Melanotic neuroectodermal tumour of the mandible:A case report

Objectives: Melanotic neuroectodermal tumour (MNT) of infancy is an uncommon pigmented neoplasm of neural crest origin. The mandible localisation is extremely rare .How can we diagnose it ?And what’s the therapeutic approach ?
Methods: We describe a rare case of melanotic neuroectodermal tumour of the mandible, in a six-month-old child. The diagnosis was made through a local biopsy and a computed tomography exploration .The treatment was based on neoadjuvant chemotherapy (6 cures) than surgical removal after one year.
Results: After 2 years follow up the child is doing well ,the aesthetic aspect is very satisfying with no signs of recurrence radiologically and clinically
Conclusions: Despite being a benign entity, the MNT is locally aggressive and has a significant recurrent risk. The treatment is based on chemotherapy (neuroblastoma protocol) and surgery.