Clinical Guidance for the Management of Melanotic Neuroectodermal Tumor of Infancy—A Consensus by the Expert Group

Melanotic neuroectodermal tumor of infancy (MNTI) is a rare neoplasm primarily affecting the craniofacial skeleton in infants. Management can be challenging in unresectable, multiply recurrent, or metastatic cases. Diagnosis requires local imaging assessment with magnetic resonance imaging (MRI) and computed tomography (CT) and histopathological confirmation. Surgery is the mainstay of treatment, achieving 80%–90% cure rates. Chemotherapy may be considered for advanced disease, whereas radiotherapy is generally avoided in young children. These recommendations were developed within European Cooperative Study Group for Pediatric Rare Tumors (EXPeRT) and European Reference Network Paediatric Cancer (ERN PaedCan) using a structured consensus process based on focused literature review and expert agreement. Multidisciplinary, risk-adapted management, and structured follow-up are essential.