Axial T1-weighted (image 1) and axial T1-weighted postcontra | Figure 1

CincyKidsRad

Cincinnati Children's Radiology

Axial T1-weighted (image 1) and axial T1-weighted postcontrast (image 2) MR images highlighting the malignant peripheral nerve sheath tumor (arrow). MRI features that can be used to help differentiate a malignant peripheral nerve sheath tumor from a plexiform neurofibroma include loss of a target appearance on T2-weighted images, inherent T1 hyperintense signal, and heterogeneous enhancement. #FridayQuizDay #FridayQuiz

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Axial T2-W image in a child with neurofibromatosis type 1, shows a large plexiform neurofibroma of the pelvis. The plexiform neurofibroma is composed of multiple lesions, each with a characteristic target appearance. The target appearance occurs because the neurofibroma contains a central area of dense collagen. The target sign is not always present in plexiform neurofibromas. However, if a tumor loses the sign, this is considered to be a finding of degeneration to a malignant peripheral nerve sheath tumor. Other findings of malignant degeneration include heterogeneous enhancement, lobulation of the tumor, and rapid change in size, and irregular margins.
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Coronal T2 weighted image from a whole body MRI (image 1) shows extensive plexiform neurofibromas involving the upper extremities. Post contrast T1 weighted MRI (image 2) shows a focal area of enhancement (arrow) of one portion of the plexiform neurofibroma concerning for degeneration to a malignant peripheral nerve sheath tumor. PET/CT (image 3) highlights FDG uptake (arrow) within the small tumor. Malignant peripheral nerve sheath tumor is a type of sarcoma. 50 to 60% of patients with malignant peripheral nerve sheath tumor have neurofibromatosis type I.


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Axial (image 1) and coronal (image 2) T2-weighted and axial T1-weighted postcontrast (image 3) MRI in a child with abdominal distention shows a large heterogeneous left Wilms tumor (arrows). The mass is displacing the renal parenchyma (arrowhead) peripherally. Wilms tumor is the second most common extracranial solid malignancy in children. The majority of cases occur in patients younger than age 5. When evaluating patients with Wilms tumor, it is important to determine if the renal vein has been invaded.