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累及肾上腺和中枢神经系统(CNS)的非霍奇金淋巴瘤:化疗后的特殊病程。

Non Hodgkin's lymphoma involving the adrenal glands and the central nervous system (CNS): a particular evolution after chemotherapy.

作者信息

Vélayoudom F-L, Cardot-Bauters C, Decouvelaere A-V, Vlaeminck V, Bauters F, Wémeau J-L

机构信息

Department of Endocrinology, Marc Linquette Clinic, 6, rue du Pr. Laguesse, CHRU de Lille, 59037 Lille.

出版信息

Ann Endocrinol (Paris). 2005 Dec;66(6):527-31. doi: 10.1016/s0003-4266(05)82113-4.

Abstract

Adrenal lymphoma is extremely rare. The prognostic depends on involvement of other organs (such as the central nervous system) responsible for lower median survival. We report the case of a 51-year-old man with non Hodgkin's Diffuse Large B Cell Lymphoma (DLBCL) involving the central nervous system (CNS) and the adrenal glands simultaneously. The endocrine exploration revealed a partial adrenal insufficiency and ruled out a pheochromocytoma. Computerized tomographic (CT) scan directed needle biopsy of the adrenal gland allowed the diagnostic of non-Hodgkin lymphoma (NHL). CNS biopsies showed similar histopathologic lesions. After aggressive polychemotherapy and methotrexate intrathecal injection, a dissociated therapeutic response was observed with a decrease of the cerebral lesion and an increase of the adrenal mass. This result may be explained by the efficacy of corticosteroid therapy on cerebral edema. The prognosis was poor with tumor infiltration of the leptomeninges and death 16 months after diagnosis.

摘要

肾上腺淋巴瘤极为罕见。其预后取决于其他器官(如中枢神经系统)是否受累,受累会导致中位生存期降低。我们报告一例51岁男性,患有非霍奇金弥漫性大B细胞淋巴瘤(DLBCL),同时累及中枢神经系统(CNS)和肾上腺。内分泌检查显示部分肾上腺功能不全,并排除了嗜铬细胞瘤。计算机断层扫描(CT)引导下的肾上腺穿刺活检确诊为非霍奇金淋巴瘤(NHL)。中枢神经系统活检显示出相似的组织病理学病变。在积极进行多药化疗和鞘内注射甲氨蝶呤后,观察到分离的治疗反应,脑部病变减轻而肾上腺肿块增大。这一结果可能是由于皮质类固醇疗法对脑水肿有效。预后较差,软脑膜有肿瘤浸润,诊断后16个月死亡。

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