Steroid Profile in an Adrenocortical Carcinoma Producing Aldosterone

Abstract

We report a rare case of primary aldosteronism due to an adrenocortical carcinoma. A 61-year-old woman with a history of hypertension and hypokalemia was referred for evaluation of a 4.2 cm measuring adrenal mass without secondary signs of malignancy. Endocrinological testing was consistent with primary aldosteronism. The patient underwent surgical resection of the adrenal mass; histology revealed an adrenocortical carcinoma. Postoperatively blood pressure, serum potassium, and aldosterone returned to normal. Four months after adrenalectomy, the patient presented again with hypokalemic hypertension and was found to have metastatic disease. Endocrinological investigation revealed primary aldosteronism and subclinical autonomous glucocorticoid hypersecretion. Careful hormonal investigation should be obtained in patients with adrenal masses causing excessive aldosterone secretion. In uncertain cases of primary aldosteronism, we would suggest to measure 18-hydroxycortisol levels, as excessive amounts may indicate adrenocortical carcinoma.

Key words

Adrenocortical carcinoma - primary aldosteronism - mineralocorticoid hypertension - 18-hydroxycortisol

References

• 1 Allolio B, Fassnacht M, Arlt W. Maligne Tumoren der Nebennierenrinde.  Internist. 2002;  43 186-195
• 2 Arteaga E, Biglieri E G, Kater C E, Lopez J M, Schambelan M. Aldosterone-producing adrenocortical carcinoma. Preoperative recognition and course in three cases.  Ann Intern Med. 1984;  101 316-321
• 3 Farge D, Chatellier G, Pagny J Y, Jeunemaitre X, Plouin P F, Corvol P. Isolated clinical syndrome of primary aldosteronism in four patients with adrenocortical carcinoma.  Am J Med. 1987;  83 635-640
• 4 Gomez-Sanchez C E, Clore J N, Estep H L, Watlington C O. Effect of chronic adrenocorticotropin stimulation on the excretion of 18-hydroxycortisol and 18-oxocortisol.  J Clin Endocrinol Metab. 1988;  67 322-326
• 23 Grumbach M M, Biller B M, Braunstein G D, Campbell K K, Carney J A, Godley P A, Harris E L, Lee J K, Oertel Y C, Posner M C, Schlechte J A, Wieand H S. Management of the clinically inapparent adrenal mass (“incidentaloma”).  Ann Intern Med. 2003;  138 424-429
• 5 Harrison L E, Gaudin P B, Brennan M F. Pathologic features of prognostic significance for adrenocortical carcinoma after curative resection.  Arch Surg. 1999;  134 181-185
• 6 Henry J F, Sebag F, Iacobone M, Mirallie E. Results of laparoscopic adrenalectomy for large and potentially malignant tumors.  World J Surg. 2002;  26 1043-1047
• 7 Hisamatsu H, Sakai H, Irie J, Maeda K, Kanetake H. Adrenocortical carcinoma with primary aldosteronism associated with Cushing syndrome during recurrence.  BJU Int. 2002;  90 971-972
• 8 Jamieson A, Ingram M C, Inglis G C, Davies E, Fraser R, Connell J M. Altered 11 beta-hydroxylase activity in glucocorticoid-suppressible hyperaldosteronism.  J Clin Endocrinol Metab. 1996;  81 2298-2302
• 9 Kopf D, Goretzki P E, Lehnert H. Clinical management of malignant adrenal tumors.  J Cancer Res Clin Oncol. 2001;  127 143-155
• 10 Kurtulmus N, Yarman S, Azizlerli H, Kapran Y. Co-secretion of aldosterone and cortisol by an adrenocortical carcinoma.  Horm Res. 2004;  62 67-70
• 11 MacGillivray D C, Whalen G F, Malchoff C D, Oppenheim D S, Shichman S J. Laparoscopic resection of large adrenal tumors.  Ann Surg Oncol. 2002;  9 480-485
• 12 Mosso L, Gomez-Sanchez C E, Foecking M F, Fardella C. Serum 18-hydroxycortisol in primary aldosteronism, hypertension, and normotensives.  Hypertension. 2001;  38 688-691
• 13 Saeger W. Nebennierenrinde. Remmele W Pathologie. 2nd ed. Berlin, Heidelberg; Springer-Verlag 1997: 641-642
• 14 Saha P K, Ura T, Suzu H, Yamashita S, Yushita Y, Kanetake H, Saito Y, Tsuchiyama H, Shigematsu K, Hukuzi S. et al . A case of deoxycorticosterone-producing benign adrenocortical tumor.  Urol Int. 1990;  45 367-369
• 15 Schulick R D, Brennan M F. Long-term survival after complete resection and repeat resection in patients with adrenocortical carcinoma.  Ann Surg Oncol. 1999;  6 719-726
• 16 Smith C D, Weber C J, Amerson J R. Laparoscopic adrenalectomy: new gold standard.  World J Surg. 1999;  23 389-396
• 17 Sturgeon C, Kebebew E. Laparoscopic adrenalectomy for malignancy.  Surg Clin North Am. 2004;  84 755-774
• 18 Ulick S, Chu M D, Land M. Biosynthesis of 18-oxocortisol by aldosterone-producing adrenal tissue.  J Biol Chem. 1983;  258 5498-5502
• 19 Vecsei P, Abdelhamid S, Mittelstädt G V, Lichtwald K, Haack D, Lewicka S. Aldosterone metabolites and possible aldosterone precursors in hypertension.  J Steroid Biochem. 1983;  19 345-351
• 20 Weiss L M. Comparative histologic study of 43 metastasizing and nonmetastasizing adrenocortical tumors.  Am J Surg Pathol. 1984;  8 163-169
• 21 Weiss L M, Medeiros L J, Vickery Jr A L. Pathologic features of prognostic significance in adrenocortical carcinoma.  Am J Surg Pathol. 1989;  13 202-206
• 22 Young Jr W F. Minireview: primary aldosteronism - changing concepts in diagnosis and treatment.  Endocrinology. 2003;  144 2208-2213