Melanotic neuroectodermal tumors of infancy (MNTIs) are rare fast-growing tumors with high recurrence rates. These tumors, which originate in the neural crest, commonly occur in the anterior maxilla of children under the age of one. Here, we describe an MNTI case in a two-month-old girl with increasing swelling in the left cheek. MNTI was diagnosed in this case following tomography and biopsy. The patient's histological and immunohistochemical profile indicated a remarkable combination of neural, melanocytic, and epithelial cell differentiation. One year following tumor excision, a follow-up examination revealed that the child exhibited no tumor recurrence. Approximately 260 cases of MNTI have been reported since this type of tumor was first described. In the present case, early diagnosis minimized the difficulties and risks associated with treatment and facilitated an optimal outcome. Despite complete surgical excision, careful followup is recommended. In addition, maxillary functional orthopedics and reconstruction may be necessary in cases of MNTI. 1. Introduction Melanotic neuroectodermal tumors of infancy (MNTIs) are rare, fast-growing, melanin-containing lesions that commonly occur in the head and neck regions of children under the age of one [1]. MNTIs are nonulcerative, painless, and pigmented lesions [2], but the pigmentation cannot always be observed through the covering tissues [3]. Uncertainties regarding the histogenesis of MNTIs have led authors in the literature to use a diverse nomenclature, and MNTIs have been described as congenital melanocarcinomas, atypical ameloblastomas and melanocytomas [4, 5]. Despite these controversies, the neural crest is accepted to be the origin of these types of tumors [1, 6–8]. MNTIs generally occur in the maxilla (68%–80%), but they can occasionally arise in the skull (10.8%), mandible (5.8%) or brain (4.3%) [1, 6, 7]. In addition to the head and neck region, other sites can be affected by the condition less frequently, including the femur, epididymis, ovaries, uterus and mediastinum [6, 7]. The effects of gender on MNTI remain controversial, but the majority of publications examining gender differences have reported no significant effects of gender [3]. MNTI lesions are regarded as benign tumors, although they can present locally aggressive behavior, including gradual invasion of the surrounding bone and sinuses. These lesions are characterized by a high recurrence rate that varies between 10% and 60% [6] and the risk of malignant transformation is 6.6% [1, 3, 6]. In plain radiographs, MNTI appears as an
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