Androgen Secreting Adenoma Co- Secreting Cortisol First Diagnosed During Pregnancy

Abstract

Background: Pure androgen secreting adenoma is rare (1) and rarer to have an adenoma that is co-secreting androgen and cortisol, and extremely rare to have it diagnosed during pregnancy. In female, an elevated serum testosterone level with normal adrenal androgens is usually regarded as ovary origin (2). Here we report an unusual case of pregnant woman with markedly elevated serum testosterone level and elevated cortisol that first presented during pregnancy. Clinical Case: A 32-year old lady who is 32 weeks pregnant, presented with signs and symptoms of Cushing and acute hirsutism developed and advanced during pregnancy. Clinically She had hypertension, gestational diabetes with sever hirsutism and virilization. Fasting total testosterone was high (52 nmoL/L n 0.2–2.9 nmoL/L), DHEAS (1.98 umol/L n 0.51–11 umol), 24 hours urine cortisol (1343 nmoL/24 hrs n 11.8–485 nmoL/24 hrs), ACTH (0.220 pmol/L n 1.6–13.9pmol/L). Adrenal MRI without contrast showed a large right adrenal mass of 6.5 x 6.2 x 5.3 cm, of heterogenous signal in all pulse sequences, with limited assessment due to the lack of IV contrast. During admission,the patient’s liver enzymes were gradually increasing which lead to the decision of an elective C-section at 34 weeks gestation followed by right robotic adrenalectomy after ruling out pheochromocytoma. The product was viable and healthy baby boy. The immediate post-op hormonal profile showed normalization of urinary cortisol and serum total testosterone level. Histopathology report confirmed an Adrenocortical adenoma. The patient was discharged on the 3rd day post-op on hydrocortisone replacement therapy. The hydrocortisone doses were gradually tapered down tell stopped. The course of follow up was uneventful, two years following the surgery, she got another pregnancy with no obvious complications.Conclusion: Co- existing testosterone and cortisol secreting adrenocortical adenoma is a rare tumor, diagnosis and management during pregnancy is challenging, with surgical resection being the only possible definite management.