Endocrine Abstracts

Introduction: Pheochromocytomas are rare neuroendocrine tumours from catecholamine producing chromaffin tissue of adrenal medulla that typically present with headache, palpitations, diaphoresis, and paroxysmal hypertension. Here we describe a case of pheochromocytoma presenting as haematuria. There are case reports of paraganglioma of urinary bladder with haematuria. In our case no bladder lesion was identified but close follow up is needed for recurrence and bladder involvement.

Case report: A 74-year-old male with history of myotonic dystonia was investigated by Urologist for haematuria and CT abdomen pelvis showed a 2.4 cm left adrenal nodule with density 25HU. No other cause of haematuria was found. He did not have hypertension or other classic symptoms of pheochromocytoma. Further biochemical studies showed raised plasma metanephrine (1521 pmol/l), normetanephrine (1601 pmol/l), and 24H urinarymetanephrines(3.94µmol/24hr) with normal Chromogranin A levels(3ng/ml).24-hour BP monitoring showed maximum systolic BP of 169 and maximum diastolic of 97 mmHg.MIBG scan confirmed left adrenal pheochromocytoma. Laparoscopic adrenalectomy was done after adequate alpha and beta blockade. The histology is consistent with pheochromocytoma with PASS score of 3. He needs close follow up and monitoring for any local or metastatic recurrence particularly for recurrence in bladder. He is referred for genetic testing as well.

Discussion: This is a case of pheochromocytoma as incidental adrenal nodule in a normotensive patient who presented with haematuria. A close follow up is needed for patients after surgery for at least 10years to look for recurrences or new tumours. Plasma or urinary metanephrines and normetanephrines to be checked every year. Those with syndromic disease like VHL, MEN2, NF-1 may develop renal cancer, Medullary cancer of thyroid. European Society of Endocrinology recommends all patients with PPGL to be considered for genetic testing as around 40% of cases carry a mutation and identifying the mutation type helps to deliver an individualised patient management plan.