以更好地了解治疗方式、放疗（RT）的作用和治疗结果。
材料/方法：我们对2000年至2021年期间诊断的所有PHL患者进行了单机构回顾性研究，定义为经活检证实的肝脏病变，不存在其他淋巴瘤实体器官受累情况，但同时诊断为肝脾淋巴瘤的除外。对弥漫性大B细胞淋巴瘤（DLBCL）和惰性淋巴瘤进行了亚组分析，惰性淋巴瘤包括边缘区淋巴瘤（MZL）、1-2级滤泡性淋巴瘤（FL）和低级别B细胞淋巴瘤（BCL），NOS。使用Cox比例风险模型对总生存期（OS）进行单变量（UVA）和多变量分析（MVA）。使用关键词“肝脏”、“淋巴瘤”和“治疗”进行文献综述，以识别相关文献。
我们在机构队列中确定了30例PHL患者，并通过全面文献综述确定了192例患者。DLBCL的亚组分析包括15例患者。在OS的MVA中，只有ECOG评分（p=0.02）和卢加诺分期（p=0.04）仍然具有显著性。惰性淋巴瘤组的亚组分析包括9例患者。在OS的MVA中，只有年龄仍然具有显著性。全身治疗是总体上最常见的治疗方式（20例患者；67%），手术、放疗和观察各有4例患者采用（13%）。在数据收集时，17例（57%）患者存活，8例（27%）死亡且5例（17%）失访。
PHL是一种极其罕见的NHL亚型，目前尚无明确的治疗共识。原发性肝DLBCL似乎主要采用化疗治疗，疾病控制良好。对于惰性PHL，低剂量放疗似乎能在毒性最小的情况下实现良好的总体疾病控制。与接受化疗、手术或观察的患者相比，我们的放疗数据受到接受放疗患者随访时间短的限制。然而，我们的结果对于将放疗用于合适的惰性PHL患者来说是令人鼓舞的。
