Endocrine Abstracts

In 1976, aged twenty, Mr A J was diagnosed with a cerebral haemangioblastoma, which was successfully removed. His father died from renal cell carcinoma and a Pheochromocytoma, raising the possibility of VHL syndrome. Genetic screening was positive but no follow-up was provided.

In 2004, aged 48, he presented with a two year history of persistent diarrhoea. A CT scan revealed a left sided 1.7 cm adrenal adenoma. Serial 24 h urine metadrenaline ranged between 0.37 and 0.67 mmol/l (0–2 mmol/l), normetadenaline ranged between 0.9 and 2.06 mmol/l (0–5 mmol/l) and 5HIAA analyses ranged between 23 and 25 mmol/d (0–50 mmol/d).

A decision was made to manage the lesion conservatively with close radiological and biochemical screening.

In 2006, surveillance imaging demonstrated two spinal haemagioblastoma (C4, L1) which were excised.

Earlier this year, CT scan demonstrated two contrast enhancing pancreatic cysts, the largest measuring 1.8×0.9 cm in the head of pancreas. A 1.5 cm simple renal cyst was also noted. Fasting Gut peptides were repeatedly normal (somatostatin, chromgranin A & B, glucagon, VIP, PPP) as was urine cytology. Over a three year period, the adrenal lesion was unchanged in size and remained biochemically inactive as assessed by urinary and plasma metanephrines.

A MIBG scan was performed demonstrating increased uptake in the area of the L adrenal gland. Although biochemical screening had not suggested the presence of excess catecholamine secretion, Mr A J had on occasions experienced symptoms that could be consistent with a phaeochromocytoma. In view of this and the increased uptake on the MIBG scan in the region of the adrenal a decision was made to alpha and beta block pre-operatively, and Mr A J received phenoxybenzamine 10 mg Tds and propanolol 10 mg Tds.

He underwent a Whipples procedure and L adrenalectomy. Interestingly, the histology of the adrenal gland was consistent with phaeochromocytoma and that of the pancreas suggested a neuroendocrine tumour.