Trimeche Ajmi S, Marmouch H, Trabelsi A, Ben Abdelkrim S, Chadli Chaieb M, Ach K, Maaroufi A, Chaieb L
Department of Endocrinology, Farhat Hached Hospital, Sousse, Tunisia.
Pathologica. 2008 Dec;100(6):470-2.
The authors report the case of a 46-year-old woman who presented with a 4 month history of paroxystic and recent hypertension accompagned by headaches, tachycardia and sweating. The patient had decreased appetite with epigastric discomfort and abdominal distension. Physical examination was initially normal with mainly normal tension and no abdominal or lombar mass in palpation. While hospitalised, she developed paroxystic crisis of flush, headaches and hypertension of 190/100 mmHg. Biological findings revealed hypokaliemia and normal kaliuria on 3 day samples, with normal glycaemia and normal creatininaemia. Hormonal investigation revealed elevated metanephrines (3 mg/24 hours). Magnetic resonance imaging showed an 11 cm x 8.5 cm retroperitoneal mass with an enhanced signal in T2, a hypotrophic non-functional left kidney and no adrenal adenoma. Clinical and hormonal features suggested a diagnosis of pheochromocytoma. After preoperative medication, open excision, including left radical nephrectomy and adrenalectomy, normalized the catecholamine urinary level, resolved hypokalemia, and improved hypertension. Pathologic examination revealed a well-differentiated liposarcoma, without any pheochromocytoma component, and left adrenal hyperplasia. The tumour cells were immunonegative for chromogranin A. No metastatic lesion was identified by thoraco-abdominal computed tomography.
作者报告了一例46岁女性病例，该患者有4个月的阵发性及近期高血压病史，伴有头痛、心动过速和出汗。患者食欲减退，伴有上腹部不适和腹胀。体格检查最初正常，血压基本正常，触诊未发现腹部或腰部肿块。住院期间，她出现了面部潮红、头痛和血压达190/100 mmHg的阵发性危象。实验室检查结果显示3天样本存在低钾血症且尿钾正常，血糖和血肌酐正常。激素检查显示间甲肾上腺素升高（3 mg/24小时）。磁共振成像显示一个11 cm×8.5 cm的腹膜后肿块，T2加权像信号增强，左肾萎缩无功能，未发现肾上腺腺瘤。临床和激素特征提示诊断为嗜铬细胞瘤。术前用药后，行开放性切除术，包括左肾根治性切除术和肾上腺切除术，使尿儿茶酚胺水平恢复正常，纠正了低钾血症，并改善了高血压。病理检查显示为高分化脂肪肉瘤，无任何嗜铬细胞瘤成分，左肾上腺增生。肿瘤细胞嗜铬粒蛋白A免疫阴性。胸腹部计算机断层扫描未发现转移病灶。
