Hypertension secondary to multiple malignant paragangliomas
Mazza, A.; Della Rocca, F.; Mormino, P.; Pessina, A.C.
Giornale Italiano di Cardiologia 26(8): 891-897
1996
ISSN/ISBN: 0046-5968 PMID: 9005172 Document Number: 461290
We describe a case of severe hypertension, due to multiple malignant norepinephrine secreting paragangliomas. Neuroendocrine system tumours are responsible for 0.1-0.5% cases of hypertension. Their extra-adrenal localisation is about 10%. Only 20% of paragangliomas are catecholamine secreting, and causing a syndrome similar to that of pheochromocytoma. In our patient the diagnosis of retroperitoneal multiple paragangliomas was made two years after the beginning of the typical symptoms. At surgery 6 paragangliomas were removed; some vascular and muscular structures (inferior vena cava, ileopsoas) were infiltrated. A radiotherapy treatment was conducted for a total dose of 55.8 Gy. Immediately after the operation blood pressure decreased, but the administration of alpha and beta blockers was required to reach normotension. This treatment was confirmed at six months follow-up, in consideration of the persistently high norepinephrine plasma levels.