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

Abstract

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.

Keywords

biphasic neoplasm of infancy - Hopkin's rigid endoscope - infantile neoplasm - melanotic neuroectodermal tumor of infancy (MNTI) - vanillylmandelic acid (VMA)

Note

The study is reported out after taking proper consent from the patient's caregivers and conforming to the Declaration of Helsinki.

Publication History

Article published online:
31 July 2023

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