Endocrine Abstracts

Pheochromocytoma is a rare disorder of chromafin cells and can be incidentally found in 20% of adrenal masses. It can be malignant in upto 10% of the cases. Metastatic disease poses a great challenge and no definitive cure is yet established to treat these tumors.

We report a case of 54-year-old male patient with past medical history of anxiety who presented with acute left sided abdominal pain to the urology department at Norfolk and Norwich University Hospital with suspicion of renal colic. Computed Tomography Thorax and Abdomen with contrast Incidentally picked up 9 cm left adrenal lesion presumed to be hemorraghic with simple liver cysts and no evidence of malignancy. He was then referred to the endocrinology department for work up of the adrenal lesion. Clinical examination blood pressure was 142/87 and he had microspocic hematuria. Skin examination showed few benign intradermal naevi, skin tags, multiple neurofibromas and one café au lait Spot. There was no axillary freckling. Biochemical investigation revealed raised urine Normetadrealine 9.2 μmol/24 h (0.0–3.8) and plasma normetanephrine of 1606 pmol/l (120–1180) which raised suspicion of pheochromocytoma.

In consultation with Urologist Adrenalectomy was planned and optimization of Blood pressure was done with alpha and beta blockage. Pre Operative MIBG I123 whole body scan showed uptake of tracer in the left adrenal gland and the 4^th segement of the liver. This was suggestive of Left sided pheochromocytoma with hepatic deposit which was then characterised with an MRI scan as solitary liver deposit measuring 1.7 cm.

Laparoscopic adrenalectomy was complicated by excessive haemorrhage due to large size of the tumor hence open laparotomy and nephrectomy was carried out. Patient remained hypotensive postoperatively and was started on replacement Hydrocortisone which was stopped after normal synacthen response. Histology confirmed immunoreactivity for CD56, Chromogranin A, Synaptophysin and S100 (weakly) and high proliferation fraction (Ki-67) of 10–20% in keeping with an aggressive pheochromocytoma. Genetic testing was still pending.

Patient was then referred to UCL for management of the solitary liver lesion. Urinary and plasma normetanephrine had normalised at 1 month interval, but he was unfortunately found to have widerspread Metastasis to the liver, lungs and bones on repeat CT scanning. It is presumed that due the aggressive nature of the tumor and intra-operative bleeding the malignant cells were seeded in the circulation and spread widely and rapidly. Although Surgical resection is rarely curative in Malignant pheohromcytomas, tumor debulking can reduce the toxic effects of catecholamines on the mycocardium and improve survival. Mostly treatment is palliative and without treatment the 5-year survival is generally less than 50%. This widespread metastasis in such short interval has not been reported previously in literature and the levels of catacholamines may not depict the true malignant potential of the tumor as in our case they were only modestly raised. We also suggest that in presence of metastatic disease with malignant pheochromocytoma adjuvant therapy is considered concomitantly and patients are counselled pre-operatively about poor prognosis.