Orbital melanotic neuroectodermal tumor of infancy successfully treated with chemotherapy and subtotal excision

Melanotic neuroectodermal tumor of infancy is a rare condition that typically presents within the first 6 months of life. It rarely metastasizes but is locally aggressive. Melanotic neuroectodermal tumor of infancy has been reported under several other names including retinal anlage tumor and melanotic progonoma. Most commonly, melanotic neuroectodermal tumor of infancy originates in the maxilla. Tumors in previously reported orbital cases have arisen from adjacent structures or from orbital bone. Recommended treatment of melanotic neuroectodermal tumor of infancy has consisted of complete excision with wide tissue margins, although success has been reported after incomplete resection. For those patients in whom surgical resection is not possible, various chemotherapy approaches have been reported with mixed results ranging from failure to sustained tumor regression. We report a case of orbital melanotic neuroectodermal tumor of infancy adherent to sclera and successfully treated with chemotherapy and subtotal excision.