Nerve Sheath Myxoma in the Lower Extremity: A Rare Case with Description of Magnetic Resonance Imaging and Sonographic Findings

Objective: Rare disease Background: This report is of a nerve sheath myxoma presenting as a slow-growing mass in the back of the left ankle of a 36-year-old man that was investigated by ultrasound and magnetic resonance imaging (MRI) before the diagnosis was confirmed by histopathology. Case Report: We report a nerve sheath myxoma of the ankle in a 36-year-old man. The palpable abnormality was falsely assumed to be a ganglion cyst prior to advanced imaging. Magnetic resonance imaging demonstrated a lobular mass with high T2 and intermediate T1 signal as well as moderate enhancement. T2 sequences also demonstrated distinctive internal septae. These internal septae were also noted on sonographic evaluation prior to biopsy. The patient was treated with surgical excision, and pathologic analysis showed myxoid nodules with loose arrangements of spindled cells separated by fibrous septae. S-100 protein and glial fibrillary acidic protein positivity by immunohistochemistry staining was demonstrated. Follow-up imaging at 12 months showed no evidence of tumor recurrence. Conclusions: This case highlights that while nerve sheath myxomas are rare tumors, they should be considered in cases of cutaneous soft-tissue masses with myxoid imaging features. Ultrasound and magnetic resonance imaging features of thin internal septae may be present and correspond well with the unique histopathological characteristics of these lesions. This report shows the importance of imaging of peripheral soft-tissue masses, including ultrasound and MRI, which can identify localized and benign features and the solid, cystic, and myxoid areas, which were characteristic in this case of benign nerve sheath myxoma.