Melanotic Neuroectodermal Tumor of Infancy in the Maxilla

Author:

de Souza Daniel Falbo Martins12,Sendyk Daniel Isaac1ORCID,Seo Juliana3ORCID,da Fonseca Eduardo Vasques4,Naclério-Homem Maria da Graça1,Deboni Maria Cristina Zindel1ORCID

Affiliation:

1. Department of Oral Surgery, School of Dentistry, University of São Paulo, Avenida Prof. Lineu Prestes 2227, Butantã, 05508-000 São Paulo, SP, Brazil

2. Residency Program in Oral and Maxillofacial Surgery at Conjunto Hospitalar do Mandaqui, São Paulo, SP, Brazil

3. Department of Oral Pathology, School of Dentistry, University of São Paulo, São Paulo, SP, Brazil

4. Department of Oral and Maxillofacial Surgery, Conjunto Hospitalar do Mandaqui, São Paulo, SP, Brazil

Abstract

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.

Publisher

Hindawi Limited

Subject

General Dentistry

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