Plexiform Neurofibromatosis of the Liver: An Extremely Rare Case

Herein, we report an extremely rare case of histopathologically proven neurofibromatosis of the liver. A 15-year-old male, a known case of type I neurofibromatosis (NF1), referred to our hospital with a complaint of right upper quadrant pain. He had a café-au-lait spot and positive family history of NF1 in her mother. Laboratory data were within normal limits, and computed tomography (CT) revealed a large predominantly less attenuated infiltrative liver mass along the porta hepatis with extension to both lobes of the liver. Moreover, magnetic resonance imaging showed a large hypo signal mass in T1-weighted images and hypersignal lesion in T2-sequences with faint enhancement, periportal distribution, and encasing of major branches of the portal vein without evidence of narrowing and invasion. Furthermore, the CT guided biopsy was taken from both liver lobe lesion, and pathological diagnosis of the biopsy specimens confirmed plexiform neurofibromas of the liver. According to the extensive intrahepatic extension and periportal infiltration, the mass was unrespectable. Therefore, the radiologists need to be familiar with the typical imaging features of the uncommon hepatic neoplasms. If imaging findings are not typical or diagnostic, a further biopsy should be performed once again.