Paraganglioma: A cause of hypertension in a young patient

Abstract

Introduction. Collections of neuroendocrine cells dispersed throughout the body are known as paraganglia and the tumor arising from these paraganglia are known as paragangliomas. Paragangliomas located along side the aorta are associated with the pheochromocytoma and they secrete and store catecholamines. Case report. We reported a 29-year-old woman with episodes of headache, palpitations, dizziness and sweats, associated with hypertension. Elevated urine catecholamines were consistent with pheochromocytoma. Nuclear magnetic resonance (NMR) was performed and the symptoms were caused by associated mediastinal and retroperitoneal paragangliomas. The tumors were surgically removed. In a 3-year follow-up period the patient had two recidivate lesions, one of them was surgically removed, and for the last one a 6- month follow-up was recommended, because urine cathecholamine level was not significantly elevated and blood pressure was normal. Conclusion. Clinical and imaging data of patients with extra adrenal paragangliomas are not specific. Many of them may be asymptomatic even when the lesion is large, but if tumor is functional, diagnosis may be easier. Patients should be initially evaluated by determining catecholamine level, followed by computerized tomography (CT) or NMR to locate the primary lesion. Since there are no definite microscopic criteria for the distinction between benign and malignant tumors, radical excision and prolonged follow-up is necessary.Uvod. Grupe neuroendokrinih celija koje su lokalizovane u razlicitim delovima tela poznate su kao paraganglije, a tumori poreklom od ovih celija su paragangliomi. Paragangliomi lokalizovani duz aorte imaju iste karakteristike kao feohromocitom, tj. luce povecane kolicine kateholamina. Prikaz bolesnika. Prikazana je 29-godisna bolesnica sa povremenim glavoboljama, palpitacijama, mucninama i preznojavanjem praceni hipertenzijom. Povecan nivo kateholamina u 24-casovnom urinu ukazivao je na postojanje feohromocitoma. Uradjena je nuklearna magnetna rezonanca (NMR) toraksa i abdomena i utvrdjeno je da su opisani simptomi uzrokovani paragangliomima u posteriornom medijastinumu i retroperitoneumu. Tumorske mase bile su operativno odstranjene. Tokom 3-godisnjeg pracenja, bolesnica je imala dva recidiva, a jedan od njih je hirurski otklonjen. Posle otkrivenog drugog recidiva, krvni pritisak i nivo kateholamina u 24-casovnom urinu bili su u granicama normale, te smo savetovali 6- mesecne redovne kontrole. Zakljucak. Klinicki i radioloski znaci ekstraadrenalnih paraganglioma su nespecificni. Mnogi su asimptomaticni, cak i kada se radi o vrlo velikim lezijama, ali ako su funkcionalne, dijagnoza je laksa. Kod takvih bolesnika odredjuje se nivo kateholamina u urinu i radi se kompjuterizovana tomografija (KT) ili NMR radi lociranja primarne lezije. Kako ne postoji definitivan mikroskopski kriterijum za razdvajanje benignih od maligne lezije, neophodni su radikalna ekscizija i klinicko pracenje operisanih bolesnika.