原发性肾上腺非霍奇金淋巴瘤七例报告

Primary adrenal non-Hodgkin＇ s iymphoma： report of 7 cases

目的探讨原发性肾上腺非霍奇金淋巴瘤的临床特点和诊治方法。方法以肾上腺肿瘤为首发表现的淋巴瘤患者7例。男5例，女2例。年龄33～62岁，平均48岁。单侧2例、双侧5例。体检发现2例；低热、乏力伴体质量下降3～4个月2例；睾丸肿大2个月1例；单侧腰痛伴脾肿大2例。7例血清乳酸脱氢酶367～568u／L（正常值100～245U／L），8。微球蛋白5．9～6．3mg／L（正常值〈2．4mg／L）。CT检查示肾上腺肿瘤直径6．3～13．0cm，呈不规则肿块，境界不清、密度不均、增强后肿瘤轻度强化，未见出血和钙化。结果术前误诊4例，分别误诊为皮质腺癌2例，肾上腺转移瘤、嗜铬细胞瘤各1例。行肾上腺肿瘤切除4例，其中2例行同侧肾切除，穿刺活检证实2例，睾丸活检证实1例。病理诊断为非霍奇金淋巴瘤，T细胞型1例、B细胞型6例。免疫组化T细胞型CD3、CD45-RO阳性、B细胞型L26、CD79a阳性。术后4例接受化疗，1例随访2年健在，余3例分别生存2、6、20个月后死亡；穿刺活检和睾丸活检3例接受放化疗，分别生存19、32、38个月后死亡。结论以肾上腺肿瘤为首发表现的淋巴瘤临床表现缺乏特异性，术前诊断困难，肿瘤局部病变广泛，影像学表现形态不规则。原发性肾上腺淋巴瘤恶性度较高，预后差，治疗上主要采用手术联合放化疗。

Objective To discuss the diagnosis and treatment of primary adrenal lymphoma. Methods The clinical data of 7 adrenal primary lymphoma cases were retrospectively analyzed. Five cases were male,2 were female. Age ranged from 33 to 62 years,mean 48 years. Two cases presented with unilateral and 5 cases with bilateral masses. Two cases were found by regular health examination. Two cases had fever and weakness, with body weight loss for 3-4 months. One case had enlarged testis for 1 month. Two cases had lumbar pain accompanied by enlarged spleen. Abdominal ultrasonography and CT showed adrenal neoplasms. All 7 cases had elevated serum lactate dehydrogenase （367-568 U/L, normal range 100-245 U/L） and 132 microglobulin （5.9-6.3 mg/L, normal range 2.4 mg/L）. The CT showed irregular,inhomogeneous adrenal mass which was mildly enhanced. Results Four of the 7 patients were misdiagnosed before operation. Two patients were diagnosed as adrenal lymphoma by biopsy. One patient was diagnosed by testicular biopsy. One was T cell non- Hodgkin＇s lymphoma. Six cases were diagnosed as diffuse large B cell non-Hodgkin＇s tymphoma by pathology. Immunohistochemically,the tumor cells were positive for CD3 ,CD45-RO, L26 and CD79 a. Four patients had their adrenal mass removed and received chemotherapy afterwards. As follow up of 2 years, 1 patient had no evidence of recurrence. Three patients died after 2,6,20 months after operation. Three cases took chemotherapy and radiation therapy after diagnosed. They died 19,32,38 months during follow up. Conclusions Because adrenal mass as the primary representation of primary adrenal lymphoma has no characteristic clinical appearance, diagnosis could not be made preopera tively. The principal treatment consists of adrenalectomy and adjuvant combination chemotherapy.