Clinical Overview > Condition/ Syndrome > Adrenocortical adenoma

You are looking at 11 - 13 of 13 items

Tohru Eguchi Department of Internal Medicine, Uwajima City Hospital, 1-1 Gotenmachi, Uwajima, Ehime, 798-8510, Japan

Search for other papers by Tohru Eguchi in
Google Scholar
PubMed
Close
and
Shozo Miyauchi Department of Internal Medicine, Uwajima City Hospital, 1-1 Gotenmachi, Uwajima, Ehime, 798-8510, Japan

Search for other papers by Shozo Miyauchi in
Google Scholar
PubMed
Close

Summary

A 43-year-old Japanese woman was admitted to our hospital with weakness. Laboratory findings showed hypokalemia, hypocalcemia and elevation of the serum creatinine phosphokinase levels, but intact parathyroid hormone levels. Further evaluations suggested that she had primary aldosteronism (PA), secondary hyperparathyroidism and bilateral adrenal tumors. She was treated successfully by laparoscopic right adrenalectomy. This case not only serves to the diagnosis of bilateral adrenal tumors in which selective adrenal venous sampling (SAVS) proved to be useful, but also for physicians to be aware of secondary hyperparathyroidism and the risk of secondary osteoporosis caused by PA.

Learning points

  • The classic presenting signs of PA are hypertension and hypokalemia.

  • Hypokalemia can induce rhabdomyolysis.

  • PA causes secondary hyperparathyroidism.

  • Patients with PA have the risk of osteoporosis with secondary hyperparathyroidism.

  • SAVS is useful in bilateral adrenal tumors.

Open access
Sophie Comte-Perret Service of Endocrinology, Diabetology and Metabolism, Department of Medicine, CHUV-University Hospital, CH-1011 Lausanne, Switzerland

Search for other papers by Sophie Comte-Perret in
Google Scholar
PubMed
Close
,
Anne Zanchi Service of Endocrinology, Diabetology and Metabolism, Department of Medicine, CHUV-University Hospital, CH-1011 Lausanne, Switzerland

Search for other papers by Anne Zanchi in
Google Scholar
PubMed
Close
, and
Fulgencio Gomez Service of Endocrinology, Diabetology and Metabolism, Department of Medicine, CHUV-University Hospital, CH-1011 Lausanne, Switzerland

Search for other papers by Fulgencio Gomez in
Google Scholar
PubMed
Close

Summary

Medical therapy for Cushing's syndrome due to bilateral macronodular adrenal hyperplasia (BMAH) is generally administered for a limited time before surgery. Aberrant receptors antagonists show inconsistent efficacy in the long run to prevent adrenalectomy. We present a patient with BMAH, treated for 10 years with low doses of ketoconazole to control cortisol secretion. A 48-year-old woman presented with headaches and hypertension. Investigations showed the following: no clinical signs of Cushing's syndrome; enlarged lobulated adrenals; normal creatinine, potassium, and aldosterone; normal urinary aldosterone and metanephrines; elevated urinary free cortisol and steroid metabolites; and suppressed plasma renin activity and ACTH. A screening protocol for aberrant adrenal receptors failed to show any illegitimate hormone dependence. Ketoconazole caused rapid normalisation of cortisol and ACTH that persists over 10 years on treatment, while adrenals show no change in shape or size. Ketoconazole decreases cortisol in patients with Cushing's syndrome, and may prevent adrenal overgrowth. Steroid secretion in BMAH is inefficient as compared with normal adrenals or secreting tumours and can be controlled with low, well-tolerated doses of ketoconazole, as an alternative to surgery.

Learning points

  • Enlarged, macronodular adrenals are often incidentally found during the investigation of hypertension in patients harboring BMAH. Although laboratory findings include low ACTH and elevated cortisol, the majority of patients do not display cushingoid features.

  • Bilateral adrenalectomy, followed by life-long steroid replacement, is the usual treatment of this benign condition, and alternative medical therapy is sought. Therapy based on aberrant adrenal receptors gives disappointing results, and inhibitors of steroidogenesis are not always well tolerated.

