Section 1: Case history: A 68 year old lady presented with a 2 week history of pedal swelling, with large blisters appearing on her feet. Her past medical history included adrenal Cushings treated with a right laparoscopic adrenalectomy on a 4.7x5.2 cm lipid poor adenoma 12 years previously; she had been discharged 7 years ago after repeated normal overnight dexamethasone suppression tests. Over the last year she had gained 12 kg in weight and been diagnosed with type 2 diabetes. She also reported hair loss and proximal myopathy. She was treated with furosemide although her BNP was only 28ng/l. An ultrasound suggested a right renal mass. She was referred to the Endocrine service.
Section 2: Investigations: On review in clinic, she was noted to be clinically Cushingoid. Her random cortisol was 638 nmol/l at 5pm and she failed to supress in an overnight dexamethasone suppression test with a cortisol of 584 nmol/l at 9am after 1mg of dexamethasone. A CT abdomen demonstrated a large heterogenous mass in the right suprarenal region, suspicious for malignancy in keeping with likely adrenocortical cancer with near complete effacement of the IVC.
Section 3: Results and treatment: Her case was discussed in the adrenal MDT. The initial histology from the previous adrenalectomy was suggestive of an adenoma, but a breach of the capsule was noted. It was not possible to determine if this was an intraoperative breach. A surgical resection was recommended, and she was commenced on metyrapone to control her cortisol prior to surgery. She was unable to tolerate this due to nausea, so was switched to fluconazole which allowed adequate cortisol control. The patient subsequently underwent resection of the adrenal bed mass with right nephrectomy. The histology demonstrated adrenocortical carcinoma with a high Weiss score of 5, scoring for high nuclear grade, confluent tumour necrosis, increased mitotic activity, and atypical mitotic figures. Post operatively she has been started on adjuvant mitotane with concomitant hydrocortisone.
Section 4: Conclusions and points for discussion: This is a case of a patient who had originally presented with adrenal Cushings due to what was thought to be a benign adenoma, representing after a significant period of time as an adrenocortical cancer. This highlights the challenges of predicting behaviour of adrenal tumours, particularly in larger adrenal masses, and also raises the question of how long it is appropriate to follow up patients with adrenal Cushings disease following surgical resection.