Abstract

Melanotic neuroectodermal tumor of infancy (MNTI) is a rare, fast-growing tumor with a high recurrence rate. Commonly it develops in the anterior maxilla of children younger than age 1 year. Tumor origin is the neural crest. Girl, 2 months, had an increasingly large area of swelling on the left cheek. After tomography and biopsy, the diagnosis of MNTI was established. Histological examination indicated a remarkable combination of neural, melanocytic, and epithelial cell differentiation. One year after tumor excision, the child has had no recurrence. Since its first description, about 260 cases of this tumor have been reported. Early diagnosis minimized difficulties and risks during treatment and leads to an optimal outcome. Despite complete surgical excision, careful follow-up is recommended. Maxillary functional orthopedic care and reconstruction may be necessary.

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