Melanotic Neuroectodermal Tumor of Infancy

Melanotic neuroectodermal tumor of infancy (MNTI) is a rare, rapidly growing, and locally aggressive tumor of neural crest origin. More than 90% of MNTI occurs in infancy, with a median age of about 5 months, slightly higher in males than females. With the anterior maxilla (>60%) being the most prevalent, followed by the skull, mandible, and brain, with occasional reports in the mediastinum, ovaries, uterus, testes and epididymis, limb bones, and soft tissues [1–11]. MNTI now has no clear treatment guidelines, and surgical resection is considered as the best therapy because the tumor rarely metastasizes to distant sites [12–15]. But, local recurrence is common, and the scope of surgical excision remains controversial.