de Souza Daniel Falbo Martins, Sendyk Daniel Isaac, Seo Juliana, da Fonseca Eduardo Vasques, Naclério-Homem Maria da Graça, Deboni Maria Cristina Zindel
Department of Oral Surgery, School of Dentistry, University of São Paulo, Avenida Prof. Lineu Prestes 2227, Butantã, 05508-000 São Paulo, SP, Brazil ; Residency Program in Oral and Maxillofacial Surgery at Conjunto Hospitalar do Mandaqui, São Paulo, SP, Brazil.
Case Rep Dent. 2013;2013:726815. doi: 10.1155/2013/726815. Epub 2013 Sep 8.
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.
婴儿黑色素性神经外胚层肿瘤（MNTIs）是罕见的生长迅速且复发率高的肿瘤。这些起源于神经嵴的肿瘤通常发生在1岁以下儿童的上颌前部。在此，我们描述了一名两个月大左侧脸颊肿胀加剧的女童的MNTI病例。该病例经断层扫描和活检后诊断为MNTI。患者的组织学和免疫组化特征显示神经、黑素细胞和上皮细胞分化的显著组合。肿瘤切除一年后，随访检查显示该患儿无肿瘤复发。自首次描述此类肿瘤以来，已报告约260例MNTI病例。在本病例中，早期诊断将治疗相关的困难和风险降至最低，并促成了最佳结果。尽管已进行了完整的手术切除，但仍建议进行仔细的随访。此外，对于MNTI病例，可能需要进行上颌功能矫形和重建。
