RESUMO
OBJECTIVES: Melanotic neuroectodermal tumor of infancy (MNTI) of the head and neck is a rare entity with uncertain clinical behavior. Radical surgical resection is the current recommended treatment, however this can cause severe aesthetic and functional sequelae. The aim of this study was to clinically characterize MNTIs and to stratify risk factors that may influence locoregional recurrence. METHODS: A retrospective multicenter study, including 11 patients from eight centers with a confirmed diagnosis of MNTI, was conducted. Epidemiological, clinical, radiological, pathological, and immunohistochemical examinations were reviewed. A statistical analysis using a t-test was conducted to calculate parameters correlating with tumor recurrence. RESULTS: MNTIs mainly occurred in the maxilla, with a mean age at diagnosis of 3.18 months (range: 0-6 months). Primary surgery was performed on 10 patients, with a clear margin resection on two patients. Overall recurrence rate was 27% with a survival of 100% at time of follow-up. No statistical correlation between recurrence rate, age at diagnosis, localization, resection margins, and pathological and immunohistochemical characteristics could be established. CONCLUSION: In our study, locoregional tumor recurrence did not seem to correlate with resection margins, so a conservative surgical approach may need to be considered to avoid functional and aesthetic sequelae.
Assuntos
Neoplasias de Cabeça e Pescoço/etiologia , Tumor Neuroectodérmico Melanótico/etiologia , Feminino , França/epidemiologia , Neoplasias de Cabeça e Pescoço/epidemiologia , Neoplasias de Cabeça e Pescoço/patologia , Neoplasias de Cabeça e Pescoço/cirurgia , Humanos , Lactente , Recém-Nascido , Imageamento por Ressonância Magnética , Masculino , Neoplasias Mandibulares/epidemiologia , Neoplasias Mandibulares/etiologia , Neoplasias Mandibulares/patologia , Neoplasias Mandibulares/cirurgia , Neoplasias Maxilares/epidemiologia , Neoplasias Maxilares/etiologia , Neoplasias Maxilares/patologia , Neoplasias Maxilares/cirurgia , Recidiva Local de Neoplasia/epidemiologia , Recidiva Local de Neoplasia/etiologia , Tumor Neuroectodérmico Melanótico/epidemiologia , Tumor Neuroectodérmico Melanótico/patologia , Tumor Neuroectodérmico Melanótico/cirurgia , Neoplasias Orbitárias/epidemiologia , Neoplasias Orbitárias/etiologia , Neoplasias Orbitárias/patologia , Neoplasias Orbitárias/cirurgia , Estudos Retrospectivos , Fatores de Risco , Tomografia Computadorizada por Raios XRESUMO
O tumor neuroectodérmico melanocítico da infância, também conhecido como progonoma, é uma enfermidade rara, benigna, originária da crista neural e que aparece no primeiro ano de vida, acometendo preferencialmente a maxila. Os autores relatam um caso raro deste tumor na maxila de uma criança de dez meses de idade, dando ênfase aos aspectos diagnósticos na tomografia computadorizada, e fazem uma revisão da literatura.
Assuntos
Animais , Feminino , Lactente , Tumor Neuroectodérmico Melanótico , Neoplasias Cranianas/diagnóstico , Neoplasias Cranianas/patologia , Tumor Neuroectodérmico Melanótico/diagnóstico , Tumor Neuroectodérmico Melanótico/etiologia , Maxila , Crista Neural , Tomografia Computadorizada por Raios XAssuntos
Humanos , Pré-Escolar , Feminino , Tumor Neuroectodérmico Melanótico/cirurgia , Tumor Neuroectodérmico Melanótico/diagnóstico , Tumor Neuroectodérmico Melanótico/etiologia , Distribuição por Idade , Chile , Diagnóstico Diferencial , Histocitoquímica/métodos , Neoplasias Maxilomandibulares , Microscopia Eletrônica/métodos , Recidiva , Distribuição por Sexo , Tumor Neuroectodérmico Melanótico/epidemiologia , Tumor Neuroectodérmico MelanóticoRESUMO
Melanotic neuroectodermal tumour of infancy is an uncommon neoplasm that usually occurs in children aged one year or less. Difficulty in deciding the cellular origin of this tumour has led to numerous names, including congenital melanocarcinoma, melanotic epithelial odontoma, melanotic ameloblastoma, and retinal anlage tumour. Electron microscopy and histochemical studies, however, have now established the neural crest origin. The most frequent site of occurrence is the maxilla followed by the skull, the brain and the mandible. The genital organs are the most frequent extracranial site. We present two cases of melanotic neuroectodermal tumour of infancy arising in the maxilla.
