Hepatocellular Carcinoma in a Boy with Progressive Familial Intrahepatic Cholestasis Type II: Challenging Identification : Case Report

Hepatocellular carcinoma (HCC) is rare in children. Progressive familial intrahepatic cholestasis type II (PFIC2 and also called BSEP ((Bile Salt Export Pump)) deficiency) is an inherited disease that initiates end-stage liver cirrhosis which can predispose to HCC. HCC can occur in 15% of patients w...

Full description

Saved in:
Bibliographic Details
Published in:International journal of health sciences 2013-06, Vol.7 (2), p.252-255
Main Author: AlSalloom , Abdulaziz
Format: Article
Language:English
Subjects:
ANATOMY
BILIARY SYSTEM
Case Report
DIAGNOSIS
LIVER DISEASES
MAGNETIC RESONANCE IMAGING
NEOPLASMS
PATIENTS
RADIOTHERAPY
THERAPY
أمراض الكبد
الأورام
التشخيص
التشريح
التصوير بالطنين المغنطيسي
الجهاز الصفراوي
العلاج
العلاج بالأشعة
المرضى
Citations: Items that cite this one
Online Access:Get full text
Tags: Add Tag
No Tags, Be the first to tag this record!
Description
Summary:Hepatocellular carcinoma (HCC) is rare in children. Progressive familial intrahepatic cholestasis type II (PFIC2 and also called BSEP ((Bile Salt Export Pump)) deficiency) is an inherited disease that initiates end-stage liver cirrhosis which can predispose to HCC. HCC can occur in 15% of patients with PFIC2. In this case report, an 11-month-old boy with PFIC 2 was admitted for liver transplant work up. The finding of HCC was made incidentally by histopathology on the explanted liver after suspiciously gross examination. In this article, we found that the radiology (US) alone is not enough to exclude HCC. Finally, we conclude that any case of PFIC 2 (male or female) needs routine screening of serum AFP concentration, advanced radiological examination (CT, MRI) as well as careful macroscopic examination of their explanted liver (triple assessment) to exclude HCC.
ISSN:1658-3639
1658-7774
DOI:10.12816/0006048