Timely Diagnosis and Multidisciplinary Management of Melanotic Neuroectodermal Tumor of Infancy Through Total...

Melanotic neuroectodermal tumor of infancy (MNTI) is a rare, typically benign but locally aggressive tumor that most commonly affects the maxilla in infants under 1 year of age. Although surgical resection is the standard treatment, cases requiring total maxillectomy and immediate reconstruction have rarely been reported.

Here, we report a 6-month-old male infant who presented with a rapidly enlarging maxillary mass. Imaging revealed a 60 × 57 × 50 mm lesion with extensive bone destruction and displacement of the primary teeth. A biphasic tumor composed of small round cells and melanin-containing epithelioid cells was identified histologically and confirmed to be MNTI by immunohistochemistry. Following a multidisciplinary evaluation, a total maxillectomy was performed 6 days after the initial visit. Immediate orbital floor reconstruction was achieved using cryopreserved autologous bone harvested from the resected specimen. The patient recovered without complications and was discharged on postoperative day 15. At 12-month follow-up, there was no recurrence or infection, and the reconstructed segment showed successful integration.

This case highlights the feasibility of immediate reconstruction using cryopreserved autologous bone after a total maxillectomy for MNTI. Prompt diagnosis and coordinated multidisciplinary care are essential for optimal outcomes in aggressive MNTI. Long-term follow-up is necessary to assess growth, function, and aesthetic outcomes.