Hosting 708 files, 2884 images, and 4 contributors.

 
Random Case

Congenital C6 Spondylolysis
Joseph Junewick, MD FACR
over 5 years ago
Enter A Workflow
Standard2   Academic2

Please choose a workflow. A standard workflow allows you to browse the repository with full case detail; the academic workflow allows you to browse the repository with limited case detail revealed. Double click on the images to launch image viewer.

ARSt Case Repository

Plexiform Neurofibroma

Case Detail

Anatomy: Gastrointestinal
Junewick
Joseph Junewick, MD FACR
Diagnostic Category: Neoplasia Benign
Created: over 5 years ago
Updated: over 5 years ago
Tags: PEDS
Modality/Study Types: US MR CT
Activities:
Adobe32 PDF Imagej32 ImageJA

History

13 year old female with neurofibromatosis.


Case Images


Diagnosis

Plexiform neurofibromatosis

Findings

CT – infiltrative hypodense lesion extending from the porta hepatis along the portal tracts into the liver.

MRI – SSFSE2 hyperintense and nonenhancing postgadolinium FST1 lesion and otherwise normal abdominal viscera.

US – heterogeneous, predominantly hypoechoic periportal mass.

Discussion

Neurofibroma of the porta hepatis is rare; most abdominal involvement of neurofibromatosis involves the gastrointestinal tract. The plexiform neurofibroma extends along the perihepatic nerves accompanying the hepatic ducts and vessels. On CT the neurofibroma is hypodense and on MR it is T1 hypointense and T2 hyperintense with no distortion of perihepatic vascularity.

Perihepatic and hepatic tumors associated with neurofibromatosis type I include: benign and malignant schwannomas, angiosarcoma, neurofibrosarcoma, and hepatoma. Additional differential considerations include: lymphoma, mesenchymal tumors and cholangiocarcinoma.

Reference

Malagari K, et al. Plexiform neurofibroma of the liver: Findings on MR imaging, angiography, and CT Portography. AJR 2001; 176:493-495.



Corporate Office: 616.363.7272, 3264 North Evergreen Drive, Grand Rapids, MI 49525

Spectrum Health Helen Devos Childrens Hospital GE HealthCare