Endocrinol Metab.  2015 Dec;30(4):607-613. 10.3803/EnM.2015.30.4.607.

Bilateral Adrenocortical Masses Producing Aldosterone and Cortisol Independently

• Lee SE
• Kim JH
• Lee YB
• Seok H
• Shin IS
• Eun YH
• Kim JH
• Oh YL

• KMID: 2169672
• DOI: http://doi.org/10.3803/EnM.2015.30.4.607

Abstract

A 31-year-old woman was referred to our hospital with symptoms of hypertension and bilateral adrenocortical masses with no feature of Cushing syndrome. The serum aldosterone/renin ratio was elevated and the saline loading test showed no suppression of the plasma aldosterone level, consistent with a diagnosis of primary hyperaldosteronism. Overnight and low-dose dexamethasone suppression tests showed no suppression of serum cortisol, indicating a secondary diagnosis of subclinical Cushing syndrome. Adrenal vein sampling during the low-dose dexamethasone suppression test demonstrated excess secretion of cortisol from the left adrenal mass. A partial right adrenalectomy was performed, resulting in normalization of blood pressure, hypokalemia, and high aldosterone level, implying that the right adrenal mass was the main cause of the hyperaldosteronism. A total adrenalectomy for the left adrenal mass was later performed, resulting in a normalization of cortisol level. The final diagnosis was bilateral adrenocortical adenomas, which were secreting aldosterone and cortisol independently. This case is the first report of a concurrent cortisol-producing left adrenal adenoma and an aldosterone-producing right adrenal adenoma in Korea, as demonstrated by adrenal vein sampling and sequential removal of adrenal masses.

Keyword

Adrenocortical adenoma; Hyperaldosteronism; Cushing syndrome

MeSH Terms

Adenoma
Adrenalectomy
Adrenocortical Adenoma
Adult
Aldosterone*
Blood Pressure
Cushing Syndrome
Dexamethasone
Diagnosis
Female
Humans
Hydrocortisone*
Hyperaldosteronism
Hypertension
Hypokalemia
Korea
Plasma
Veins
Aldosterone
Dexamethasone
Hydrocortisone

Figure

• 

  Fig. 1 Adrenal Images. (A, B) Adrenal computed tomography. Axial images showed bilateral adrenal masses (arrows), including a 1.6-cm mass on the right adrenal gland (A) and a 2-cm mass on the left (B). (C) Adrenal NP-59 scan demonstrated focally increased radioactive uptakes in the bilateral adrenal gland areas (arrow, arrowhead), especially on the left side (arrowhead).

• 

  Fig. 2 Adrenalectomy specimens (gross and microscopic findings). (A) The right adrenal mass was a 1.4×1.4×1.2-cm ovoid, bright yellow, and well-circumscribed mass arising from the cortex. (B) Microscopically, the right adrenal mass consisted primarily of clear cells (H&E stain, ×200). (C) The left adrenal mass was brown, solid in appearance, and encapsulated. (D) The left adrenal mass was composed of both clear cells and eosinophilic compact cells (H&E stain, ×200).

Reference

1. Young WF. Primary aldosteronism: renaissance of a syndrome. Clin Endocrinol (Oxf). 2007; 66:607–618.

2. Kim SW. Primary aldosteronism. Korean J Med. 2012; 82:396–402.

3. De Leo M, Cozzolino A, Colao A, Pivonello R. Subclinical Cushing's syndrome. Best Pract Res Clin Endocrinol Metab. 2012; 26:497–505.

4. Terzolo M, Pia A, Reimondo G. Subclinical Cushing's syndrome: definition and management. Clin Endocrinol (Oxf). 2012; 76:12–18.

5. Morelli V, Reimondo G, Giordano R, Della Casa S, Policola C, Palmieri S, et al. Long-term follow-up in adrenal incidentalomas: an Italian multicenter study. J Clin Endocrinol Metab. 2014; 99:827–834.

