Melanotic neuroectodermal tumour of infancy

Area of Science:

• Pediatric Oncology
• Oral Pathology
• Developmental Biology

Background:

• Melanotic neuroectodermal tumour of infancy (MNTI) is a rare, benign neoplasm originating from neural crest cells, predominantly affecting infants.
• The tumor typically presents in the first year of life, with a high predilection for the premaxillary region, though other sites can be involved.
• Clinical features include bluish discoloration, tooth bud displacement, and local aggressiveness.

Observation:

• This report details two cases of MNTI in a 5-month-old girl and a 6-month-old boy presenting at a dental college in Kolkata, India.
• Comprehensive evaluation involved clinical, radiological, histological, and immunohistochemical analyses to confirm the diagnosis.
• Flow cytometry was utilized to assess aneuploidy in the tumor cells.

Findings:

• Clinical, radiological, histological, and immunohistochemical findings confirmed MNTI in both pediatric patients.
• Analysis of aneuploidy via flow cytometry provided additional data on the tumor's characteristics.
• Surgical excision was the chosen treatment modality for both cases.

Implications:

• The successful surgical management of these two MNTI cases suggests a favorable prognosis with complete resection.
• Accurate diagnosis through integrated clinical and pathological assessments is crucial for appropriate treatment planning.
• Further research into the genetic underpinnings, such as aneuploidy, may refine understanding and treatment of MNTI.