Melanotic Neuroectodermal Tumor of Infancy in the Epididymis: A Brief Report and Review of the Role of Chemotherapy in Management.

Melanotic neuroectodermal tumor of infancy is a rare benign tumor of neural crest origin. The tumor generally presents in the jawbones; however, it occasionally occurs in extracranial sites. Although 95% of these tumors present within the first year of life and 15% in extracranial locations, we report an unusual case of a 15-month-old male with melanotic neuroectodermal tumor of infancy of the epididymis. The patient underwent orchiectomy without adjuvant chemotherapy or radiation. Twenty months later, there was no sign of recurrence. In addition, we discuss the role of chemotherapy and radiation and the potential importance of molecular genetics in establishing guidelines for management.