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  • 季鸿翔,崔龙久,司马辉,匡悦,赵军.原发性肝淋巴瘤误诊肝血管瘤1例及国内文献分析[J].第二军医大学学报,2020,41(6):691-696    [点击复制]
  • JI Hong-xiang,CUI Long-jiu,SIMA Hui,KUANG Yue,ZHAO Jun.Primary hepatic lymphoma misdiagnosed as hepatic hemangioma: a case report and domestic literature review[J].Acad J Sec Mil Med Univ,2020,41(6):691-696   [点击复制]
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原发性肝淋巴瘤误诊肝血管瘤1例及国内文献分析
季鸿翔1,崔龙久2,司马辉3,匡悦1,赵军1*
0
(1. 海军军医大学(第二军医大学)东方肝胆外科医院特需诊疗二科, 上海 200438;
2. 海军军医大学(第二军医大学)东方肝胆外科医院胆道二科二病区, 上海 200438;
3. 海军军医大学(第二军医大学)东方肝胆外科医院肝外五科, 上海 200438
*通信作者)
摘要:
目的 探讨原发性肝淋巴瘤的诊断、治疗、预后特点,提高对该肿瘤的认识。方法 报道1例误诊为肝血管瘤的原发性肝淋巴瘤患者的诊治经过,并回顾分析1975―2019年国内相关文献,探讨原发性肝淋巴瘤诊治和预后特点。结果 本例患者无特异临床表现,在外院误诊为肝血管瘤。我院行MRI检查见肿瘤呈恶性表现并可见血管漂浮征,考虑恶性可能。予肝多发肿瘤切除+肝十二指肠淋巴结清扫+胆囊切除术。手术标本病理组织学表现:肿瘤由大片细胞质少、核具异型性的淋巴样细胞构成,无明显包膜,周边肝组织受淋巴细胞浸润;免疫组织化学染色示:滤泡区细胞大部CD20(+),生发中心细胞CD10(+)、Bcl-6(+)、Bcl-2(-)、Ki-67(80%)。最终诊断为原发性肝B细胞非霍奇金淋巴瘤。检索到的91篇国内文献共报道376例原发性肝淋巴瘤患者,平均年龄为(48.8±9.6)岁,男女比例为1.69∶1。影像学检查以单发病灶多见,乏血供肿瘤表现,以血管漂浮征为特点。146例(38.8%)患者出现乳酸脱氢酶升高。132例(35.1%)患者发生误诊,最终确诊依赖病理结果。生存分析结果显示,B细胞非霍奇金淋巴瘤患者预后较T细胞非霍奇金淋巴瘤患者好(中位总生存时间:36.4个月vs 20.7个月,P=0.009);B细胞非霍奇金淋巴瘤患者中,黏膜相关淋巴组织淋巴瘤患者预后较弥漫大B细胞淋巴瘤患者好(52.0个月vs 30.0个月,P=0.049),手术联合术后化学治疗的患者预后较单纯化学治疗和手术患者好(45.6个月vs 23.9和7.7个月,P=0.016)。结论 原发性肝淋巴瘤作为一种少见肿瘤,临床特征不典型,需结合病史、临床表现、影像学、实验室检查综合判断,一旦确诊应采取手术联合化学治疗或综合化学治疗方案进行积极治疗。
关键词:  原发性肝淋巴瘤|弥漫性大B细胞淋巴瘤|黏膜相关组织淋巴瘤|预后
DOI:10.16781/j.0258-879x.2020.06.0691
投稿时间:2019-12-31修订日期:2020-02-16
基金项目:
Primary hepatic lymphoma misdiagnosed as hepatic hemangioma: a case report and domestic literature review
JI Hong-xiang1,CUI Long-jiu2,SIMA Hui3,KUANG Yue1,ZHAO Jun1*
(1. Department of VIP Medicine (Ⅱ), Eastern Hepatobiliary Surgery Hospital, Naval Medical University (Second Military Medical University), Shanghai 200438, China;
2. The Second Ward, Department of Hepatobiliary Surgery (Ⅱ), Eastern Hepatobiliary Surgery Hospital, Naval Medical University (Second Military Medical University), Shanghai 200438, China;
3. Department of Hepatic Surgery (Ⅴ), Eastern Hepatobiliary Surgery Hospital, Naval Medical University (Second Military Medical University), Shanghai 200438, China
*Corresponding author)
Abstract:
Objective To explore the characteristics of diagnosis, treatment and prognosis of primary hepatic lymphoma (PHL), so as to better understand the tumor. Methods The diagnosis and treatment of a patient with PHL misdiagnosed as hepatic hemangioma were reported, and the domestic literatures were reviewed from 1975 to 2019 to sum up the diagnosis, treatment and prognosis characteristics of PHL. Results This patient had no specific clinical manifestations, and was misdiagnosed as hepatic hemangioma in other hospital. Magnetic resonance imaging (MRI) examination in our hospital showed that the tumor had malignant features and vessel floating sign, suggesting the possibility of malignancy. The patient was treated with resection of multiple hepatic tumors+hepatoduodenal lymph node dissection+cholecystectomy. Postoperative pathological results showed that the tumor was composed of large patches of lymphoid cells with few cytoplasm and atypical nuclei; the tumor had no obvious envelope; and the peripheral liver tissues were infiltrated. Immunohistochemical staining showed that the cells in follicular zone were mostly CD20 (+), and the cells in germinal center were CD10 (+), Bcl-6 (+) and Bcl-2 (-), Ki-67 (80%). Primary hepatic B-cell non-Hodgkin lymphoma (B-NHL) was diagnosed finally. A total of 376 PHL patients were reported in 91 domestic literatures, with an average age of 48.8±9.6 years and a male-to-female ratio of 1.69:1. The imaging was characterized by single lesion and vessel floating sign, lack of blood supply. The levels of lactic dehydrogenase were increased in 146 cases (38.8%). Totally 132 cases (35.1%) were misdiagnosed and the final diagnosis depended on pathological findings. Survival analysis results showed that the prognosis of B-NHL patients was significantly better than that of T-cell non-Hodgkin lymphoma (T-NHL) patients (median overall survival time:36.4 months vs 20.7 months, P=0.009); the prognosis of mucosa-associated lymphoid tissue lymphoma patients was significantly better than that of diffuse large B-cell lymphoma patients (52.0 months vs 30.0 months, P=0.049); and the prognosis of B-NHL patients treated with surgery combined with postoperative chemotherapy was significantly better compared with those treated with surgery alone and those treated with chemotherapy alone (45.6 months vs 23.9 months and 7.7 months, P=0.016). Conclusion Primary hepatic lymphoma is a rare tumor without characteristic clinical features, and its diagnosis needs to be combined with medical history, clinical manifestations, and imaging and laboratory examination. Once PHL is confirmed, it should be treated actively by surgery combined with chemotherapy or comprehensive chemotherapy.
Key words:  primary hepatic lymphoma|diffuse large B-cell lymphoma|mucosa associated tissue lymphoma|prognosis