D1 MTE2 Case 5: An adrenal mass...

A 35-year-old woman presented with weight gain (10kg), increased appetite, hirsutism, amenorrhoea, and deepening of voice for 8 months. On examination, blood pressure was 180/110 mm Hg, wide (1.5-2 cm), purple striae over anterior abdomen, hirsutism (mFMG score: 14), temporal baldness, clitoromegaly and proximal myopathy were noticed. Investigations revealed loss of circadian rhythm of cortisol with markedly elevated 24h- urinary cortisol (1371 μg) and serum testosterone (212.20 ng/dl) suggesting adrenocortical carcinoma (ACC). A non-contrast CT Abdomen showed a 65*63 mm well-defined hypo-dense left adrenal mass with homogeneous enhancement. She underwent laparoscopic left adrenalectomy and histopathology confirmed ACC with mitotic activity of 2-3/10 HPF. Post-operatively patient had features of adrenal insufficiency which was managed appropriately and discharged on oral prednisolone 5mg/day and fludrocortisone 100 μg/day. She discontinued medications a year later. Ten years later, she presented to us with similar complaints for 6 months. Her blood pressure was 112/86 mm Hg and mFGS score was 8. There were no striae, bruises, mycotic lesions, or proximal muscle weakness. Investigations revealed suppressed ONDST cortisol (9.55 μg/dl), elevated DHEAS (549.9 μg/dl), elevated total testosterone (143.16 ng/dl) and suppressed plasma ACTH (7.7 pg/ml). A CECT abdomen showed normal right adrenal with no evidence of recurrent or residual lesions in the abdomen. But, FDG-PET CT scan showed an FDG avid heterogeneously enhancing soft tissue mass of 9.3*7.5*8.7 cm (max SUV: 9.5) in the omentum-mesentry of the left middle quadrant of abdomen, likely representing metastasis. Patient underwent laprotomy and lesion was excised, which was confirmed to be low-grade ACC (mitotic activity: 6-8/10 HPF). Post-operatively, serum 8:00am cortisol was 0.43 μg/dl, DHEAS was 6.8 μg/dl and total testosterone < 10 ng/dl suggesting complete remission. She was replaced with glucocorticoids. Mitiotane therapy was offered but the patient deferred. She had documented remission at 1 year follow-up.