Case history: We present the case of a 72-year-old lady who presented to the surgical team with sudden onset abdominal (flank) pain. On initial assessment, she was significantly hypertensive (213/110 mmHg) and tachycardic (pulse 98 bpm) but afebrile. She was not on any anticoagulation. On physical examination, her abdomen was tender with mild guarding and retroperitoneal tenderness.
Investigations: Admission blood tests showed a normal haemoglobin (149 g/l), normal clotting profile, renal function, liver function tests and calcium. Serum amylase was not raised. Urine dip stick showed no evidence of infection with no blood. Urgent CT scan of the abdomen revealed a 4.8 cm right adrenal mass with acute haemorrhage (with no evidence of extravasation or active bleeding). Urgent endocrine review on the same day raised the possibility of phaeochromocytoma, hence patient was initiated on phenoxybenzamine. Further investigations revealed elevated 24-hour urinary normetadrenaline (10.65 mmol/24 hr; reference interval 0.13.3) and metadrenaline (2.31 umol/24 hr; reference interval 0.11.2) but normal methoxytyramine level. Plasma normetadrenaline was elevated at 7287 pmol/L (1201180) and metadrenaline at 596 pmol/l (80510). Aldosterone and renin were normal (446 pmol/L and 39 mu/l respectively; ratio 12) as was the overnight dexamethasone suppression test (9 am cortisol 38 nmol/L after 1 mg dexamethasone at 11 pm). MIBG scan showed a focal area of increased uptake in the region of the right adrenal gland with no other lesions.
Results and treatment: Patient was closely followed up in Endocrine clinic and phenoxybenzamine was titrated up to 30 mg twice daily. Propranolol was added later to the regimen. Her blood pressure significantly improved (126/66 mmHg). Consequently, she underwent a laparoscopic adrenalectomy and tissue biopsy confirmed phaeochromocytoma. Patient has been referred for a full genetic work up.
Conclusions and points for discussion: Spontaneous adrenal haemorrhage is considered to be a rare phenomenon. There are very few cases in literature where the first presentation of phaeochromcytoma is a haemorrhagic adrenal gland presenting as an acute abdomen. Adrenal phaeochromocytoma rupture leading to intraperitoneal haemorrhage and shock can be fatal. Proceeding to surgery with an occult phaeochromocytoma can be catastrophic, therefore a high index of suspicion is required for pre-operative acute diagnosis. Urgent involvement of a multidisciplinary team is advised. Our case demonstrates that imaging is the most useful investigation for diagnosis, whilst medical optimisation followed by elective surgery is the best definitive treatment.
08 - 10 Apr 2019
Society for Endocrinology