Hepatocellular Carcinoma with Congenital Absence of the Portal Vein in a Child

Nurdan Tacyildiz Emine Gulsah

doi:10.4172/2165-7920.1000504

Abstract

Congenital absence of the portal vein (CAPV) is such a rare malformation of the splanchnic venous system. Also it can be a reason for hepatic tumors, for example adenoma, hepatoblastoma or hepatocellular carcinoma (HCC). The cooccurence of CAPV and HCC in adult patients have been reported in the literature. It is the second case of a pediatric patient whom has also CAPV and HCC.

Highlights

Congenital absence of the portal vein (CAPV) and its intra-hepatic branches is a malformation of the splanchnic venous system described by John Abernethy in 1973 [1]
We present a pediatric patient with concomitant occurrance of hepatocellular carcinoma (HCC) and CAPV
Hepatic viral infection like HBV, HCV or cirrhosis is related with hepatocellular carcinoma and it can take decades for malignancy to develop, because of this it is seen in very young children rarely

Summary

Introduction

Congenital absence of the portal vein (CAPV) and its intra-hepatic branches is a malformation of the splanchnic venous system described by John Abernethy in 1973 [1]. The patient was referred to pediatric oncology department of our hospital for further investigation She has a history of term birth with normal birth weight and she has no significant medical history. A search for tumor markers CA125: 55.1 U/ml (035) and HBsAg, anti-HBs antibodies and Anti-HCV antibodies were negative She had been investigated for metabolic disorders which can cause HCC, for example hereditary tyrosinemia and alpha-1 antitrypsin deficiency, serum tyrosin level: 142.44 μmol/L (32-275) and α-1 AT: 1. The smaller lesion was located in the segment 6 It was isodense with the liver parenchyma in the arterial phase and hypodense on delayed venous phase images. Microscopic examination demonstrated “Multifocal hepatocellular carcinoma, grade II” noting surgical margins and the liver capsule was tumor-free and base structure of the parenchyma was protected. The patient is doing well during 8 months follow-up

Discussion

Conclusion