Melanotic Neuroectodermal Tumor of Infancy

Area of Science:

• Pediatric Oncology
• Oral and Maxillofacial Surgery
• Developmental Biology

Background:

• Melanotic neuroectodermal tumor of infancy (MNTI) is a rare, benign neoplasm of neural crest origin.
• It typically affects infants under 1 year old, often in the anterior maxilla.
• Clinical presentation can mimic eruption cysts, delaying diagnosis.

Purpose of the Study:

• To report a case of MNTI in an infant.
• To highlight diagnostic challenges and management strategies.
• To emphasize the importance of prompt surgical intervention.

Main Methods:

• A 4-month-old male infant presented with a rapidly enlarging maxillary swelling.
• Diagnostic evaluations included clinical assessment, radiography, and histopathology.
• Management involved excisional biopsy under general anesthesia, with preoperative urinary vanillylmandelic acid (VMA) assessment.

Main Results:

• Radiographs revealed an expansile osteolytic lesion displacing a primary incisor.
• Histopathology confirmed MNTI with biphasic cell populations and positive immunohistochemical markers (HMB-45, vimentin, cytokeratin).
• The patient underwent complete excision and remained recurrence-free post-operatively with normal VMA levels.

Conclusions:

• MNTI, though benign, demonstrates rapid growth and potential for significant bone destruction.
• Accurate diagnosis and complete surgical excision with clear margins are crucial for preventing recurrence.
• Increased clinician awareness is vital for managing rapidly growing infant maxillary masses.