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.
Institute of Pathology, University of Padova, Padova, 44100, Italy. altavill@ux1.unipd.it.
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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