Endocrine Abstracts

A 39-year-old female presented with an 18-month history of borderline hypertension, headaches, palpitations and some anxiety symptoms. Both parents also had hypertension. Blood pressure was 160/102 on no medications. She had a large single cafe au lait spot but no neurofibromata. Urinary dopamine levels were repeatedly elevated (5398–8653 nmol/24 h (n<3900)) with normal noradrenaline and adrenaline levels. Serum calcium was also elevated at 2.83 mmol/l (n=2.2–2.6). Meta-iodobenzylguanidine (MIBG I-123) scanning indicated a single focus of activity further defined on SPECT–CT as a 12 mm mass in the lower para-aortic region. Parathormone was elevated at 274 pg/ml (n=15–70) and a nuclear uptake scan indicated a right lower parathyroid focus. Calcitonin was unmeasurable. Following control of blood pressure with amlodipine and lisinopril she proceeded to surgery where a black lobulated paraganglioma was removed at the organ of Zuckerkandl. A 30 s asystolic episode occurred on first manipulation of the tumour. Urinary dopamine levels normalised, antihypertensives were reduced and 5 months later a parathyroid adenoma was removed uneventfully. Antihypertensives have recently been withdrawn. Genetic testing to date has been negative including MEN2a, succinate dehydrogenase B, C and D mutations. Dopamine secreting paragangliomas are extremely rare and are usually metastatic at diagnosis. They are usually associated with nonspecific symptoms, normotension and present with mass effects. Alpha blockade is contraindicated because of its association with cardiovascular collapse which is due to the unopposed hypotensive action of dopamine when the pressor catecholamines are blocked. This is the first reported case presenting with a coexistent parathyroid adenoma.