Department of HPB Surgery and Liver Transplantation, Beaujon Hospital, Clichy, France.
Service de Geriatrie, Hôpital "Simone Veil", Eaubonne, France.
Crit Rev Oncol Hematol. 2018 Mar;123:1-6. doi: 10.1016/j.critrevonc.2018.01.004. Epub 2018 Jan 30.
Primary hepatic lymphoma (PHL) is defined as a lympho-proliferative disorder limited to the liver without any involvement of the spleen, lymph nodes, bone marrow or blood. Diffuse large B-cell lymphoma (DLBCL) is the most common histological type counting more than 60-80% of all PHL. Usually, it occurs in middle-aged men with aspecific symptoms and diagnosis is confirmed by histopathology. In order to expand current knowledge and to investigate an optimal therapeutic strategy, a systematic review of literature was conducted in February 2016. A total of 274 articles were retrieved, and after exclusion, 55 were retained, reporting 147 cases of PHL. Patients were mainly men (64.9%) with a median age at diagnosis of 57 years (range: 17-92) and right hepatic lobe involvement (69.6%). Among the 147 patients, 9% received no therapy while 77% underwent treatment including chemotherapy, surgery and radiotherapy in 64%, 26% and 1% of cases, respectively. Mean follow-up was 22.6 months (range: 0.2-360). Overall mortality was 29.2% with a 90-day mortality of 26%. Risk factors for increased mortality include; bilobar lesions (p = 0.001), right lobe localisation (p = 0.003) and non-surgical approach or the absence of any treatment (p = 0.001). A trend towards favourable outcomes for young patients (mean = 50.4 years) with a large liver lesion was achieved by surgical management of PHL but this did not achieve statistical significance. Statistical analysis indicates that in cases of resectable disease, an aggressive surgical approach in selected patients leads to increase long-term survival. Thus, two hypotheses should be assessed in further randomized studies: 1°) resectable PHL is a less severe form or 2°) hepatectomy is an effective treatment for PHL.
原发性肝淋巴瘤（PHL）是指一种局限于肝脏的淋巴组织增生性疾病，无脾脏、淋巴结、骨髓或血液受累。弥漫性大 B 细胞淋巴瘤（DLBCL）是最常见的组织学类型，占所有 PHL 的 60-80%以上。通常，它发生在中年男性，症状不特异，诊断需通过组织病理学证实。为了扩展现有知识并探讨最佳治疗策略，我们于 2016 年 2 月进行了文献系统回顾。共检索到 274 篇文章，排除后保留了 55 篇，报道了 147 例 PHL 患者。患者主要为男性（64.9%），中位年龄为 57 岁（范围：17-92 岁），右肝叶受累（69.6%）。在 147 例患者中，9%未接受治疗，而 77%接受了治疗，包括化疗、手术和放疗，分别占 64%、26%和 1%。平均随访时间为 22.6 个月（范围：0.2-360 个月）。总体死亡率为 29.2%，90 天死亡率为 26%。增加死亡率的危险因素包括：双侧病变（p=0.001）、右叶定位（p=0.003）和非手术方法或无任何治疗（p=0.001）。通过手术治疗 PHL，可使大肝病变的年轻患者（平均年龄为 50.4 岁）获得较好的预后，但差异无统计学意义。统计学分析表明，在可切除病变的情况下，选择合适的患者采用积极的手术方法可增加长期生存。因此，需要在进一步的随机研究中评估两个假设：1°）可切除的 PHL 是一种较轻的形式或 2°）肝切除术是 PHL 的有效治疗方法。
