Abstract | Feokromocitomi su rijetki tumori nadbubrežne žlijezde koji u najvećem broju slučajeva luče katekolamine (adrenalin i noradrenalin) zbog čega ostvaruju niz različitih i nespecifičnih simptoma u pacijenata. Najčešći znak u pacijenata s feokromocitomom je hipertenzija. Često su povezani s nasljednim sindromima (MEN2, VHL, NF1 i dr.), no najveći dio je sporadičan. Uglavnom su benigni, no u manjem broju slučajeva mogu biti i maligni što onda rezultira znatno lošijim kliničkim ishodom. Otežano se dijagnosticiraju zbog varijabilne kliničke prezentacije koja uključuje i asimptomatske pacijente pa uobičajeno prođe i nekoliko godina do dijagnoze. U najvećem broju pacijenta otkriju se slučajno (incidentalom) u sklopu radiološke obrade rađene zbog nekog drugog stanja ili bolesti. Liječenje je kirurško.
Cilj ovoga rada je bio prikazati i analizirati kliničke karakteristike 54 pacijenta koji su liječeni na Zavodu za endokrinologiju Klinike za unutarnje bolesti Kliničkog bolničkog centra Zagreb u zadnjih 5 godina te ih usporediti s rezultatima sličnih radova. Podatci su prikupljeni iz bolničke baze podataka; prikazani u obliku slika i tablica te analizirani.
U promatranoj skupini 39% je muških, a 61% ženskih pacijenata te je njihova prosječna dob u vrijeme dijagnoze bila 53 godine. Prosječna veličina tumora iznosila je 45 mm.
U 93% pacijenata tumor je lokaliziran unilateralno te je većina bila hormonski aktivna (85,2%). Najčešći znak bila je hipertenzija prisutna u 75,9% pacijenata, a od ostalih simptoma i znakova najčešće su zabilježeni skokovi tlaka u 44,4%, tahikardija/palpitacije u 33,3% i glavobolja u 29,6% pacijenata. Većina pacijenata (72,2%) imala je 2 ili više simptoma istodobno. Simptomi su prosječno trajali 3 i pol godine do trenutka dijagnoze te su tumori uglavnom otkriveni slučajno bez obzira na biokemijski profil tumora.
Zaključak: Većina pacijenata prilikom postavljanja dijagnoze feokromocitoma ima neke simptome i znakove, ali zbog njihove nespecifičnosti ovi se tumori ipak najčešće otkrivaju slučajno s odgodom od tri godine. |
Abstract (english) | Pheochromocytomas are rare adrenal tumors that, in most cases, secrete catecholamines (adrenaline and noradrenaline), causing a number of different and non-specific symptoms in patients. The most common sign in patients with pheochromocytoma is hypertension. They are often associated with hereditary syndromes (MEN2, VHL, NF1, etc.), but most of them are sporadic. They are mostly benign, but in a smaller number of cases they can also be malignant, which then results in significantly worse clinical outcome. They are difficult to diagnose due to the variable clinical presentation which includes asymptomatic patients, so it usually takes several years before diagnosis. In majority of patients they are discovered accidentally (incidentaloma) as a part of radiological findings performed for another condition or disease. Treatement is surgical.
The aim of this work was to present and analyze the clinical characteristics of 54 patients who were treated at the Department of Endocrinology of the Clinic for Internal Medicine of the Clinical Hospital Center Zagreb in the last 5 years and to compare them with the results of similar studies. Data were collected from the hospital database; presented in a form of pictures and tables and analized.
In the observed group, 39% were male and 61% were female patients, and their average age at the time of diagnosis was 53 years. The average size of the tumors was 45 mm. In 93% of patients the tumor was localized unilaterally and majority of them were hormonally active (85,2%). The most common sign is hypertension presented in 75,9% of patients, and of the other symptoms and signs, pressure spikes were the most often recorded in 44,4%, tachycardia/palpitations in 33,3%, and headache in 29,6% of patients. The majority of patients (72,2%) had 2 or more symptoms at the same time. The symptoms lasted on average for 3 and a half years until the moment of diagnosis, and the tumors were mostly discovered by chance, regardless of the biochemical profile of the tumor.
Conclusion: Most patients when diagnosed with pheochromocytoma have some symptoms and signs, but due to their non-specificity, these tumors are most often discovered by chance with a delay of three years. |