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Melanotic neuroectodermal tumor of infancy (MNTI) of the hard palate: presentation and management
R Puchalski1, U K Shah, D Carpentieri
1Division of Pediatric Otolaryngology, The Children's Hospital of Philadelphia, PA 19104-4399, USA.
Insights
Melanotic neuroectodermal tumor of infancy (MNTI) is a rare, benign hard palate tumor. Complete surgical excision is curative, but may require follow-up for palatal reconstruction and dental care.
Area of Science:
- Oral pathology
- Pediatric oncology
- Developmental biology
Background:
- Melanotic neuroectodermal tumor of infancy (MNTI) is a rare, benign, pigmented tumor of neural crest origin.
- MNTI most commonly affects the maxilla, particularly the anterior hard palate in infants.
- Early diagnosis and management are crucial for optimal outcomes.
Observation:
- A 6-month-old female infant presented with a slow-growing, painless mass on the anterior hard palate.
- Radiologic imaging demonstrated a well-circumscribed, cystic lesion containing unerupted teeth.
- Histopathological and electron microscopic examination confirmed the diagnosis of MNTI.
Findings:
- MNTI of the hard palate typically presents as a smooth, firm, painless, slow-growing lesion.
- Imaging often reveals a well-circumscribed, cystic appearance, sometimes with calcifications or tooth-like structures.
- Complete surgical excision is considered the definitive treatment for MNTI.
Implications:
- Complete excision of MNTI generally leads to a favorable prognosis with no recurrence.
- Management may necessitate multidisciplinary approaches, including plastic surgery for palatal reconstruction.
- Long-term follow-up is important to address potential orthodontic needs and secondary nasal deformities.
Objective:
To discuss the presentation and management of melanotic neuroectodermal tumor of infancy (MNTI) of the hard palate.
Method:
Case presentation and literature review.
Case:
A 6-month-old girl presented with a slow growing, non-tender anterior oral hard palate mass. Radiologic imaging revealed a well-circumscribed cystic lesion containing teeth. After excision, histopathologic and electron microscopic evaluation revealed MNTI. No recurrence was seen at 12-month follow-up.
Conclusions:
This case and a review of the literature reveal MNTI to be a rare, benign hard palate tumor, which may present as a smooth, firm, painless, slow-growing anterior palatal lesion. Imaging reveals a well-circumscribed cystic lesion. Complete excision should be curative. Management requires attention to the potential need for palatal reconstruction, orthodontic care and correction of secondary nasal deformities.
