Melanotic Neuroectodermal Tumor of Infancy: A Case Report and Literature Review

Melanotic neuroectodermal tumor of infancy (MNTI), first described almost a century back, is of neural crest origin, locally aggressive, a rare biphasic neoplasm of infancy with a slightly higher male preponderance. In the last 100 years since the first description of MNTI, only around 500 cases have been described from 32 countries. We present a 7-month-old female child with 3 × 2 cm hard swelling in the oral cavity and right-side facial region for 3 months. Contrast-enhanced computed tomography scan and contrast-enhanced magnetic resonance imaging scan revealed a hypodense lesion of size 2.5 × 2 × 1.6 cm with relatively well-corticated walls. The lesion appeared to arise from the right maxillary alveolus with erosion of the floor of the maxillary sinus. A 7 × 5 mm tooth was visualized within the lesion. There was minimal enhancement in the postcontrast study. With the following provisional diagnoses-odontogenic keratocyst, dentigerous cyst, and unicystic ameloblastoma-the child underwent excision of the lesion. Intraoperatively, Hopkin's rigid endoscope 4 mm was used to ensure complete tumor removal in the maxillary sinus. Histopathological and immunohistochemistry examination resulted in the diagnosis of MNTI. On 1-year follow-up, the child did not show any signs of recurrence. A high index of suspicion, early diagnosis, and timely treatment are needed to diagnose such a rare tumor, to avoid morbidity, and to plan effective management when an infant presents with facial swelling. It should be complemented with close follow-up to identify recurrence early. Use of endoscope whenever feasible is encouraged by the authors to ensure adequate tumor removal.