  • However, ketoconazole at low, well-tolerated doses appeared appropriate to control adrenal steroid secretion indefinitely, while preventing adrenal overgrowth. This treatment probably constitutes the most convenient long-term alternative to surgery.

Open access
Vivienne Yoon Department of Internal Medicine/Division of Endocrinology and Metabolism, VA North Texas Health Care System and the University of Texas Southwestern Medical Center, 5323 Harry Hines Boulevard, Dallas, Texas 75390-8857, USA

Search for other papers by Vivienne Yoon in
Google Scholar
PubMed
Close
,
Aliya Heyliger Department of Internal Medicine/Division of Endocrinology and Metabolism, VA North Texas Health Care System and the University of Texas Southwestern Medical Center, 5323 Harry Hines Boulevard, Dallas, Texas 75390-8857, USA

Search for other papers by Aliya Heyliger in
Google Scholar
PubMed
Close
,
Takashi Maekawa Department of Pathology, Tohoku University School of Medicine and Hospital at Sendai, Sendai, Japan

Search for other papers by Takashi Maekawa in
Google Scholar
PubMed
Close
,
Hironobu Sasano Department of Pathology, Tohoku University School of Medicine and Hospital at Sendai, Sendai, Japan

Search for other papers by Hironobu Sasano in
Google Scholar
PubMed
Close
,
Kelley Carrick Departments of Pathology

Search for other papers by Kelley Carrick in
Google Scholar
PubMed
Close
,
Stacey Woodruff Surgery, University of Texas Southwestern Medical Center, Dallas, Texas, USA

Search for other papers by Stacey Woodruff in
Google Scholar
PubMed
Close
,
Jennifer Rabaglia Surgery, University of Texas Southwestern Medical Center, Dallas, Texas, USA

Search for other papers by Jennifer Rabaglia in
Google Scholar
PubMed
Close
,
Richard J Auchus Department of Internal Medicine/Division of Metabolism, Endocrinology and Diabetes, University of Michigan Health System, Ann Arbor, Michigan, USA

Search for other papers by Richard J Auchus in
Google Scholar
PubMed
Close
, and
Hans K Ghayee Department of Internal Medicine/Division of Endocrinology and Metabolism, VA North Texas Health Care System and the University of Texas Southwestern Medical Center, 5323 Harry Hines Boulevard, Dallas, Texas 75390-8857, USA

Search for other papers by Hans K Ghayee in
Google Scholar
PubMed
Close

Summary

Objective: To recognize that benign adrenal adenomas can co-secrete excess aldosterone and cortisol, which can change clinical management.

Methods: We reviewed the clinical and histological features of an adrenal tumor co-secreting aldosterone and cortisol in a patient. Biochemical testing as well as postoperative immunohistochemistry was carried out on tissue samples for assessing enzymes involved in steroidogenesis.

Results: A patient presented with hypertension, hypokalemia, and symptoms related to hypercortisolism. The case demonstrated suppressed renin concentrations with an elevated aldosterone:renin ratio, abnormal dexamethasone suppression test results, and elevated midnight salivary cortisol concentrations. The patient had a right adrenal nodule with autonomous cortisol production and interval growth. Right adrenalectomy was carried out. Postoperatively, the patient tolerated the surgery, but he was placed on a short course of steroid replacement given a subnormal postoperative serum cortisol concentration. Long-term follow-up of the patient showed that his blood pressure and glucose levels had improved. Histopathology slides showed positive staining for 3β-hydroxysteroid dehydrogenase, 11β-hydroxylase, and 21 hydroxylase.

Conclusion: In addition to the clinical manifestations and laboratory values, the presence of these enzymes in this type of tumor provides support that the tumor in this patient was able to produce mineralocorticoids and glucocorticoids. The recognition of patients with a tumor that is co-secreting aldosterone and cortisol can affect decisions to treat with glucocorticoids perioperatively to avoid adrenal crisis.

Learning points

  • Recognition of the presence of adrenal adenomas co-secreting mineralocorticoids and glucocorticoids.

  • Consideration for perioperative and postoperative glucocorticoid use in the treatment of co-secreting adrenal adenomas.

Open access