HORMONE RESEARCH
Case Report
Horm Res 2004;62:67-70 DOI: 10.1159/000079322
Co-Secretion of Aldosterone and Cortisol by an Adrenocortical Carcinoma
Neslihan Kurtulmusa Sema Yarmana Halil Azizlerlia Yersu Kapranb
aDivision of Endocrinology, Metabolism and Nutrition, Department of Internal Medicine, and
bDepartment of Pathology, Istanbul Faculty of Medicine, Istanbul University, Çapa, Istanbul, Turkey
Key Words
Adrenocortical carcinoma . Hyperaldosteronism . Hypokalemia
Abstract
We report a rare case of adrenocortical carcinoma. A 26- year-old woman presented with hypokalemia and hyper- tension due to hyperaldosteronism. She had no signs of Cushing’s syndrome. Endocrinological data showed ex- cess of aldosterone production and nonsupressible corti- sol production on 2 mg of dexamethasone. Magnetic res- onance imaging showed left adrenal tumor. Transab- dominal left adrenalectomy was performed and histo- pathological diagnosis was adrenocortical carcinoma. Her blood pressure and hypokalemia returned to normal after adrenalectomy. There is no recurrence after 36 months. We want to emphasis the importance of adrenal tests before the operation even if there are no signs of excess cortisol production.
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Introduction
Adrenocortical carcinoma is an extremely rare tumor with estimated incidence of only 0.023 percent of all malignancies and this tumor is highly malignant neo- plasm [1-4]. Adrenocortical carcinomas are slightly more frequent in women than men. These tumors have been divided into nonfunctional and functional. Although al- dosterone may be the only adrenal steroid overproduced early in the course of the disease, hypersecretion of corti- sol usually occurs at some point [1, 3]. It may also cause hyperandrogenism [3, 5]. Some patients only demonstrate excess precursor steroids in the absence of endocrine syn- dromes. We report a rare case with a clinical picture of primary hyperaldosteronism and silent Cushing’s syn- drome.
Case Report
A 26-year-old woman was referred to our clinic due to hyperten- sion. Her past medical history included weakness of lower limbs, left upper abdominal pain, hypertension, and amenorrhea for a few months before referral. On physical examination, there was a palpa- ble left abdominal mass. She showed slight hypertrichosis on the cheeks but had no androgenic and cushingoid appearance. She had
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Neslihan Kurtulmus
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hypertension with a blood pressure of 150/100 mm Hg. Her serum potassium was 2.0 mEq/l. Hormonal studies demonstrated sup- pressed plasma renin activity and increased serum aldosterone and plasma 17a-hydroxyprogesterone levels. Her laboratory results be- fore the operation are shown in table 1.
Although the basal plasma cortisol concentration was within the normal range, loss of suppressibility by dexamethasone suggested autonomous cortisol secretion. Abdominal magnetic resonance imaging (MRI) revealed an 8-cm mass in the region of the left adre- nal. Thorax and abdominal MRI scans showed no metastasis. She was treated by spironolactone 200 mg daily which improved her hypokalemia and blood pressure and after 3 weeks her left adrenal mass was surgically removed successfully under steroid coverage. Grossly, this was a bulky tumor weighted 185 g and 8 x 6.5 x 6 cm in diameter. Microscopic examination of the tumor revealed differ- ent architectural patterns and cellular morphology. In some areas a trabecular pattern of the neoplastic cells was observed (fig. 3a), in other areas there was a prominent myxoid change with cells forming serpiginous cords (fig. 3b). Neoplastic cells had both acidophilic- compact and lipid-rich cytoplasm with nuclear pleomorphism and hyperchromasia (fig. 3a-c).Both vascular and capsular invasion was observed. Histopathological diagnosis was adrenocortical carcino- ma. After the operation, hypokalemia and serum aldosterone re- turned to normal range (K: 4.8 mmol/l; aldosterone: 66.2 pg/ml) and there was no hypertension, but basal cortisol level was low (<1 µg/dl). Steroid replacement therapy could be stopped after 1 year. DHEA-S was not evaluated after operation, because its basal level was normal. She remains well 3 years after the operation without any signs of metastasis.
Discussion
Adrenocortical carcinoma is a rare tumor. The clinical presentation of adrenocortical carcinoma may be similar to that of adrenal adenoma. But, modernday imaging, including ultrasonography, computerized tomography, and magnetic resonance imaging, have improved the
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diagnosis and staging of adrenal carcinoma [1]. Some- times, imaging studies cannot reliably differentiate be- tween benign and malign tumors and the diagnosis of adrenal carcinoma may depend on the histologic demon- stration of invasion of the capsule and blood vessels by tumor cells [3]. The most common presenting symptom of adrenocortical carcinoma is abdominal pain, which is often associated with a palpable abdominal mass on examination as seen in our case [1, 3, 6, 7]. Other clinical features of adrenocortical carcinoma include large tumor size, an abrupt onset of disease, pyrexia, and symptoms of hormonal syndromes [1]. Adrenocortical adenoma and carcinoma can produce a variety of steroid hormone syn- dromes. The most common clinical syndrome associated with a functional adrenocortical carcinoma was Cushing’s syndrome, as reported in literature [1-3]. However, aldo- sterone-producing adrenocortical carcinoma is rare [4, 5, 8, 9]. Our case presented with hyperaldosteronism. The nonsuppressibility of 17a-hydroxyprogesterone, and cor- tisol together with severe hypokalemia and very high lev-
els of aldosterone proves that the adrenal tumor is secret- ing cortisol, aldosterone, and 17a-hydroxyprogesterone autonomously. She had no classical features of Cushing’s syndrome, but Cushing’s syndrome was diagnosed with low- and high-dose dexamethasone suppression tests.
The benign adrenal adenoma is capable of co-secreting aldosterone and cortisol-like adrenal carcinoma [10, 11].
Any adrenal mass, whether benign or malignant, that produces cortisol will suppress the contralateral adrenal gland through the suppression of the hypothalamus-pitu- itary-adrenal axis. Some cases as presented here may not show the clinical appearance of the excess hormone syn- drome. If tests are not done to prove that there is no autonomous function, these products will be at great risk during and after the operation since coverage with ste- roids will not be given.
In conclusion, we think that all the hormonal activity of the adrenocortical gland should be investigated in adre- nocortical masses even though the clinical presentation is not suggestive of a clinical syndrome.
References
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