Abstract:

Melanotic neuroectodermal tumor of infancy (MNTI) is a rare, rapidly growing, and pigmented neoplasm of neural crest origin. It predominantly affects the maxilla of infants during the first year of life. A seven-month-old boy presented with a mass approximately 5 cm in diameter in the right oral cavity. On computerized tomography, a lytic expansile lesion was detected in the right maxilla. Microscopically, the tumor consisted of two different neoplastic cell proliferation, located peripherally and centrally, arranged in alveolar clusters within the fibrous connective tissue. Immunohistochemically, peripheral tumor cells showed diffuse staining for Pancytokeratin and HMB-45, the central cells were positive for CD56 and Synaptophysin. MNTI is a rare tumor that can be easily confused with malign small round cell tumors, especially in small biopsies. It has characteristic histomorphological and immunohistochemical findings. Its biological behavior is not fully understood. These tumors can present locally aggressive behavior and a high recurrence rate.

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