| Abstract | Neuroendokrini tumori su heterogena skupina tumora. Pojavljuju se diljem tijela, u različitim tkivima. Njihova incidencija je u velikom porastu, djelom i zbog sve većih i boljih dijagnostičkih metoda koje nam omogućavaju da ih otkrijemo u što ranijoj fazi. Unatoč boljim dijagnostičkim metodama još uvijek se ovi tumori otkrivaju u prekasnoj fazi za potpuno izlječenje. Prezentiraju se različitim kliničkim slikama, ovisno o tome luče li hormone ili su inaktivni. U neuroendokrine tumore gušterače, osim inaktivnih tumora, spadaju inzulinom, gastrinom, glukagonom, vipom i somatostatinom. Po WHO klasifikaciji možemo ih podijeliti u neuroendokrine tumore gradusa I i II, te neuroendokrine karcinome, gradus III. Najčešće se pojavljuju kod pacijenata srednje dobi. Klinička slika može dugo vrijeme ostati neprepoznata. Inaktivni tumori ne luče hormone. Prezentiraju se simptomima koji nastaju zbog mase tumora i pritiska na okolne strukture. Najčešći simptomi su žutica, bolovi u abdomenu i gubitak težine, no mnogi se slučajno otkriju. Aktivni neuroendokrini tumori luče hormone, te se prezentiraju kliničkim sindromima koji nastaju zbog djelovanja hormona kojeg luče. Najučestaliji je inzulinom koji se prezentira simptomima hipoglikemije. Gastrinom se prezentira brojnim peptičkim ulkusima koji ne reagiraju na konzervativnu terapiju inhibitorima protonske pumpe, proljevima, bolovima u abdomenu. Vipomi se prezentiraju dugotrajnim i obilnim vodenastim proljevima. Za pacijente oboljele od glukagonoma tipična su 4 simptoma, dermatoza, dijabetes, duboka venska tromboza i depresija. Bolesnici sa somatostatinomom nemaju specifične simptome, nego najčešće bol u abdomenu i gubitak na težini. S obzirom na brojne nespecifične simptome kojima se prezentiraju ponekad je dosta teško posumnjati o kojemu se tumoru radi, no uvijek ih trebamo razmatrati kod diferencijalne dijagnoze. Za dijagnozu tumora možemo koristiti mnoge metode. Kromogranin A je biokemijski marker koji možemo otkriti u granulama tumorskih stanica. Od slikovnih metoda koriste se CT, MR, UZV, EUZ, 18FDG pozitronska emisija i prikazivanje somatostatinskog receptora. Za prikazivanje primarnog tumora u gušterači najvažniji nam je endoskopski ultrazvuk pomoću kojega možemo odrediti točnu lokaciju tumora te njegovu proširenost na okolne strukture. Možemo koristiti i octreoscan, 68Ga pozitronsku emisiju i patohistološku analizu. U liječenju je bitno što je više moguće kirurški odstraniti tumorsko tkivo. Koriste se i analozi somatostatina, interferon alfa, kemoterapeutici poput cisplatine ili karboplatine kombinirane sa etopozidom, te mTOR inhibitori poput everolimusa i VEGF inhibitori. |
| Abstract (english) | Neuroendocrine tumors are a heterogeneous tumor group. They appear across the body in different tissues. Their incidence is on the rise, due to the growing and better diagnostic methods that enable us to detect them as early as possible. Despite the better diagnostic methods, these tumors are still being discovered in the far-reaching phase for complete cure of the patient. They are presented in various clinical conditions, depending on whether the hormones are or aren't produced. In the group of neuroendocrine pancreatic tumors, in addition to the inactive tumors, there are insulinoma, gastrinoma, glucagonoma, vipoma and somatostatinoma. By WHO classification we can divide them into neuroendocrine tumors of grade I and II, and neuroendocrine carcinoma, grade III. Most commonly they appear in middle-aged patients. Clinical appearance can be long time unrecognizable. Inactive tumors do not produce hormones. They are presented with symptoms caused by tumor mass and pressure on surrounding structures. The most common symptoms are jaundice, abdominal pain and weight loss, but many are accidentally discovered. Active neuroendocrine tumors produce hormones
and are presented by clinical syndromes that are caused by the action of the hormones. The most common is insulinoma presented with hypoglycaemia symptoms. Gastrinoma is presented by numerous peptic ulcers that do not respond to conservative therapy with proton pump inhibitors, diarrhoea, abdominal pain. Vipomas are presented with long lasting and abundant watery diarrhoea. For patients with glucagonoma, four symptoms, dermatosis, diabetes, deep venous thrombosis and depression are typical. Patients with somatostatinoma do not have specific symptoms, but most commonly, abdominal pain and weight loss. Given the many imprecise symptoms that are presented, sometimes it is quite difficult to suspect what the tumor is, but it should always be considered in differential diagnosis. Many diagnostic methods can be used to diagnose tumors. Chromogranin A is a biochemical marker that can be detected in tumor cell granules. CT, MR, USV, EUS, 18FDG positron emission and somatostatin receptor expression are used for image methods. To detect the primary tumor in the pancreas, the most important is the endoscopic ultrasound, to determine the exact location of the tumor and its extension to the surrounding structures. We can also use octreoscan, 68G positron emission and pathohistological analysis. It is essential in the treatment that surgical removal of tumor tissue is as effective as possible. Somatostatin, interferon alfa, chemotherapeutic agents such as cisplatin or carboplatin combined with etoposide, and mTOR inhibitors such as everolimus and VEGF inhibitors are also used. |
