Melanotic neuroectodermal tumor of infancy (MNTI) is an uncommon, rapidly growing neoplasm of neural crest origin that primarily develops in the maxilla of infants during their first year of life. Mandibular lesions are rare and account for about 6% of all cases. Radical surgical excision is usually curative, but patients should be followed up closely because recurrence may occur in approximately 10% to 20% of cases. In this study, we report a case of mandibular MNTI in a 4-month-old male patient that was conservatively treated with enucleation and curettage and has shown no recurrence 11 years after surgery. Review of the English-language literature revealed that of more than 350 cases of MNTI reported so far, only 23, including the present one, have been encountered in the mandible. Most patients (91.3%) were younger than 1 year, whereas the male-to-female ratio was 1.3:1. Most lesions were treated with wide surgical excision, with only 2 cases being conservatively treated with enucleation. Recurrence was noted in 36.8% of cases at less than 3 months postoperatively. In conclusion, MNTI lesions in the mandible, albeit rare, show high recurrence rate. However, small-size mandibular MNTI lesions may be successfully treated with conservative enucleation. Close follow-up is highly recommended, in particular during the first 6 postoperative months.

Ameloblastomas can present in various clinical and histomorphologic patterns. The granular cell variant accounts for only 3.5% to 5% of ameloblastomas. The aim of this case report is to present an example of ameloblastoma with unusual granular cell component, affecting a 63-year-old woman, in which both the inner and peripheral layers of follicles composed exclusively by eosinophilic granular cells. Assessment of the immunohistochemical and histochemical profile of the lesion was performed and the challenges of such a diagnosis were also addressed.