The secondary case of melanotic neuroectodermal tumor in the infant maxilla: Long-term observation after the enucleation

Melanotic neuroectodermal tumor of infancy (MNTI) is an extremely rare tumor with head and neck lesions. It is almost always diagnosed within the first year of age. MNTI is classified as a benign tumor by the World Health Organization (WHO) and develops in the cranio-maxillo-facial region. Since some reports described the local relapse or distant metastasis like a malignant tumor, we here present the secondary case of a 3-month-old boy with maxillary MNTI and the good prognosis in the follow-up over a 10-year span. He underwent two surgeries. The first operation was performed at another hospital, but his left face enlarged again after 2 months. When he visited our hospital, the tumor seemed to invade his left pre-maxillary to maxillary region and nose. Based on the clinical exam, we diagnosed his symptom as a relapse, and therefore we again performed enucleation under general anesthesia. The tumor was located around incisor canal and left posterior maxillary region occupying the left nasal passage. Pathologically, the tumor was composed of neuroblastic and epithelial-like cells with melanin, which was immunohistochemically stained as positive for epithelial and neural markers, resulting in MNTI. Postoperative prognosis has no sign of relapse. However, he presented with missing permanent teeth and suppressed maxillary growth due to the surgeries which led to skeletal reversed occlusion. To support orthognathic treatment, we recently performed a bone graft using chin bone. Our case report suggests that the aspects of functional or aesthetic disorders following enucleation require attention in the treatment of MNTI.