Melanotic neuro-ectodermal tumour of infancy

Area of Science:

• Pediatric Oncology
• Oral Pathology
• Developmental Biology

Background:

• Melanotic neuro-ectodermal tumor of infancy (MNTI) is a rare, benign, but locally aggressive neoplasm.
• Typically presents in the first year of life, often involving the maxilla or mandible.
• Early diagnosis and prompt intervention are crucial for optimal outcomes.

Observation:

• A 5-month-old infant presented with a rapidly enlarging oral mass that significantly obstructed the oral cavity.
• Clinical presentation suggested a rapidly growing tumor requiring urgent evaluation.
• Elevated urinary vanillyl mandelic acid (VMA) levels were detected, a marker associated with neuroblastoma and other neural crest tumors.

Findings:

• The mass was surgically excised in its entirety.
• Histopathological examination confirmed the diagnosis of melanotic neuro-ectodermal tumor of infancy.
• Post-operative VMA levels returned to normal, indicating successful tumor removal.

Implications:

• This case highlights the importance of considering MNTI in infants with rapidly growing oral masses.
• Prompt surgical management is effective in achieving complete remission.
• Monitoring urinary VMA levels can aid in diagnosis and post-operative assessment of MNTI.