Melanotic neuroectodermal tumor of infancy

A 6-month-old female presented with a swelling of the left superior maxillary bone. A radiograph revealed a radiolucent lesion in the left anterior maxilla, and CT, showed a solid mass. At surgery, the lesion was a cystic dental tumor of ectopic location which was treated by excision. The pathological diagnosis was melanotic neurorectodermal tumor. This is an uncommon benign tumor of the neural crest origin that occurs mainly in the maxilla (70% of cases) but can occur in other areas such as the skull and the mandible. CT reveals a hyperdense mass and MR shows a hypointense mass on T1-weighted images and an iso-intense mass on T2-weighted images. The evolution is usually benign after surgical removal.