A malignant peripheral nerve sheath tumor in association with a paratesticular ganglioneuroma

A malignant peripheral nerve sheath (PNS) tumor and a benign ganglioneuroma were present as a composite tumor in the paratesticular area of a 15-year-old boy. The pathologic diagnosis was made by characteristic histologic and ultrastructural features and was supported by the demonstration of neuron-specific enolase (NSE)-positive ganglion cells and S-100-protein-positive spindle cells consistent with schwannian cells. The patient has remained disease-free for 3 years since the orchiectomy and the retroperitoneal lymph node dissection, which was followed by combination chemotherapy.