1. DOC secreting adrenal adenoma, a rare cause of hypertension
EP 128
Paja M1, Dublang M1, Zabalegui A1, Arrizabalaga C1, Expósito A2, Espiga J1.
Endocrinology1 and Endocrine Surgery Department2. Basurto University Hospital. Bilbao. Spain.
Introduction:
Secondary endocrine hypertension affects around 10% of all hypertensive population, most frequently primary aldosteronism (PA). Less frequent forms of adrenal hypertension include pheochromocytoma and mineralocorticoid excess other than PA, featuring suppressed renin without excess of aldosterone.
A 29-year-old woman with difficult to control hypertension diagnosed two years before and poor response to ACEI and ARA II was referred. She had not Cushing's stigmata, hirsutism, overweight nor clinical suspect of pheochromocytoma. She had regular menstrual cycles. Biochemical study was normal except for slightly low potassium. Hormonal study is shown in Table. Abdominal CT (Figure 1) revealed a well-defined heterogeneous and much vascularized right adrenal mass of 6 cm in diameter. Deoxycorticosterone (DOC), measured in previous stored sample, was markedly elevated (Table) with normal deoxycortisol, testosterone, DHEAs and plasmatic and UFC. Open right adrenalectomy was performed. The tumour was an adenoma of 6.5 cm not encapsulated, composed of cells with clear and eosinophilic cytoplasm and large nuclei without mitosis nor necrosis (Figure 2). Weiss's criteria classified it as adrenocortical adenoma. Postoperatively, DOC level fell to normal levels and blood pressure and potassium normalized.(Table)
54,9%
Fig. 1. Abdominal CT – arterial phase
More detailed picture of tumour
Figure 2 - Image of the tumor border at low magnification K
PRA
ALDO
17OHP
Cortisol (F)
ACTH
DOC*
11 DeoxyF*
DHEAS
Preop
3.14
<0.2
7.3
0.8
10.88
34.5
237.9
4.5
1.31
Postop 1 m
4.34
0.2
4.7 -
18.35
7.1
3.9
Reference range
3.5-5 mEq/l
1.5-3 ng/ml/h
10-22 ng/dl
0.3-1 ng/ml
µg/dl
10-50 pg/ml
< 15 ng/ml
<7.2 ng/dl
µg/ml
Abdominal CT – venous phase
Biochemical evaluation – preoperative DOC and 11-deoxycortisol were measured in frozen samples after knowing the other results
Conclusion
DOC-producing adrenal neoplasm are exceptionally reported, and they are usually malignant tumours. They should be suspected in the presence of adrenal tumours with supressed renin but inappropriate low aldosterone, not suggesting PA. Early diagnosis can be very important because malignancy is the rule in this rare pathology.