Pekic Sandra, Damjanovic Svetozar, Djurovic Marina, Doknic Mirjana, Petakov Milan, Miljic Dragana, Milovanovic Zorka, Kovacs Kalman, Popovic Vera
Neuroendocrine Unit, Institute of Endocrinology, University Clinical Center, Belgrade, Serbia.
Endocrine. 2004 Jun;24(1):99-103. doi: 10.1385/ENDO:24:1:099.
We report the case of a 52-yr-old man with a mass in the area of the left adrenal. The clinical features, MIBG uptake, and elevated urinary dopamine levels suggested the diagnosis of pheochromocytoma. He presented with unstable hypertension, tachycardia, weight loss, and the "inflammatory syndrome" (fever, leukocytosis, and high sedimentation rate). Clinical findings, preoperative radiographic (sonography, CT scan, [131I]MIBG scintigraphy), and endocrine evaluations (elevated 24-h urinary dopamine) were suggestive of a dopamine-secreting adrenal tumor. The mass was resected and on histologic examination showed the characteristic features of a malignant fibrous histiocytoma (MFH). The tumor cells were immunopositive for neuron-specific enolase (NSE), vimentin, CD-68, S-100, desmin, and immunonegative for chromogranin A, synaptophysin, neurofilament protein, and low-molecular-weight keratin, indicating that this tumor was not able to synthesize catecholamines. The prolonged retention of the tracer (MIBG) was interpreted as a consequence of obstructive hydronephrosis, while elevated urinary dopamine levels were assumed to be due to compression of the renal vessels by the large retroperitoneal mass.
我们报告了一例52岁男性患者，其左肾上腺区域有一肿块。临床特征、间碘苄胍（MIBG）摄取及尿多巴胺水平升高提示诊断为嗜铬细胞瘤。他表现为不稳定型高血压、心动过速、体重减轻及“炎症综合征”（发热、白细胞增多和血沉加快）。临床检查结果、术前影像学检查（超声、CT扫描、[131I]MIBG闪烁显像）及内分泌评估（24小时尿多巴胺升高）提示为分泌多巴胺的肾上腺肿瘤。该肿块被切除，组织学检查显示为恶性纤维组织细胞瘤（MFH）的特征性表现。肿瘤细胞对神经元特异性烯醇化酶（NSE）、波形蛋白、CD-68、S-100、结蛋白免疫阳性，对嗜铬粒蛋白A、突触素、神经丝蛋白和低分子量角蛋白免疫阴性，表明该肿瘤不能合成儿茶酚胺。示踪剂（MIBG）的长时间滞留被解释为梗阻性肾积水的结果，而尿多巴胺水平升高被认为是由于巨大的腹膜后肿块压迫肾血管所致。
