Adrenocortical Carcinoma Presenting as Reversible Dilated Cardiomyopathy.

We present a 32-year-old woman with no morbidities who was admitted with a dilated cardiomyopathy and cardiac failure due to adrenocortical carcinoma (ACC) which improved completely with surgical resection. Awareness regarding such rare presentations can avoid undue delay in diagnosis and management.

INTRODUCTION

Adrenocortical carcinoma (ACC) is a rare heterogeneous neoplasm accounting for 0.02% of all annual cancers reported. About 60% are functional tumors secreting hormones, with its consequent clinical manifestations, the Cushing's syndrome due to cortisone, virilization due to androgens, feminization due to estrogens, or hypertension due to aldosterone. However, a growing proportion of patients with ACC (currently >15%) is initially diagnosed incidentally.

CASE PRESENTATION

A 32-year-old woman with no premorbidities presented with bilateral pedal edema and breathlessness for 1 week. She had hirsutism (modified Ferriman–Gallwey score– 18) [Figure 1 upper panel a and b], clitoromegaly, high blood pressure, and bilateral basal fine crepitations.

Figure 1

Clinical photograph showing increased facial hair growth (upper panel a and b).

Hemoglobin was 15.2 g/dl, total leukocyte count 6600/μl, platelet count 2.2 × 109/L, erythrocyte sedimentation rate 30 mm in1 h, and C-reactive protein was normal. Routine biochemical parameters were normal. Chest X-ray showed cardiomegaly and electrocardiogram showed sinus tachycardia, left axis deviation with left ventricular hypertrophy and strain pattern. Echocardiogram showed moderate left ventricular dysfunction with dilated cardiomyopathy. Ultrasonography of abdomen showed left solid suprarenal mass. Contrast-enhanced computed tomography of the abdomen showed an 8.9 cm × 6.8 cm contrast enhancing left adrenal mass [Figure 2a and b].

Figure 2a

Computed tomography of abdomen showing left adrenal mass.

Figure 2b

Adrenalectomy specimen.

Serum cortisol was 26.9 mcg/dl (10–20 mcg/dl), dehydroepiandrosterone sulfate 660 mcg/dl (35–430 μg/dl), androstenedione 375 ng/dl (50–220 ng/dl), and adrenocorticotropic hormone 12 pg/ml (10–50 pg/ml). She had lack of cortisol suppression after low-dose dexamethasone. She underwent open left adrenalectomy, and the histopathology revealed ACC. When followed up after 6 months, she was asymptomatic and blood pressure was controlled. Review of echocardiogram showed normal ventricular function.

DISCUSSION

Adrenocortical carticnoma (ACC) is a rare malignancy with an incidence of 0.7–2.0 cases/million habitants/year often with an unfavorable prognosis.[1] ACC occurs at any age, with two peak incidence: the first one in the first decade and the second one between 40 and 50 years. Women are most frequently affected (55%–60%). Most patients (40%–60%) present steroid hormone excess (glucocorticoids, mineralocorticoids, and androgens) or abdominal mass effects (30%), but 15%–20% of patients with ACC are initially diagnosed incidentally.[2]

Biochemically or clinically apparent adrenocortical hormone production is evident in up to 45%–70%. Symptoms related to the hormone excess are the major cause for presentation, leading to imaging and clinical investigation among them. However, syndromes of hormone excess are often not readily recognized by physicians, leading to delay in diagnosis and treatment.[3] Nearly 50%–80% of patients having hormone-secreting ACCs present with features of hypercortisolism including plethora, diabetes mellitus, muscle weakness, and osteoporosis. Hypokalemia and hypertension are commonly observed in ACC patients with hypercortisolism secondary to glucocorticoid-mediated mineralocorticoid receptor activation. Nearly 40%–60% of hormone-secreting ACCs produce adrenal androgens leading to rapid-onset male pattern baldness, hirsutism, virilization, and menstrual irregularities in women.[4] About 50% of patients with hormone-secreting ACCs have increased in both androgen and cortisol.

Hypercortisolism can result in cardiovascular manifestations like cardiomegaly, myocardial ischemia, left ventricular hypertrophy, and congestive heart failure. Long-term exposure to excessive cortisol, rather than the blood pressure levels, was associated with progression of cardiac remodeling in Cushing's syndrome patients in various studies.[56] Hypokalemia, diabetes mellitus, and hypercortisolemia were the major contributing factors to cardiac dysfunction in adrenal Cushing's syndrome patients in another study.[7]

There have been a few case reports of Cushing's patients with dilated cardiomyopathy, which was fully reversed after treatment. The mechanism of this dilated cardiomyopathy has not been defined.[8] Studies have suggested that among men, low DHEA-S levels have been consistently associated with an increased risk of all-cause mortality and cardiovascular disease but there are no studies correlating high levels of DHEA-S and cardiac disease.[9] The patient had features of excess androgen and cortisol, and the cardiac failure might be secondary to hypercortisolism.

CONCLUSION

We present a patient with a dilated cardiomyopathy and cardiac failure secondary to ACC which improved completely with surgical resection. Awareness regarding such rare presentations can avoid undue delay in diagnosis and management.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

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Conflicts of interest

There are no conflicts of interest.