Sestre Milosrdnice University hospital and Institute of Clinical Medical Research
Abstract
Hemangiopericytoma is a rare malignant vascular tumor deriving from pericytes. It usually occurs in the extremities and retroperitoneum, and extremely rarely in the gastrointestinal tract. A case of hemangiopericytoma in the gastroesophageal region is reported. A 56-year-old man was treated with H2-receptor antagonist for dyspeptic symptoms that had occurred three months before. Therapy failed to prove successful and gastroendoscopy was performed in March 2001 to reveal slightly reddish ventricular mucosa with normal rugal folds and a sessile, polypoid, grayish mass of rough surface located at the gastroesophageal junction. Histopathologic examination of polypoid mass samples revealed a vascular tumor covered with squamous epithelium. The hallmarks of the tumor were numerous branching, slit-like capillary channels with "staghorn" appearance, and large gaping sinusoidal spaces enclosed within nests of ovoid cells. Vascular invasion, mitotic activity, giant cells and necrosis were not found. Immunohistochemical analysis showed diffuse and strong reactivity for vimentin and focal, low or moderate reactivity for actin, S-100 and smooth muscle actin. Proliferation index measured by PCNA and Ki-67 ranged from 2% to 13% (median 11.2%) and from 5% to 18% (median 7.6%), respectively. A diagnosis of hemangiopericytoma with low malignant potential was made. Almost two years of the intervention, the patient is alive and free from any signs of tumor spread.Hemangiopericitom je rijedak maligni vaskularni tumor koji potječe od pericita. Najčešće je javlja na ekstremitetima, te u području peritoneuma, a iznimno rijetko ga se nalazi u probavnom sustavu. Opisuje se slučaj hemangiopericitoma nađenog u gastroezofagusnom području. Bolesnik star 56 godina liječen je tri mjeseca antagonistima H-2 receptora zbog dispeptičnih problema. Preporučena terapija nije pokazala očekivani rezultat, pa je učinjena gastroskopija. Nađena je sesilna polipozna tvorba veličine novčića, sivkaste boje i neravne površine u gastroezofagusnom području. Histopatološki je odstranjena tvorba bila građena od razgranatih kapilarnih prostora tipa "staghorn", okruženih gnijezdima ovalnih stanica koje su tvorile solidan uzorak. Nekroze, vaskularna invazija, velike stanice i mitoze nisu nađene. Tumorske stanice su pokazivale difuznu, izrazito pozitivnu imunoreakciju na vimentin, dok je reakcija na aktin, S-100 i glatkomišićni aktin bila žarišna i slabog intenziteta. Proliferacijska aktivnost tumora iznosila je između 2% i 13% (medijan 11,2%) mjerena pomoću PCNA, te između 5% i 18% (medijan 7,6%) mjerena pomoću Ki-67. Postavljena je dijagnoza hemangiopericitoma niskog malignog potencijala. Skoro dvije godine nakon postavljene dijagnoze bolesnik je bez znakova bolesti
