Melanotic neuroectodermal tumor of infancy: a wolf in sheep's clothing.

INTRODUCTION: The melanotic neuroectodermal tumor of infancy (MNTI) is an exceptionally rare neoplasm. Despite their potential for local invasion, MNTI are considered benign neoplasms. Sporadic cases have been reported of MNTI acting in a malignant fashion; however, the majority of these tumors were of extragnathic origin. METHODS: A 2-month-old male presented with a rapidly expanding maxillary mass. The patient underwent excisional biopsy of the mass and a diagnosis of MNTI was returned. Tumor recurrence was appreciated postoperatively. Neoadjuvant chemotherapy was initiated to render it more amenable to surgical resection. Chemotherapy was suspended prematurely and the patient returned to the operating room for hemimaxillectomy. The postchemotherapy surgical pathology report demonstrated a unique mixed chemotherapy response. DISCUSSION: This report highlights the aggressive nature of gnathic MNTI, the importance of early diagnosis and definitive intervention, and the mixed clinical and histologic response of the lesion to neoadjuvant chemotherapy.