Vascular liver tumors

Vaskularni tumori jetre su skupina neoplazmi koja je vrlo raznolika po svojoj učestalosti, simptomima i prognozi. Kavernozni hemangiom je najčešći benigni tumor jetre i druga najčešća jetrena neoplazma. Većinom ima promjer ispod 5 cm i ne izaziva simptome. Može se prezentirati s boli u abdomenu, anoreksijom i hepatomegalijom, a rjeđe poremećajima zgrušavanja ili kompresijom okolnih struktura. Terapija je rijetko potrebna i često je samo simptomatska, a u slučaju perzistentnih ili progresivnih simptoma ili rizika od rupture potrebno je učiniti kiruršku eksciziju. Primarni angiosarkom jetre vrlo je agresivan maligni tumor incidencije ispod 0,1 na 100 000 stanovnika. Simptomima oponaša kroničnu bolest jetre, a u vrijeme dijagnoze često već stvara metastaze. Kako još uvijek ne postoji efektivna terapija, prognoza je loša i većina pacijenata ne preživi dulje od godinu dana. Kirurška resekcija nešto produljuje preživljenje. Epiteloidni hemangioendoteliom je tumor koji slijedi klinički tok između hemangioma i angiosarkoma. U jetri ima incidenciju ispod 0,1 na 100 000 stanovnika. Prezentira se nespecifičnim simptomima poput boli u gornjem desnom kvadrantu abdomena, a može biti i asimptomatski. Transplantacija jetre nudi dobre rezultate, a modaliteti liječenja uključuju i kiruršku resekciju, TACE te kemoterapiju. Kavernozni hemangiom u zdravoj jetri moguće je dijagnosticirati ultrazvukom, dok je MRI ili CT potreban za epiteloidni hemangioendoteliom i angiosarkom. Biopsija i imunohistokemijsko bojanje za vaskularne faktore (CD34, CD31, faktor VIII) razjašnjavaju nesigurne dijagnoze. Cilj ovog rada je dati preglednu sintezu nedavnih spoznaja o patogenezi, kliničkoj slici, dijagnostici i liječenju ovih jetrenih novotvorina.Vascular tumors of the liver are a group of neoplasms that vary greatly in presentation, prognosis and incidence. Cavernous hemangioma is the most common benign liver tumor, and second most common liver neoplasm overall. While almost exclusively smaller than 5 cm in diameter and asymptomatic, it can present with abdominal pain, anorexia, hepatomegaly or, rarely, coagulopathy and compressive symptoms. Treatment is seldom necessary and often merely symptomatic, while surgical resection is indicated in cases with a risk of rupture or persistent and progressive symptoms. Primary hepatic angiosarcoma is very aggressive malignant tumor with an incidence of less than 0.1 per 100,000. It mimics chronic liver disease, and is often metastatic at the time of diagnosis. As there is still no effective therapy, prognosis remains dismal with overall survival rarely higher than one year. Surgical resection can somewhat increase survival. Epithelioid hemangioendothelioma is a tumor that pursues a clinical course intermediate between a hemangioma and conventional angiosarcoma. As a liver tumor, it has an incidence of less than 0.1 per 100,000 and presents with unspecific symptoms such as upper right quadrant pain, but can also be asymptomatic. Liver transplant offers good results, while other treatments include surgical resection, TACE and chemotherapy. Cavernous hemangioma can be diagnosed in an otherwise healthy liver using only ultrasound, while MRI or CT is necessary for epithelioid hemangioendothelioma and angiosarcoma. Biopsy with immunohistochemical staining for vascular factors (CD34, CD31, factor VIII) should be used to clarify uncertain diagnosis. This paper is a systemic review of the recent discoveries in the parthenogenesis, clinical presentation, diagnosis and treatment of these liver tumors