Adrenal tumor with hirsutism and hypertension

We studied a 48 year old white female, presenting facial hirsutism and hypertension. Hysterectomy and right oophorectomy because of a fibromiome 10 years before. Rapid development of facial hirsutism (in four months) and persistent high blood pressure, controlled with Enalapril 10 mg/day; no weight changes, fever galactorrhoca and other signs of masculinization or hypercortisolism. Physical examination confirmed excessive growth of hair only in the face without other physical alterations, including palpable abdominal mass or abnormal genitalia. Laboratory tests showed normal haemogram, ionogram, liver and renal tests. Normal thyroid function tests. Normal prolactinemia. Low seric levels of: ACTH 5 pg/ml (n=9-52 pg/ml). FSH 0.46mlU/ml (menopause: 41-111 mUl/ml). LH 0.79 mUl/ml (menopause\gr14 mUi/ml). High seric levels of: cortisol 36.8 ug/dl (n=5-25 ug/dl), 17-OHProgesterone 11.4 ng/ml (n= 0.1-0.6 ng/ml). DHEA-S 836 ug/dl (n=35-430 ug/dl). D4-androstenedione 4.5 ng/ml (n= 0.46-2.67 ng/ml), total testosterone 1.2 ng/ml (n=0.06-0.86 ng/ml) and free testosterone 2.7 pg/ml (n=0.6-2.5 pg/ml), estradiol 383 pg/ml (n<14 pg/ml), progesterone 5.2 ng/ml (n= 0.3-0.9 ng/ml). High urinary levels of cortisol 199 g/24 hours (n=10-80 g/24H). Normal urinary levels of Vanilmandelic acid in three successive determinations. Normal plasmatic levels of aldosterone and renine (ortostatic and supine). CT scan of the abdomen revealed a homogeneous mass in the left adrenal gland with 13 cm in diameter, non invasive and without necrosis, calcifications or hemorrhage. The patient underwent left adrenalectomy. The histological diagnosis showed a well differentiated adrenocortical carcinoma with a weight of 450 gr and 13 cm in diameter. The tumor was well capsulated without vascular invasion and presenting 2 mitosis/50 HPF with discrete celular pleomorphism: immunohistochemically the cels were immunoreactive for vimentin, NSE, chromogranin and negative for EMA and low-molecular-weight keratin. Tumoral staging T1N0M0. Two months latter, the patient is asymptomatic, on hydrocortisone 10 mg/day, with normal hormonal levels and without evidence of metastases or tumor recurrence. Hirsutism associated with hypertension, hyperandrogenism and hypercortisolism, suggested an adrenal tumor. This tumor has been clinically benign despite the high hormonal levels. The weight (\gr50 gr) and the size (\gr5 cm) suggested a diagnosis of well differenciated adrenocortical carcinoma. Adrenocortical carcinomas are rare tumors (incidence of 2 per million population) and the differential diagnosis with adenomas can be difficult. Some authors consider that when only two criteria of malignancy are present metastization or recurrence are unlikely, but if there are more than four criteria they are usual. No single parameter can discriminate between benign and malignant tumors. Diagnosis is only reached through a combined evaluation of clinical features, size and weight, microscopic appearence and evolution.