Melanotic neuroectodermal tumor of infancy (MNTI) is an uncommon osteolytic, pigmented neoplasm involving craniofacial sites, especially the maxilla of newborn infants. Since the first report by Krompecher in 1918, approximately 400 cases have been reported to date.We present a case of MNTI occurring in the maxilla of a 3-month-old girl. On initial examination, a grayish bony enlargement was noted at the left side of the anterior maxilla. Plain CT scanning demostrated two cyst-like radiolucent lesions measuring 15 mm and 10 mm in diameter and involving the central and lateral deciduous incisors.The clinical diagnosis was a maxillary tumor. Enucleation of the tumor was performed with the patient under general anesthesia. The tumor was lobulated and black and included two deciduous teeth. Histopathollogically, the tumor showed a nest-like proliferation of epithelial cells and small round cells with dense fibrous connective tissue. Most epithelial cells contained melanin pigments.On immunohistochemical staining of the excised tumor, NSE, synaptophysin, AE1/AE3, vimentin and HMB45were positive, and GFAP, NFP, MIC2, EMA, desmin, CEA, and S-100 protein were negative. On the basis of these findings, the tumor was finally diagnosed as MNTI. There has been no sign of recurrence as of 2 years after operation.
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