Prikaz serije bolesnika liječenih u Kliničkom bolničkom centru Zagreb zbog malignog feokromocitoma

master's thesis

• Title (english): A review of a series of patients treated at the University Hospital Center Zagreb for malignant pheochromocytoma
• Author: Ivana Mišak
• Mentor: Tina Dušek (mentor)
• Committee member: Darko Kaštelan (predsjednik povjerenstva)
• Committee member: Jasenka Markeljević (član povjerenstva)
• Committee member: Tina Dušek (član povjerenstva)
• Granter: University of Zagreb; School of Medicine; (Department of Internal Medicine); Zagreb
• Defense date and country: 2021-07-16, Croatia
• Scientific / art field, discipline and subdiscipline: BIOMEDICINE AND HEALTHCARE; Clinical Medical Sciences; Internal Medicine

Abstract

Feokromocitomi su rijetki tumori, uglavnom benignog karaktera, no u 10% slučajeva javljaju se maligni feokromocitomi. Prema kriterijima Svjetske zdravstvene organizacije, dijagnoza malignih feokromocitoma temelji se na dokazu metastaza u nekromafino tkivo udaljeno od tumora poput limfnih čvorova, kosti, jetre, pluća, mozga i drugdje. Temeljem isključivo histoloških parametara nije moguće postaviti dijagnozu malignosti, iako je kod malignih feokromocitoma izraženija mitotička aktivnost, stanična atipija ili vaskularna invazija. Maligni feokromocitomi rastu razmjerno sporo, a bolesnici umiru od komplikacija na srcu i krvnim žilama.
Cilj ovog rada je prikazati karakteristike bolesnika s malignim feokromocitomom, modalitete liječenja i ishod liječenja te ih usporediti s rezultatima sličnih studija. Korišteni su podaci o bolesnicima liječenim u Zavodu za endokrinologiju Klinike za unutrašnje bolesti Kliničkog bolničkog centra Zagreb. Podaci su prikupljeni iz bolničke baze podataka. Analizirano je osam bolesnika, 4 žene i 4 muškarca. Prosječna dob u kojoj je postavljena dijagnoza u žena je bila 56.5 godina, a u muškaraca 49.4 godine. U pet bolesnika (63.5%) primarno sijelo tumora bila je nadbubrežna žlijezda, a u tri (36.5%) primarni tumor nađen je u kromafinom tkivu izvan nadbubrežne žlijezde (abdomen, toraks). Primarni tumor (medijan veličine 7 cm) je u svih bolesnika kirurški otklonjen. Poslijeoperacijski su svi praćeni (medijan praćenja je 61 mjesec), a medijan do pojave metastaza bio je 21 mjesec. U tri (37.5%) bolesnika su metastaze bile prisutne pri postavljanju dijagnoze. Najčešće sijelo metastaza je bila jetra, zatim limfni čvorovi, kosti i pluća, a druga linija liječenja je provedena radioterapijom s MIBG-131 u 7 (87.5%) bolesnika, a u 4 (50%) bolesnika u kombinaciji s kemoterapijom. Tijekom praćenja dvoje bolesnika je umrlo zbog proširene maligne bolesti. Ovim istraživanjem pokazano je da samo kontinuirano poslijeoperacijsko praćenje bolesnika s feokromocitomom odnosno paragangliomom može otkriti metastasku bolest. U svih je provedena individualna klinička procjena razvoja bolesti te su liječeni i praćeni prema smjernicama priznatim u svijetu.

Abstract (english)

Pheochromocytomas are rare tumors, mostly benign, but in 10% of cases malignant pheochromocytomas occur. According to the World Health Organization, the diagnosis of malignant pheochromocytomas is based on evidence of metastases to necromaphilic tissue away from tumor such as lymph nodes, bones, liver, lungs, brain and elsewhere. Based exclusively on histological parameters, it is not possible to diagnose malignancy although mitotic activity, cell atypia or vascular invasion are more expressed in malignant pheochromocytomas. Malignant pheochromocytomas grow relatively slowly and patients die from complications of the heart and blood vessels. The aim of this study was to present the characteristics of patients with malignant pheochromocytoma, treatment modalities and treatment outcome, and to compare them with the results of similar studies. Data of patients treated at the Department of Endocrinology of the Clinic for Internal Medicine of the Clinical Hospital Center Zagreb was used. Data was collected from the hospital database. Eight patients, 4 women and 4 men, were analyzed. The mean age at which diagnosis was made in women was 56.5 years and in men 49.4 years. In five patients (63.5%) the primary site of the tumor was the adrenal gland and in three (36.5%) tumor was found in chromaphilic tissue outside the adrenal gland (abdomen, thorax). The primary tumor (median size 7 cm) was surgically removed in all patients. Postoperatively, all were monitored (median follow-up was 61 months) and the median to metastases was 21 months. In three (37.5%) patients metastases were present at diagnosis. The most common site of metastases was the liver, followed by lymph nodes, bones and lungs, and the second line of treatment was performed with MIBG-131 radiotherapy in 7 (87.5%) patients and in 4 (50%) patients in combination with chemotherapy. During follow-up, two patients died of widespread malignancy. This study showed that only continuous postoperative monitoring of patients with pheochromocytoma or paraganglioma can detect metastatic disease. All of them underwent an individual clinical assessment of the development of the disease and were treated and monitored according to guidelines recognized worldwide.

• Keywords: feokromocitom; metastaze; modaliteti liječenja
• Keywords (english): pheochromocytoma; metastases; treatment modalities
• Language: croatian
• URN:NBN: urn:nbn:hr:105:314761
• Study programme: Title: Medicine; Study programme type: university; Study level: integrated undergraduate and graduate; Academic / professional title: doktor/doktorica medicine (doktor/doktorica medicine)
• Type of resource: Text
• File origin: Born digital
• Access conditions: Open access
• Created on: 2022-03-16 12:14:39