Melanotic neuroectodermal tumour of infancy: report of two cases and review of the literature

Abstract

Melanotic neuroectodermal tumour of infancy (MNTI) is an uncommon tumour affecting predominantly the craniofacial bones of the newborn infants. The neural crest origin of the tumour has been confirmed. MNTI is generally accepted as a benign tumour despite of its rapid and locally infiltrative growth. Recurrence rate varies between 10% and 60%, and malignant behaviour has been reported in 6.5% of MNTIs. Systematic review of the literature revealed 445 MNTIs published between 1918 and 2010. We present additional two cases of MNTI from our Department, typical in all terms, which equals a total number of 447 reported cases. One of our cases revealed histological features consistent with malignant behaviour, but at present, 18 months after the surgical excision, there is no evidence of recurrence. Biological behaviour of MNTI cannot be predicted by gross or histologic characteristics, thus early diagnosis and careful follow-up after the complete surgical excision is required.