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

Authors

Malgorzata A Krawczyk, Ewa Bien, Jelena Roganovic, Daniel Orbach, Dominik T Schneider, Michaela Kuhlen, Tal Ben-Ami, Andrea Ferrari, Maja Cesen Mazic, Calogero Virgone, Ricardo Lopez-Almaraz, Gianni Bisogno, Michael Abele, Malgorzata Styczewska, Cecile Faure-Conter, Rita Alaggio, Celine Richard, Theodore W Laetsch, Ines B Brecht, Yves Reguerre

Published in

Pediatric blood & cancer. Pages e70458. Jul 29, 2026. Epub Jul 29, 2026.

Abstract

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.

PMID:
42527893
Bibliographic data and abstract were imported from PubMed on 30 Jul 2026.

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