Department of Endocrinology, Metabolism, Rheumatology and Nephrology, Faculty of Medicine, Oita University, Yufu City, Oita, 879-5593, Japan.
Department of Diagnostic Pathology, Faculty of Medicine, Oita University, Yufu City, Oita, 879-5593, Japan.
BMC Endocr Disord. 2023 Feb 16;23(1):44. doi: 10.1186/s12902-023-01297-3.
Primary aldosteronism (PA) is a common cause of secondary hypertension, whereas pheochromocytoma is a rare cause of it. Thus, concomitant PA and pheochromocytoma is a very rare condition.
A 52-year-old woman was admitted to our hospital with suspected PA based on the presence of hypertension, spontaneous hypokalemia, and a high aldosterone-to-renin ratio. She had no catecholamine excess symptoms other than hypertension. Abdominal computed tomography (CT) showed a right lipid-rich adrenal mass and a left lipid-poor adrenal mass. PA was diagnosed by the captopril challenge test. The 24-h urinary fractionated metanephrines were slightly elevated. Adrenal vein sampling (AVS) confirmed that the right adrenal gland was responsible for aldosterone hypersecretion. Medical therapy with eplerenone was started because the patient refused surgery. Five years later, she requested surgery for PA. The second AVS confirmed right unilateral hyperaldosteronism, as expected. Repeated abdominal CT showed the enlargement of the left adrenal mass. The 24-h urinary fractionated metanephrines had risen to the diagnostic level. I- metaiodobenzylguanidine (MIBG) scintigraphy showed a marked tracer uptake in the left adrenal mass with no metastatic lesion. After preoperative management with α-blockade, laparoscopic left partial adrenalectomy was performed. Immunohistochemical examination of the tumor showed chromogranin A positivity leading to the diagnosis of left pheochromocytoma.
We report an extremely rare case of concomitant unilateral PA and contralateral pheochromocytoma. When diagnosing unilateral PA by AVS, especially in cases with a lipid-poor adrenal mass, clinicians should rule out the possibility of the presence of pheochromocytoma before proceeding to undergo unilateral adrenalectomy. Although there is no standard treatment for this rare condition, it is essential to select personalized treatment from the perspective of conserving the adrenal gland.
原发性醛固酮增多症(PA)是继发性高血压的常见病因,而嗜铬细胞瘤则是其罕见病因。因此,同时患有 PA 和嗜铬细胞瘤是一种非常罕见的情况。
一名 52 岁女性因存在高血压、自发性低钾血症和高醛固酮-肾素比值而被怀疑患有 PA 而入院。除高血压外,她没有儿茶酚胺过多的症状。腹部计算机断层扫描(CT)显示右侧富脂肾上腺肿块和左侧贫脂肾上腺肿块。卡托普利激发试验诊断为 PA。24 小时尿分馏间甲肾上腺素略有升高。肾上腺静脉取样(AVS)证实右侧肾上腺负责醛固酮分泌过多。由于患者拒绝手术,开始给予螺内酯进行药物治疗。五年后,她因 PA 要求手术。第二次 AVS 证实右侧单侧醛固酮增多症,正如预期的那样。反复腹部 CT 显示左侧肾上腺肿块增大。24 小时尿分馏间甲肾上腺素已升高至诊断水平。碘-间位碘苄胍(MIBG)闪烁显像显示左侧肾上腺肿块有明显示踪剂摄取,无转移病灶。在进行术前α受体阻断治疗后,进行了腹腔镜下左侧部分肾上腺切除术。肿瘤的免疫组化检查显示嗜铬粒蛋白 A 阳性,导致诊断为左侧嗜铬细胞瘤。
我们报告了一例极其罕见的同时患有单侧 PA 和对侧嗜铬细胞瘤的病例。当通过 AVS 诊断单侧 PA 时,特别是在存在贫脂肾上腺肿块的情况下,临床医生在进行单侧肾上腺切除术之前,应排除存在嗜铬细胞瘤的可能性。尽管这种罕见情况没有标准的治疗方法,但从保留肾上腺的角度出发,选择个性化的治疗方法至关重要。