Malignant peripheral nerve sheath tumors (MPNST) are rare, aggressive soft tissue sarcomas arising from peripheral nerves that present great challenges to known treatment modalities. Here, we present a case of MPNST involving the dorsum of a penis in a 13-month-old boy. The mass was incomplete excised and a circumcision was performed. Histological study revealed spindle cell tumor of the lesion. Immunohistochemistry showed a strong positive staining for tumor cells. A high grade malignant peripheral nerve sheath tumor was diagnosed.
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