Department of Forensic Medicine, Kyungpook National University School of Medicine, Daegu, Republic of Korea.
Medicine (Baltimore). 2022 Nov 25;101(47):e31403. doi: 10.1097/MD.0000000000031403.
Adrenal mixed corticomedullary tumors (MCMTs) are single tumor masses composed of an intimately admixed population of both adrenal cortical cells and medullary components. Most medullary tumor components are pheochromocytomas; however, MCMTs composed of ganglioneuroma and adrenal cortical adenoma are extremely rare. The current case is a rare case of adrenal MCMT composed of ganglioneuroma and adrenal cortical adenoma with primary aldosteronism.
A 49-year-old male was admitted because of hypokalemia and an adrenal mass. He was diagnosed with hypertension in his 20s and was taking blood pressure medications.
Plasma aldosterone concentration 376.5 pg/dL (normal 37.8233.0 pg/mL) and potassium 2.8 mmol/L (normal 3.44.9 mmol/L) were detected. The aldosterone-to-renin ratio [the ratio of plasma aldosterone concentration (ng/dL) to PRA (ng/mL/hour)] was 38. The saline loading test showed that serum aldosterone (49.4 ng/dL) was not suppressed, compared with the basal level (28.4 ng/dL). The adrenal venous sampling test showed that the aldosterone level markedly increased to 1521.2 pg/mL. Abdominal computed tomography revealed an enlarged relatively well-circumscribed multinodular mass (35 × 13 × 30 mm) in the right adrenal gland.
Laparoscopic right adrenalectomy was performed under the clinical diagnosis of a functioning adrenal cortical adenoma.
After laparoscopic right adrenalectomy, the serum aldosterone and renin levels returned to normal. The patient maintained a normal aldosterone level without recurrence for 16 months.
Adrenal MCMTs of the ganglioneuroma and cortical adenomas in the ipsilateral adrenal gland are extremely rare. Adrenal MCMTs exhibit benign clinical behavior, with no metastasis or death due to the tumor. With the development of diagnostic imaging technology, it is possible to identify mixed tumors. However, surgical resection of adrenal gland is a common treatment and a final diagnosis should be made based on the pathological results after surgery. Because this is to rule out the occurrence of rare malignant tumors and confirm the pattern of mixed tumors.
肾上腺皮质-髓质混合肿瘤(MCMT)是由肾上腺皮质细胞和髓质成分的紧密混合而成的单一肿瘤。大多数髓质肿瘤成分是嗜铬细胞瘤;然而,由神经节细胞瘤和肾上腺皮质腺瘤组成的 MCMT 极为罕见。目前的病例是一例由神经节细胞瘤和肾上腺皮质腺瘤组成的罕见的肾上腺 MCMT,伴原发性醛固酮增多症。
一名 49 岁男性因低钾血症和肾上腺肿块入院。他在 20 多岁时被诊断为高血压,并一直在服用降压药物。
检测到血浆醛固酮浓度 376.5 pg/dL(正常 37.8233.0 pg/mL)和钾 2.8 mmol/L(正常 3.44.9 mmol/L)。醛固酮-肾素比值[血浆醛固酮浓度(ng/dL)与 PRA(ng/mL/hour)的比值]为 38。盐水负荷试验显示,血清醛固酮(49.4 pg/dL)与基础水平(28.4 pg/dL)相比未被抑制。肾上腺静脉取样试验显示,醛固酮水平显著升高至 1521.2 pg/mL。腹部计算机断层扫描显示右侧肾上腺有一个增大的相对边界清楚的多结节肿块(35×13×30 mm)。
在临床诊断为功能性肾上腺皮质腺瘤的情况下,行腹腔镜右侧肾上腺切除术。
腹腔镜右侧肾上腺切除术后,血清醛固酮和肾素水平恢复正常。患者在 16 个月内保持正常的醛固酮水平,无复发。
同侧肾上腺的神经节细胞瘤和皮质腺瘤的肾上腺 MCMT 极为罕见。肾上腺 MCMT 表现出良性的临床行为,无转移或因肿瘤死亡。随着诊断成像技术的发展,混合肿瘤是可以识别的。然而,肾上腺切除术是一种常见的治疗方法,最终诊断应基于手术后的病理结果。因为这是为了排除罕见的恶性肿瘤的发生,并确认混合肿瘤的模式。