Endocrine Abstracts

A 34-year-old male with no significant past medical history presented with severe abdominal pain. On detailed questioning his symptoms included tremors, headache, sweating, and agitation. Despite recent weight gain he had obvious muscle wasting. On admission blood pressure was 166/107 mmHg. Abdominal examination revealed a palpable left upper quadrant mass.

CT scan demonstrated a 15 cm mass in the left supernal region with extensive signs of haemorrhage in and around the tumour. Endocrine work up documented increased 24-h urine normetadrenaline (5.05 μmol/24 h, normal 0–3.8) and 24-h urinary free cortisol (1754 nmol/24 h, normal <130). Serum cortisol failed to suppress overnight after 1 mg dexamethasone (687 nmol/l, normal <50). The initial working diagnosis was of a phaeochromocytoma co-secreting catecholamines and ACTH.

In view of his hypertension and elevated catecholamines he was initially managed with phenoxybenzamine followed by Verapamil for rate control (intolerant to β-blockers). Despite four antihypertensive agents hypertension persisted. Later, suppressed plasma ACTH was discovered, excluding the rare entity of an ACTH-producing phaeochromocytoma. Surprisingly, MIBG imaging demonstrated no uptake in the area of adrenal mass. Plasma catecholamines repeated 3 months after initial presentation had normalised. Phenoxybenzamine was discontinued. Steroid analysis confirmed persistence of Cushing’s and documented increased levels of DHEAS, androstenedione, 17-OH progesterone and progesterone consistent with the diagnosis of adrenocortical carcinoma.

The patient underwent left adrenalectomy without complications and was commenced on hydrocortisone perioperatively. The adrenal mass weighed 2195 g, measuring 21×16×10 cm. Histology confirmed adrenocortical carcinoma with a Ki-67 of 4.4% and modified Weiss score of 6/7. He will be commenced on mitotane.

Unilateral adrenal haemorrhage causing a pseudo-phaeochromocytoma presentation can occur in the context of adrenal pathology such as adrenal adenoma, adrenal carcinoma or metastatic disease. We present an unusual case of adrenocortical carcinoma secreting cortisol complicated by transient increase in catecholamines due to adrenal haemorrhage into the tumour.