Malignant transformation in a sciatic plexiform neurofibroma in Neurofibromatosis Type 1 - imaging features that aid...

A 41-year-old Asian male with NF1 and bilateral sciatic plexiform neurofibromas, presented with unintentional weight loss, increasing size of a left thigh mass associated with increasing pain and radiculopathy. MRI of the left thigh demonstrated imaging features suspicious of malignant transformation. The patient had a new left lung mass, demonstrating avid FDG uptake, raising suspicion for metastasis. Surgical resection of the left thigh mass confirms malignant transformation in a preexisting sciatic plexiform neurofibroma. Diagnosis of malignant transformation in a nerve sheath tumour can be challenging. MRI remains the main preferred imaging modality in the evaluation of these tumours. Imaging features that raise suspicion for malignant transformation are discussed. Although none of these are specific for malignant transformation, studies suggest that the presence of two to four of these features should prompt further investigations.