6. Kempers MJ, Lenders JW, van Outheusden L, van der Wilt GJ, Schultze Kool LJ, Hermus AR, et al. Systematic review: diagnostic procedures to differentiate unilateral from bilateral adrenal abnormality in primary aldosteronism. Ann Intern Med. 2009; 151:329–337.

7. Levinson PD, Zadik Z, Hamilton BP, Mersey JH, White RI, Kowarski AA. Adrenal vein epinephrine levels: a useful aid in venous sampling for primary aldosteronism. Ann Intern Med. 1982; 97:690–693.

8. Lee JS, Kang MY, Kim SW, Shin CS, Kim SY, Chung JW. The clinical implication and problems of adrenal vein sampling in patients with primary aldosteronism. J Korean Endocr Soc. 2007; 22:428–435.

9. Young WF Jr, du Plessis H, Thompson GB, Grant CS, Farley DR, Richards ML, et al. The clinical conundrum of corticotropin-independent autonomous cortisol secretion in patients with bilateral adrenal masses. World J Surg. 2008; 32:856–862.

10. Ku EJ, Hong AR, Kim YA, Bae JH, Chang MS, Kim SW. Adrenocorticotropic hormone-independent cushing syndrome with bilateral cortisol-secreting adenomas. Endocrinol Metab (Seoul). 2013; 28:133–137.

11. Cho YY, Suh S, Joung JY, Jeong H, Je D, Yoo H, et al. Clinical characteristics and follow-up of Korean patients with adrenal incidentalomas. Korean J Intern Med. 2013; 28:557–564.

12. Wu VC, Chueh SC, Chang HW, Lin WC, Liu KL, Li HY, et al. Bilateral aldosterone-producing adenomas: differentiation from bilateral adrenal hyperplasia. QJM. 2008; 101:13–22.

13. Fujimoto K, Honjo S, Tatsuoka H, Hamamoto Y, Kawasaki Y, Matsuoka A, et al. Primary aldosteronism associated with subclinical Cushing syndrome. J Endocrinol Invest. 2013; 36:564–567.

14. Hiraishi K, Yoshimoto T, Tsuchiya K, Minami I, Doi M, Izumiyama H, et al. Clinicopathological features of primary aldosteronism associated with subclinical Cushing's syndrome. Endocr J. 2011; 58:543–551.

15. Oki K, Yamane K, Sakashita Y, Kamei N, Watanabe H, Toyota N, et al. Primary aldosteronism and hypercortisolism due to bilateral functioning adrenocortical adenomas. Clin Exp Nephrol. 2008; 12:382–387.

16. Choi SE, Kim YC, Noh DY, Youn YK, Oh SK. A case of bilateral adrenal cortical adenomas causing Cushing's syndrome and primary aldosteronism. J Korean Surg Soc. 1998; 55:769–774.

17. Nagae A, Murakami E, Hiwada K, Kubota O, Takada Y, Ohmori T. Primary aldosteronism with cortisol overproduction from bilateral multiple adrenal adenomas. Jpn J Med. 1991; 30:26–31.

18. Okura T, Miyoshi K, Watanabe S, Kurata M, Irita J, Manabe S, et al. Coexistence of three distinct adrenal tumors in the same adrenal gland in a patient with primary aldosteronism and preclinical Cushing's syndrome. Clin Exp Nephrol. 2006; 10:127–130.

19. Onoda N, Ishikawa T, Nishio K, Tahara H, Inaba M, Wakasa K, et al. Cushing's syndrome by left adrenocortical adenoma synchronously associated with primary aldosteronism by right adrenocortical adenoma: report of a case. Endocr J. 2009; 56:495–502.

20. Lack EE, Wieneke J. Chapter 19, Tumors of the adrenal gland. Diagnostic histopathology of tumors. 4th ed. Philadelphia: Elsevier Health Sciences;2013. p. 1294–1325.