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原发性肾非霍奇金淋巴瘤。一个不寻常的结外部位。

Primary renal non-Hodgkin's lymphoma. An unusual extranodal site.

作者信息

Okuno S H, Hoyer J D, Ristow K, Witzig T E

机构信息

Department of Internal Medicine, Mayo Clinic, Rochester, MN 55905, USA.

出版信息

Cancer. 1995 May 1;75(9):2258-61. doi: 10.1002/1097-0142(19950501)75:9<2258::aid-cncr2820750911>3.0.co;2-s.

DOI:10.1002/1097-0142(19950501)75:9<2258::aid-cncr2820750911>3.0.co;2-s
PMID:7712433
Abstract

BACKGROUND

Primary renal non-Hodgkin's lymphoma (NHL) is rare. Because the renal parenchyma does not have lymphatics, the existence of this entity has been questioned. The goal of this study was to determine the clinical presentation, pathologic features, and disease course of patients with primary renal NHL and review the pertinent literature on this unusual extranodal NHL.

METHODS

All medical records from the Mayo Clinic from 1976 to 1992 with the diagnosis of renal NHL were retrospectively reviewed. One-hundred seventy-six cases were identified, five of which met the criteria for primary renal NHL. The clinical, pathologic, and radiographic features were reviewed in detail and are the basis of this report.

RESULTS

The median age at diagnosis of the five patients with primary renal NHL was 60 years (range, 52-63 years) with a male-to-female ratio of 2:3. All patients had flank pain as their initial presentation. Urinalysis was abnormal in only one patient. In three patients, the serum creatinine was elevated. Tumor histology was diffuse large cell in four cases; and small noncleaved non-Burkitt's in one. All five were B-cell immunophenotype. All patients received combination chemotherapy. Although the median survival for the group was only eight months, two remain in complete remission longer than 80 months from therapy. These two had total removal of macroscopic lymphoma and received combination chemotherapy and consolidation radiotherapy.

CONCLUSIONS

Primary renal non-Hodgkin's lymphoma does exist. Patients whose lymphomas were completely resected macroscopically and who received combination chemotherapy with consolidation radiation therapy had long disease free survival. Patients with bilateral renal involvement or no debulking of the renal lymphoma tended to have poorer survival.

摘要

背景

原发性肾非霍奇金淋巴瘤(NHL)较为罕见。由于肾实质没有淋巴管,这一实体的存在一直受到质疑。本研究的目的是确定原发性肾NHL患者的临床表现、病理特征和疾病进程,并回顾关于这种不寻常的结外NHL的相关文献。

方法

回顾性分析梅奥诊所1976年至1992年诊断为肾NHL的所有病历。共识别出176例病例,其中5例符合原发性肾NHL的标准。详细回顾了临床、病理和影像学特征,这些是本报告的基础。

结果

5例原发性肾NHL患者的诊断时中位年龄为60岁(范围52 - 63岁),男女比例为2:3。所有患者均以侧腹痛为首发表现。仅1例患者尿常规异常。3例患者血清肌酐升高。肿瘤组织学类型为弥漫大细胞型4例,小无裂非伯基特型1例。5例均为B细胞免疫表型。所有患者均接受联合化疗。尽管该组患者的中位生存期仅为8个月,但有2例自治疗后完全缓解超过80个月。这2例患者的肉眼可见淋巴瘤均被完全切除,并接受了联合化疗和巩固放疗。

结论

原发性肾非霍奇金淋巴瘤确实存在。肉眼可见淋巴瘤被完全切除且接受联合化疗及巩固放疗的患者有较长的无病生存期。双侧肾受累或肾淋巴瘤未减瘤的患者生存期往往较差。

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