Melanotic Neuroectodermal Tumor Of Infancy (MNTI)

A Case Report

Résumé

Background: Melanotic neuroectodermal tumor of infancy (MNTI) is a rapidly growing benign tumor arising from the neural crest. Clinical case: A 5-month-old boy presented with left-sided frontotemporal swelling for 3 months. It was non-tender, firm, and had no skin changes. It was rapidly progressive (4 cm in size at the time of presentation of the patient). No history of alteration in consciousness or symptoms of raised intracranial pressure was noted. On examination, he was alert and had obvious scalp asymmetry, with an obvious firm mass noted on the left frontotemporal region. It was not attached to overlying skin, had no associated skin changes, and was neither fluctuant nor non-tender. It was 4 by 4 cm in size. Imaging revealed a left frontotemporal mixed density lesion. A near-total resection was done of a firm mixed density fibrous tumour with a bluish-black hue, found to involve the fronto-temporal calvarium, abutting the dura. Histology revealed a widely infiltrating biphasic tumour composed of small round blue cells admixed with larger epithelioid cells arranged in cords and nests bearing abundant pale cytoplasm and abundant cytoplasmic melanin pigment. The background had dense fibrosis. Conclusion: MNTI is a rare benign tumour that may cause rapid progression and facial and scalp disfigurement. A high index of suspicion is warranted once a patient with similar features is encountered.