Resistant arterial hypertension in patient with pheochromocytoma/paraganglioma
Main Article Content
Abstract
Clinical case of pheochromocytoma accompanied by paraganglioma in patient with resistant arterial hypertension is presented. Pheochromocytoma/paraganglioma is the neoplasm, which produces catecholamines and consists of adrenomedullary chromaffin cells of adrenal glands or sympathic and parasympathic ganglia. Clinical course of disease depends on character and number of catecholamines which are excreted by neoplasm as well as how stable or episodic their release into the blood plasma is. Therefore, there is a persistent increase in blood pressure or hypertension has a paroxysmal (crisis) course. A feature of this clinical case is the absence of some characteristic diagnostic criteria. A targeted search was made for secondary causes of increased blood pressure despite the normal content of catecholamines in daily urine, the absence of adrenal gland changes during ultrasound. The clinical manifestations of pheochromocytoma, in addition to elevated blood pressure, were the presence of headache and palpitations. Significant weight loss by the patient over the past 1.5 years was noted which was caused by increased metabolism against the background of activation of the sympathoadrenal system. When performing duplex scanning of brachiocephalic arteries in the bifurcation region of both common carotid arteries, oval formations with clear even contours were revealed, which gave reason to suspect the atypical location of pheochromocytoma. Multispiral computed tomography with contrasting was performed of: abdominal cavity, adrenal gland, kidneys and renal arteries. In the course of the study, a rounded hypervascular formation with clear even contours was found in the body of the medial leg of the right adrenal gland. To clarify the diagnosis in the patient, the content of adrenaline and norepinephrine in the blood plasma was studied. An increase in norepinephrine by more than 4 times indicated the presence of a catecholamine-producing tumor. Thus, the diagnosis of pheochromocytoma of the right adrenal gland was confirmed and carotid paragangliomas of the neck were identified. The patient underwent laparoscopic adrenalectomy, as well as the removal of carotid paragangliomas of the neck. Doses of antihypertensive drugs were significantly reduced, blood pressure decreased to the level of 130–140/80–90 mm Hg. This clinical case illustrates the need to focus doctors on the possibility of this pathology in the absence of characteristic laboratory and instrumental abnormalities, which requires a persistent search for the causes of hypertension.
Article Details
Keywords:
References
Черенько С.М., Ларін О.С., Товкай О.А. Феохромоцитоми малого разміру: клініко-діагностичні та періопераційні аспекти захворюванння // Клінічна ендокринологія та ендокринна хірургія.– 2016.– № 4.– С. 34–42.
Chen H., Sippel R.S., Pacak K. et al. The NANETS Consensus Guideline for the diagnosis and management of neuroendocrine tumors pheochromocytoma, paraganglioma, and medullary thyroid cancer // Pancreas.– 2010.– Vol. 39 (6).– Р. 775–783. doi: https://doi.org/10.1097/MPA.0b013e3181ebb4f0.
Fräinkel F. Ein fall yon doppelseitigen, vollig latent verlaufenen Nebennierentumor und gleichzeit ger Nephritis mit Veranderungen am Circulations-apparat und Retinitis // Arch. Pathol. Anat.– 1886.– Vol. 103.– P. 244.
Guyton A.C., Hall J.E. Insulin, glucagon, and diabetes mellitus // Textbook of Medical Physiology / Ed. J.E. Hall.– 11th ed.– Philadelphia, PA: Elsevier Saunders.– 2006.– Р. 961–977.
Hickman P.E., Leong M., Chang J. Plasma free metanephrines are superior to urine and plasma catecholamines and urine catecholamine metabolites for the investigation of pheochromocytoma // Pathology.– 2009.– Vol. 41.– Р. 173–177. doi: https://doi.org/10.1080/00313020802579284.
Lenders J.W., Eisenhofer G., Mannelli M., Pacak K. Phaeochromocytoma // Lancet.– 2005.– Vol. 366.– P. 665–675. doi: https://doi.org/10.1016/S0140-6736(05)67139-5.
Mercado-Asis L.B., Tingcungco A.G., Bolong D.T. et al. Diagnosis of small adrenal pheochromocytomas by adrenal venous sampling with glucagon stimulation test // Int. J. Endocriol. Metab.– 2011.– Vol. 9 (2).– P. 323. doi: https://doi.org/10.5812/kowsar.1726913X.1951.
Pacak K., Eisenhofer G., Ahlman H. et al. Pheochromocytoma: recommendations for clinical practice from the First International Symposium // Nat. Clin. Pract. Endocrinol. Metab.– 2007.– Vol. 3 (2).– P. 92–102. doi: https://doi.org/10.1038/ncpendmet0396.
Plouin P.F., Amar L., Dekkers O.M. et al., on behalf of the Guideline Working Group. European Society of Endocrinology Clinical Practice Guideline for long-term follow-up of patients operated on for a phaeochromocytoma or a paraganglioma // Eur. J. Endocrinology.– 2016.– Vol. 174.– P. G1–G10. doi: https://doi.org/10.1530/EJE-16-0033.
Plouin P.F., Fitzgerald P., Rich T. et al. Metastatic pheochromocytoma and paraganglioma: focus on therapeutics // Horm. Metab. Res.– 2012.– Vol. 44.– Р. 390–399. doi: https://doi.org/10.1055/s-0031-1299707.
Porte D. Jr. A receptor mechanism for the inhibition of insulin release by epinephrine in man // J. Clin. Invest.– 1967.– Vol. 46.– Р. 86–94. doi: https://doi.org/10.1172/JCI105514.
Van Way C.W. III, Scott H.W. Jr., Page D.L., Rhamy R.K. Pheochromocytoma // Current Problems in Surgery.– 1974.– Vol. 11, Issue 6.– Р. 1–59. doi: https://doi.org/10.1016/S0011-3840(74)80008-0.
Zubera S.M., Kantorovich V., Pacak K. Hypertension in pheochromocytoma: characteristics and treatment // Endocrinol. Metab. Clin. North. Am.– 2011.– Vol. 40 (2).– Р. 295–311. doi: https://doi.org/10.1016/j.ecl.2011.02.002.