Melanotic Neuroectodermal Tumor of Infancy: A Systematic Review

Melanotic neuroectodermal tumour of infancy – A rare entity

“…At 5 years after diagnosis, both sexes showed equal recurrence rates. 5 The overall incidence of local recurrence was 10-15%. 11 Currently, age at manifestation is a considered to be a strong prognostic indicator in MNTI.…”

“…There is a slight male predilection. 5,6 The MNTI is never congenital. It emerges within the 1st year of life [mostly below 6 months of age].…”

“…In contrast, manifestation from 2.5 to 4 months was associated with an intermediate risk and manifestation after 4.5 months of age had a minimal risk of recurrence. 5 …”

“…2 In 1966, Borello and Gorlin suggested that the tumour originated from neural crest cells based on the fact that some of these tumours excreted large amounts of vanillylmandelic acid (VMA) which is associated with other neuroectodermal tumours as well. [3][4][5] A total of 472 cases of this tumour had been reported between 1918 and 2013 as noted in the last extensive review published in 2015. 5 We report a case of MNTI, associated with an erupted primary tooth in a 5-month-old male child.…”

“…[3][4][5] A total of 472 cases of this tumour had been reported between 1918 and 2013 as noted in the last extensive review published in 2015. 5 We report a case of MNTI, associated with an erupted primary tooth in a 5-month-old male child. We discuss the clinical, radiographic and histologic features of this rare tumour, as well as its surgical management and the follow-up.…”