A case of bilateral aldosterone-producing adenomas differentiated by segmental adrenal venous sampling for bilateral adrenal sparing surgery

Abstract

Primary aldosteronism due to unilateral aldosterone-producing adenoma (APA) is a surgically curable form of hypertension. Bilateral APA can also be surgically curable in theory but few successful cases can be found in the literature. It has been reported that even using successful adrenal venous sampling (AVS) via bilateral adrenal central veins, it is extremely difficult to differentiate bilateral APA from bilateral idiopathic hyperaldosteronism (IHA) harbouring computed tomography (CT)-detectable bilateral adrenocortical nodules. We report a case of bilateral APA diagnosed by segmental AVS (S-AVS) and blood sampling via intra-adrenal first-degree tributary veins to localize the sites of intra-adrenal hormone production. A 36-year-old man with marked long-standing hypertension was referred to us with a clinical diagnosis of bilateral APA. He had typical clinical and laboratory profiles of marked hypertension, hypokalaemia, elevated plasma aldosterone concentration (PAC) of 45.1 ng dl−1 and aldosterone renin activity ratio of 90.2 (ng dl−1 per ng ml−1 h−1), which was still high after 50 mg-captopril loading. CT revealed bilateral adrenocortical tumours of 10 and 12 mm in diameter on the right and left sides, respectively. S-AVS confirmed excess aldosterone secretion from a tumour segment vein and suppressed secretion from a non-tumour segment vein bilaterally, leading to the diagnosis of bilateral APA. The patient underwent simultaneous bilateral sparing adrenalectomy. Histopathological analysis of the resected adrenals together with decreased blood pressure and PAC of 5.2 ng dl−1 confirmed the removal of bilateral APA. S-AVS was reliable to differentiate bilateral APA from IHA by direct evaluation of intra-adrenal hormone production.

Somatic KCNJ5 mutation occurring early in adrenal development may cause a novel form of juvenile primary aldosteronism

“…Among these cAVS-success patients, blood sample collection from more than 2 TVs of each of adrenal was accomplished in 242 patients (98.3%). Recently, the Tohoku University group followed our ssAVS protocol (they call the protocol segmental adrenal venous sampling) and showed similar success rate and availability with ours, suggesting that the protocol is potentially available in high volume referral centers (Satoh et al, 2015a,b; Satani et al, 2016; Satoh et al, 2015a,b; Morimoto et al, 2016). …”

Scoring system for the diagnosis of bilateral primary aldosteronism in the outpatient setting before adrenal venous sampling

“…Although Satoh et al . also demonstrated the utility of segmental AVS and adrenal‐sparing surgery for bilateral APA with adrenal nodules on CT imaging, 7 points as per our new scoring system indicated BHA without adrenal nodules on CT imaging and that there are no indications of adrenal‐sparing surgery . Furthermore, another report for BHA diagnosis used LR of >4·0 for UHA diagnosis .…”

“…Multivariable logistic regression analysis for three variables -sparing surgery 22,23. Furthermore, another report for BHA diagnosis used LR of >4Á0 for UHA diagnosis.…”

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“…慶應義塾大学病院中央臨床検査部 3) 加藤 朋子 1) 藤村 真輝 1) 波床 朋信 1) 綾野 志保 1) 米光 新 1) 武呂 誠司 1) 天野 直子 2) 本間 桂子 3) 隠岐 尚吾 1) はじめに 17α水酸化酵素欠損症は、先天性副腎過形成症の成因 の一つとして、1966 年 Biglieri らにより初めて報告さ 血液検査において K 3.1mEq/L であり、尿中 K 排泄は 0.9g/ 日であった。内分泌学的検査において基礎値はコ ルチゾール 9.2μg/dl (正常値 5-25μg/dl) 、ACTH 113pg/ ml (正常値 7.2-63.3pg/ml) 、アルドステロン 230pg/ml (正常値 30-159pg/ml) 、レニン活性 0.4ng/ml/hr (正常 値 0.2-2.7ng/ml/hr) 、デヒドロエピアンドロステロン サ ル フ ェ ー ト (DHEA-S) 4μg/dl ( 正 常 値 53-342μg/dl) であった。低 K 血症、ACTH 及びアルドステロンの高 値、DHEA-S の低値を認め、両側副腎腫大を伴うこと から 17α水酸化酵素欠損症が疑われた。プレグネノロ ン 2.82ng/ml ( 正 常 値 0.1-1.0ng/ml) 、 プ ロ ゲ ス テ ロ ン 5.47ng/ml (正常値 0.2-1.4ng/ml) 、デオキシコルチコス テ ロ ン (DOC) 5.63ng/ml ( 正 常 値 0.08-0.28ng/ml) 、 コ ルチコステロン (B) 173ng/ml (正常値 0.38-8.42ng/ml) で あり、アルドステロン生合成過程の中間代謝産物の高値 を認めた。また、迅速 ACTH 負荷試験において ACTH 負 荷 後 DOC は 10.5ng/ml (60 分 ) 、B は 260ng/ml (30 分) へ上昇を認めた。17α-ヒドロキシプレグネノロン 0.61ng/ml (正常値 0.10-4.00ng/ml) 、17α-ヒドロキシプ ロゲステロン 0.3ng/ml (正常値 0.05-1.6ng/ml) 、11-デ オキシコルチゾール 0.57ng/ml (正常値 0.04-1.10ng/ml) であり、コルチゾール生合成過程の中間代謝産物は正 常範囲内であった。アンドロステンジオン＜ 0.10ng/ml (正常値 0.41-1.57ng/ml) 、エストラジオール＜ 15pg/ml (正常値 14.6-48.8ng/ml) 、テストステロン＜ 0.03ng/ml (正常値 2.01-7.50ng/ml) 、LH 19.2mIU/ml (正常値 1.7-8.6mIU/ml) 、FSH 72.0mIU/ml ( 正 常 値 1.5-12.4mIU/ ml) であり、性ステロイドの低値、ゴナドトロピンの高 値を認めた。尿中ステロイドプロフィルでは、プレグネ ノロン、プロゲステロン、DOC、B の代謝物の著明な 高値、アルドステロン、17α-ヒドロキシプロゲステロン、…”