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以肝脏原发性肿瘤形式出现的非霍奇金淋巴瘤:8例患者的临床表现、诊断及预后

Non-Hodgkin's lymphoma presenting as a primary tumor of the liver: presentation, diagnosis and outcome in eight patients.

作者信息

Scoazec J Y, Degott C, Brousse N, Barge J, Molas G, Potet F, Benhamou J P

机构信息

Service Central d'Anatomie et Cytologie Pathologiques, Hôpital Beaujon, Clichy, France.

出版信息

Hepatology. 1991 May;13(5):870-5.

PMID:2029991
Abstract

We report the diagnostic, prognostic and therapeutic features of non-Hodgkin's lymphoma in eight patients in whom the disease was seen as a primary tumor of the liver. This series illustrates the variety of situations in which lymphoma might be diagnosed: (a) abdominal pain and hepatomegaly (three cases), (b) incidental finding at evaluation of a patient with cirrhosis (two cases), (c) secondary neoplasm after treatment for Hodgkin's disease (one case) and (d) complication of AIDS (two cases). In most cases, clinical and/or radiological features were nonspecific. However, the combination of the following features must be considered as suggestive: occurrence of an apparently primary hepatic tumor in an immunocompromised patient, absence of the usual serum tumor markers and increased serum lactic dehydrogenase activity. The final diagnosis was based on histological examination of specimens obtained by ultrasonically guided liver biopsies or at surgery. All cases belonged to unfavorable histological subtypes. Immunohistochemical findings on paraffin-embedded sections demonstrated the B-lymphocyte lineage of the seven tumors available for study. In the three patients without coexisting disease, complete remission was obtained by surgery alone or combined with chemotherapy. In the two patients with coexisting cirrhosis, outcome was rapidly unfavorable, with death occurring less than 3 mo after diagnosis. Among the three immunocompromised patients, two experienced a rapid unfavorable outcome, and the remaining one was in complete remission after surgery and chemotherapy. In conclusion, primary non-Hodgkin's lymphoma of the liver arising in patients without coexisting disease has a slow progression and might be successfully treated by surgery.(ABSTRACT TRUNCATED AT 250 WORDS)

摘要

我们报告了8例非霍奇金淋巴瘤患者的诊断、预后及治疗特征,这些患者的疾病表现为肝脏原发性肿瘤。本系列病例说明了淋巴瘤可能被诊断的多种情况:(a)腹痛和肝肿大(3例),(b)在评估肝硬化患者时偶然发现(2例),(c)霍奇金病治疗后的继发性肿瘤(1例)以及(d)艾滋病并发症(2例)。在大多数情况下,临床和/或放射学特征不具有特异性。然而,以下特征的组合必须被视为具有提示性:免疫功能低下患者出现明显的原发性肝肿瘤、缺乏常见的血清肿瘤标志物以及血清乳酸脱氢酶活性升高。最终诊断基于通过超声引导下肝活检或手术获取的标本的组织学检查。所有病例均属于预后不良的组织学亚型。对石蜡包埋切片的免疫组化结果显示,可供研究的7例肿瘤为B淋巴细胞谱系。在3例无并存疾病的患者中,单纯手术或联合化疗获得了完全缓解。在2例并存肝硬化的患者中,预后迅速变差,诊断后不到3个月即死亡。在3例免疫功能低下的患者中,2例预后迅速变差,其余1例在手术和化疗后完全缓解。总之,无并存疾病患者发生的肝脏原发性非霍奇金淋巴瘤进展缓慢,手术治疗可能成功。(摘要截选于250词)

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