ABSTRACT
The melanotic neuroectodermal tumor of infancy (MNTI) is a rare childhood neoplasm with an alarming but classical clinical presentation. We present the case of a 2-month-old male infant treated with surgery for an aggressive MNTI on the alveolar process of the maxilla. Radiographic examination showed a diffuse osteolytic radiolucent lesion in the right maxilla, and displacement and dysmorphic changes in the developing primary tooth buds. The patient remained well without evidence of recurrence for 6 months after surgery. We discussed clinicopathological features, management alternatives, and outcome.
Subject(s)
Maxillary Neoplasms/pathology , Neuroectodermal Tumor, Melanotic/pathology , Biomarkers, Tumor/analysis , Humans , Immunohistochemistry , Infant , Male , Maxillary Neoplasms/surgery , Neuroectodermal Tumor, Melanotic/surgery , Oral Surgical ProceduresABSTRACT
A melanotic neuroectodermal tumor of infancy (MNTI) is a uncommon osteolytic pigmented neoplasm that primarily affects the jaws of newborn infants. Most patients (> 90%) present with the tumor in the first year of life. Approximately 65% form in the maxilla, 11% in the mandible, 5% in the brain and elsewhere. MNTI is normally benign, but up to 15% may recur and a few have metastasized. Approximately 200 cases of MNTI have been reported but only 2 of them presented as multifocal. A case of MNTI in a 7 month old boy was encountered. The chief complaint was maxillary anterior ridge swelling. The incisional biopsy findings were MNTI. Two months after the first operation, mild swelling of another site was observed. The infant was examined periodically since undergoing two procedures with no recurrence. This case demonstrates the possibility of a multicentric MNTI. We report a multicentric MNTI with a review of the relevant literature.
