Focal nodular hyperplasia of the liver associated with portal vein agenesis: a morphological and immunohistochemical study of one case and review of the literature.
Congenital absence of portal vein (CAPV) is a rare malformation. To our knowledge, sixteen cases are reported in western literature. All the cases are associated with other diseases, cardiac malformations (12/17 patients; 16 plus the present case) and hepatic neoplasms being the most frequent observations. We present the case of a girl with a complex malformative syndrome consisting of multicystic kidney dysplasia, CAPV and nodular tumor-like mass of the liver. Angiography showed that the splenic vein and superior mesenteric vein joined to form a common trunk directly entering the inferior vena cava above the liver. A review of the CAPV cases of the literature and the clinical and pathological features of the hepatic lesion, classified as Focal Nodular Hyperplasia (FNH), are extensively discussed.
Altavilla
G
G
Institute of Pathology, University of Padova, Padova, 44100, Italy. altavill@ux1.unipd.it.
Guariso
G
G
D000015:Abnormalities, Multiple / Q000473:pathology*; D014408:Biomarkers, Tumor / Q000378:metabolism; D002648:Child; D005260:Female; D020518:Focal Nodular Hyperplasia / Q000151:congenital* / Q000378:metabolism / Q000473:pathology; D006801:Humans; D007150:Immunohistochemistry; D008099:Liver / Q000002:abnormalities / Q000098:blood supply* / Q000378:metabolism / Q000473:pathology; D009363:Neoplasm Proteins / Q000378:metabolism; D007690:Polycystic Kidney Diseases / Q000151:congenital / Q000378:metabolism / Q000473:pathology; D011169:Portal Vein / Q000002:abnormalities* / Q000378:metabolism; D013577:Syndrome
D014408:Biomarkers, Tumor; D009363:Neoplasm Proteins