Sakai Kimiko, Asano Mai, Hamaguchi Masahide, Taniguchi Hidefumi, Ukimura Osamu, Fukui Michiaki
Department of Endocrinology and Metabolism, Graduate School of Medical Science, Kyoto Prefectural University of Medicine, Kyoto, Japan.
Department of Urology, Graduate School of Medical Science, Kyoto Prefectural University of Medicine, Kyoto, Japan.
Clin Med Insights Endocrinol Diabetes. 2023 Jan 16;16:11795514221148556. doi: 10.1177/11795514221148556. eCollection 2023.
Cushing's syndrome and pheochromocytomas (PCCs) are associated with endocrine hypertension. Cortisol-producing adrenal adenomas are a major cause of Cushing's syndrome. Simultaneous occurrence of cortisol-producing adrenal adenomas and PCCs is rare. Additionally, a PCC generally produces catecholamines in proportion to its size; therefore, micro-PCCs are rarely found in clinical practice. It is unknown whether micro-PCCs produce excess catecholamines during the pre-biochemical phase. Herein, we report the case of a 53-year-old woman who was referred to our hospital for further evaluation of left adrenal incidentaloma. She had been suffering from hypertension for 7 years. Endocrine tests indicated autonomous cortisol secretion, and she was diagnosed with cortisol-producing adrenal adenoma. A laparoscopic left adrenalectomy was performed. The final pathological examination revealed an adrenocortical adenoma measuring 26 × 24 mm. In addition, a micro-PCC measuring 3 × 2 mm was incidentally found near the cortisol-secreting adrenal adenoma in the ipsilateral adrenal gland. All catecholamine biosynthetic enzymes, tyrosine hydroxylase, aromatic l-amino acid decarboxylase, dopamine β-hydroxylase, and phenyl ethanolamine N-methyltransferase, were detected in this micro-PCC by immunohistochemical analyses. Although catecholamine levels were not biochemically elevated, the PCC expressed catecholamine biosynthetic enzymes. This is the first immunohistochemical report to show that a micro-PCC produces excess catecholamines in the pre-biochemical phase.
库欣综合征和嗜铬细胞瘤(PCCs)与内分泌性高血压相关。分泌皮质醇的肾上腺腺瘤是库欣综合征的主要病因。分泌皮质醇的肾上腺腺瘤与PCCs同时发生的情况较为罕见。此外,PCC通常根据其大小分泌相应比例的儿茶酚胺;因此,微小PCC在临床实践中很少被发现。目前尚不清楚微小PCC在生化前期是否会产生过量儿茶酚胺。在此,我们报告一例53岁女性病例,该患者因左肾上腺偶发瘤被转诊至我院作进一步评估。她患有高血压7年。内分泌检查提示皮质醇自主分泌,诊断为分泌皮质醇的肾上腺腺瘤。遂行腹腔镜下左肾上腺切除术。最终病理检查显示肾上腺皮质腺瘤大小为26×24mm。此外,在同侧肾上腺分泌皮质醇的肾上腺腺瘤附近偶然发现一个大小为3×2mm的微小PCC。通过免疫组化分析在这个微小PCC中检测到了所有儿茶酚胺生物合成酶,即酪氨酸羟化酶、芳香族L-氨基酸脱羧酶、多巴胺β-羟化酶和苯乙醇胺N-甲基转移酶。尽管儿茶酚胺水平在生化检查中未升高,但该PCC表达了儿茶酚胺生物合成酶。这是首例免疫组化报告显示微小PCC在生化前期产生过量儿茶酚胺。