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Coagulopathy of the Newborn
Joseph Junewick, MD FACR
over 7 years ago
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Case History


13 year old female with neurofibromatosis.


Case Detail

Anatomy: Gastrointestinal
Junewick
Joseph Junewick, MD FACR
Diagnostic Category: Neoplasia Benign
Created: over 8 years ago
Updated: over 8 years ago
Tags: PEDS
Modality/Study Types: US MR CT
Activities:
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Case Images


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Reveal 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.

Reveal 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.

Reveal Diagnosis

Plexiform neurofibromatosis

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