Presentation
8-year history of severe pain and edema of right lower limb without history of trauma or surgery during the past years.
Patient Data
Infiltrating mass lesion of the right lateral distal thigh and ipsilateral proximal knee with scalloping of the lateral femoral condyle and the tibial plateau, always externally. The lesion after contrast shows diffuse enhancement.
Histological report
Multinodular, plexiform tortuous bundles of bland spindle cells consistent with expanded nerve branches in a collagen and fibroblast matrix. Absent mitotic activity and nuclear atypia. Features consistent with neurofibroma.
Case Discussion
A plexiform neurofibroma is a benign peripheral nerve sheath tumor that surrounds multiple nervous fascicles and is a tumor occurring exclusively in neurofibromatosis1. It is a rare entity and is an uncommon variant of neurofibroma. A plexiform neurofibroma may rarely present as a diffuse infiltrating mass causing pressure erosion of the adjacent bone.
Case courtesy: Dr.ssa Sandra Pennacchini
Imaging technologist: TSRM Fabio Imola
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