Misiani R, Sonzogni A, Poletti E M, Cassinelli G, Gualandris L, Pericotti S, Ghislandi R, Agazzi R, Belair M F, Bruneval P
Division of Nephrology and Dialysis, Ospedali Riuniti di Bergamo, Italy.
Am J Kidney Dis. 1994 Jul;24(1):83-8. doi: 10.1016/s0272-6386(12)80164-8.
We report a case of renin-producing leiomyosarcoma associated with the hyponatremic hypertensive syndrome and nephrotic-range proteinuria. Extremely high levels of active renin and, to a greater extent, of prorenin were found in plasma and tumor tissue. Immunohistochemical and in situ hybridization studies demonstrated that the neoplastic cells were the source of renin production. The hyponatremic hypertensive syndrome and proteinuria promptly responded to treatment with angiotensin-converting enzyme inhibitors, suggesting an angiotensin II dependency of these disorders. After removal of the leiomyosarcoma, plasma concentration of active renin, but not of prorenin, normalized and the hypertension, proteinuria, and electrolyte abnormalities disappeared. However, 5 months after operation, the patient presented once again with hypertension, hypokalemia, proteinuria, and markedly increased plasma levels of both active renin and prorenin that heralded the relapse of neoplastic disease.
我们报告一例产生肾素的平滑肌肉瘤，其与低钠血症性高血压综合征及肾病范围蛋白尿相关。在血浆和肿瘤组织中发现活性肾素水平极高，且在更大程度上，前肾素水平也极高。免疫组织化学和原位杂交研究表明，肿瘤细胞是肾素产生的来源。低钠血症性高血压综合征和蛋白尿对血管紧张素转换酶抑制剂治疗迅速产生反应，提示这些病症对血管紧张素 II 有依赖性。切除平滑肌肉瘤后，活性肾素的血浆浓度恢复正常，但前肾素的血浆浓度未恢复正常，高血压、蛋白尿和电解质异常消失。然而，术后 5 个月，患者再次出现高血压、低钾血症、蛋白尿，且活性肾素和前肾素的血浆水平均显著升高，预示肿瘤疾病复发。
