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Plexiform Neurofibroma of the Liver

Findings on MR Imaging, Angiography, and CT Portography

Katerina Malagari1, Spyros Drakopoulos2, Elias Brountzos1, Athanasios Sissopulos1, Aikaterini Efthimidadou3, Evangellos Hadjiyiannakis2 and Dimitrios A. Kelekis1

1 2nd Department of Radiology, University of Athens Medical School, 19 Monis Kyccou, 15669 Papagou, Athens, Greece.
2 1st Department of Surgery and Transplantation Unit, University of Athens Medical School, Evangelismos General Hospital, Hipsilandou 45-47, 11528 Athens, Greece.
3 Department of Pathology, Evangelismos General Hospital, 11528 Athens, Greece.



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Fig. 1A. 24-year-old man with type 1 neurofibromatosis who was referred for evaluation of liver mass. T1-weighted in-phase gradient-echo MR image (TR/TE, 10/4.6; flip angle, 30°) shows low-signal-intensity mass in left hepatic lobe extending through porta hepatis.

 


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Fig. 1B. 24-year-old man with type 1 neurofibromatosis who was referred for evaluation of liver mass. Fast spin-echo fat-suppressed T2-weighted axial MR image (1800/80) clearly depicts encasement of celiac trunk (large straight arrow) and hepatic artery (small solid arrows). Tumor is hyperintense with macronodular appearance and hypointense centers (open arrow). Diaphragmatic involvement is also seen anteriorly (curved arrow).

 


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Fig. 1C. 24-year-old man with type 1 neurofibromatosis who was referred for evaluation of liver mass. Arteriogram of liver reveals slight tortuosity of distal branches of left hepatic artery (solid arrow). Slightly increased vascularity of extrahepatic component supplied by gastroduodenal artery (open arrow) is seen.

 


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Fig. 1D. 24-year-old man with type 1 neurofibromatosis who was referred for evaluation of liver mass. CT portogram shows low-attenuation, nonenhancing multilobulated tumor with relatively well-defined margins extending throughout segments II, III, and IV. Periportal location of mass with preservation of normal vessel distribution and vessel patency is well appreciated. Diaphragmatic thickening (arrows) by neurofibromas can also be seen.

 


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Fig. 1E. 24-year-old man with type 1 neurofibromatosis who was referred for evaluation of liver mass. CT portogram at level lower than D shows extrahepatic component encasing patent hepatic artery (arrows) up to its origin from celiac trunk.

 


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Fig. 1F. 24-year-old man with type 1 neurofibromatosis who was referred for evaluation of liver mass. CT portogram obtained peripheral to tumor clearly shows periportal distribution (arrows).

 

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