Kose Fatih, Sakalli Hakan, Mertsoylu Huseyin, Sezer Ahmet, Kocer Emrah, Tokmak Naime, Kilinc Ferhat, Ozyilkan Ozgur
Department of Medical Oncology, Faculty of Medicine, Baskent University, Ankara, Turkey.
Onkologie. 2009 Apr;32(4):200-2. doi: 10.1159/000203331. Epub 2009 Mar 13.
Although secondary renal involvement from systemic lymphoma is very frequent, primary renal lymphoma is a rare entity. It is characterized by aggressive histopathology, very early extra-renal infiltration and poor prognosis.
Here, we report 4 cases of primary renal lymphoma presenting with unilateral renal masses, which after radiological and clinical examination were assumed to be renal cell carcinoma. 3 patients were diagnosed with Non-Hodgkin's lymphoma by nephrectomy and one patient was diagnosed by open renal biopsy. Histopathological subtypes were diffuse large B cell lymphoma in 2 cases and non-Hodgkin's lymphoma of small B cell type in the others. While 3 of the patients were treated with systemic chemotherapy, the fourth patient refused chemotherapy. 2 patients (no. 2 and 3) were still in complete remission and were followed regularly in the second and first year after diagnosis, respectively.
Since it is difficult to diagnose primary renal lymphoma, most patients with this kind of tumor undergo radical nephrectomy, and diagnosis of primary renal lymphoma is delayed. The authors believe that both the delayed diagnosis due to anatomical difficulties and the histological aggressive characteristics of this disease are equally responsible for the poor outcome in the case of primary renal lymphoma.
尽管系统性淋巴瘤引起的继发性肾受累非常常见,但原发性肾淋巴瘤是一种罕见的疾病。其特点是组织病理学侵袭性强、肾外浸润非常早且预后差。
在此,我们报告4例表现为单侧肾肿块的原发性肾淋巴瘤病例,经影像学和临床检查后被误诊为肾细胞癌。3例患者通过肾切除术诊断为非霍奇金淋巴瘤,1例患者通过开放性肾活检确诊。组织病理学亚型中,2例为弥漫性大B细胞淋巴瘤,其余为小B细胞型非霍奇金淋巴瘤。3例患者接受了全身化疗,第4例患者拒绝化疗。2例患者(第2例和第3例)仍处于完全缓解状态,分别在诊断后的第二年和第一年接受定期随访。
由于原发性肾淋巴瘤难以诊断,大多数此类肿瘤患者接受了根治性肾切除术,导致原发性肾淋巴瘤的诊断延迟。作者认为,解剖学困难导致的诊断延迟以及该疾病的组织学侵袭性特征,同样是原发性肾淋巴瘤预后不良的原因。