Melanocytic Neuroectodermal Tumour of Infancy-A Rare Case Report

Melanotic neuroectodermal tumour of infancy (MNTI) is an unusual, rapid growing, pigmented tumour of neural crestal origin that is seen in infants. It is more common in males with predilection for the maxilla and an unpredictable malignant potential. It constitutes a great challenge in diagnosis, treatment planning and prognosis.

This is a case of MTNI in a 5-month-old female child, who presented to the Department of Oral and Maxillofacial Surgery with progressive swelling on the right side of the upper jaw over the last two weeks with 'teeth floating appearance on imaging'.

There was significant extraoral diffuse swelling and facial asymmetry. Intraorally a well-defined mass noted on alveolar ridge of anterior maxilla and hard palate, crossing midline and associated with unerupted teeth. A subsequent contrast-enhanced computed tomography (CECT) scan and 3D CT scan revealed an expansile radiolucent lesion involving the right maxilla with destruction of cortical bone and displacement of primary central, lateral, canine and molar teeth, which appeared as a 'free-floating teeth appearance' in axial and coronal sections, confirmed by an open biopsy as Melanotic neuro-ectodermal tumour of infancy. Immunohistochemistry was used for precise diagnosis due to biopsy sample limitations and tumour variability.

Surgical resection was done after obtaining informed consent from the parents under general anaesthesia. The removal of unerupted primary central and lateral incisors, canine and molar teeth and also peripheral ostectomy was done as visible flecks were observed.

The postoperative period was uneventful. Regular follow-up after 26 months postoperatively showed acceptable healing with no portent of recurrence.

Early diagnosis and conservative surgical treatment and periodic follow-up postoperatively gives an excellent result with good prognosis.