Long-term response with adjuvant chemotherapy for recurrent Melanocytic Neuroectodermal Tumor of Infancy

Abstract Melanocytic neuroectodermal tumor of infancy (MNTI) is a rare, pigmented tumor generally occurring in the head and neck region in children 12 months of age or younger. As of 2016, only 472 cases have been reported in the literature. Although usually a benign tumor, MNTI can demonstrate locally invasive behavior and requires surgical resection as a first-line treatment. Only around 4% of all reported cases in the literature have included chemotherapy at any stage in the treatment of MNTI. This has been primarily limited to its use as an adjuvant in patients with metastases or recurrent disease, often with limited benefit. We report here a child who remains disease-free at the 7-year follow-up, having been successfully treated using an OPEC/OJEC regimen for recurrent MNTI following incomplete surgical resection. We present a review of the literature and discuss the surgical and chemotherapeutic complications that arose in the management of this case to help inform future management of patients.