Abstract
MNTI is a rare dysembryogenic tumor of infancy. It is a benign neoplasm usually seen in the first 6 months of life. The predominant site of origin is the pre-maxilla with rapid expansile growth manifesting with feeding difficulty. The tumor arises from the neural crest cells. On histological examination, typical biphasic population of cells with deposits of melanin pigment is seen. Immunohistochemistry (IHC) study helps in confirming the diagnosis. It has a locally aggressive course with high recurrence rate. Malignant transformation and metastatic spread is extremely rare. Wide excision of the tumor is the treatment. Thorough follow-up is recommended for a period of 1 year. Early detection and proper surgery decreases complications with favourable outcome. A case report of this tumor affecting a 5-month old male child with involvement of the right maxillary antrum is being presented here. This case is reported for its rarity, and classical clinico-pathological findings.
Highlights
Melanotic Neuroectodermal Tumor of Infancy (MNTI) is an osteolytic-pigmented benign neoplasm of neural crest cells, primarily affecting the jaws of infants
MNTI is a relatively uncommon, benign neoplasm of early infancy with locally aggressive growth accompanied by high recurrence rate
Borello and Gorlin suggested the origin of the tumor to be from neural crest and as the incidence was common in infants, they coined the term ‘Melanotic Neuroectodermal Tumor of Infancy’ (MNTI) which is universally accepted now.[7]
Summary
MNTI is an osteolytic-pigmented benign neoplasm of neural crest cells, primarily affecting the jaws of infants. It is a locally aggressive tumor with rapid growth. It is composed of pigment-producing relatively primitive cells.[1] The two characteristic features of this tumor are the predilection for bones of skull in children below 1 year of age and the presence of melanin pigment in it, which makes the tumor distinct in clinico-pathological, immunohistochemical, ultrastructural and imaging fields.[2]. The mean age of patients at diagnosis is 4.3 months, with male to female incidence of 6:7; slightly commoner in female infants.[3] Approximately 92.8% of MNTI is seen in the head and neck region; the commonest site is maxilla in about 68-80% of cases, followed by skull (10.8%), mandible (5.8%) and brain (4.3%). Radiograph only reveals radiolucency whereas computed tomography (CT) scan accurately defines the extent of the lesion and helps in good surgical planning.[4]
Sign-in/Register to view highlights & summary Sign-in/Register to access full text options 
Talk to us
Join us for a 30 min session where you can share your feedback and ask us any queries you have
Schedule a callDisclaimer: All third-party content on this website/platform is and will remain the property of their respective owners and is provided on "as is" basis without any warranties, express or implied. Use of third-party content does not indicate any affiliation, sponsorship with or endorsement by them. Any references to third-party content is to identify the corresponding services and shall be considered fair use under The CopyrightLaw.
