INTRADUCTAL PAPILLARY MUCINOUS NEOPLASM OF THE PANCREAS: CASE REPORT AND REVIEW OF THE LITERATURE
Autori:
Hrvoje Iveković, Nadan Rustemović, Milorad Opačić, Ognjan Deban, Boško Romić, Jasminka Jakić Razumović, Rajko Ostojić, Roland Pulanić, Boris Vucelić
Sažetak
Intraduktalna papilarna mucinozna novotvorina (IPMN) gušterače relativno je rijedak klinički entitet, čija je osnovna karakteristika izlučivanje sluzi. Širenje IPMN-a duž vodova gušterače i stvaranje sluzi dovodi do dilatacije i opstrukcije vodova, što se klinički očituje epizodama akutnog pankreatitisa. U podlozi IPMN-a nalazi se nekoliko molekularnih poremećaja, među kojima je mutacija K-ras-gena vjerojatno inicijalna aberacija. IPMN se zbog indolentnog rasta najčešće dijagnosticira u sedmom desetljeću života. IPMN, ovisno o histološkom tipu, ima stanoviti maligni potencijal pa je kirurški zahvat »zlatni standard« liječenja. Podmukli i spori razvoj bolesti, uz nedostatak bilo kakve simptomatologije u stanovitog broja bolesnika čini dijagnostičko prepoznavanje ovog entiteta iznimno složenim. U članku prikazujemo bolesnika s IPMN-om gušterače u kojeg su tegobe pankreatitisa trajale 22 godine.
Summary
Intraductal papillary mucinous neoplasm (IPMN) of the pancreas is a relatively rare clinical entity with a main characteristic being mucus production. Extension of IPMN along pancretic ducts and mucus production lead to ductal obstruction and dilatation, resulting in recurrent episodes of acute pancreatitis. Molecular background of IPMN-a comprises several aberrations, with the K-ras gene mutation being the likely trigger that initiates further genetic changes. Due to its indolent nature, IPMN is most commonly diagnosed in the 7th decade of life. Depending on the histology type, IPMN has a malignant potential. Therefore, surgical therapy remains a »gold standard« of treatment. Insidious, slow progression of the disease and absence of symptoms in a certain number of patients makes diagnostic approach to this entity difficult. In this paper we present a patient with IPMN of the pancreas, in whom the episodes of acute pancreatitis had been present for 22 years.
