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Plexiform Neurofibroma
Case Detail
| Anatomy: Gastrointestinal |
 Joseph Junewick, MD FACR |
| Diagnostic Category: Neoplasia Benign |
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| Created: over 3 years ago |
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| Updated: over 3 years ago |
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| Tags:
PEDS
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| Modality/Study Types:
US
MR
CT
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Activities:  PDF  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.