RESUMEN
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.
Asunto(s)
Neoplasias de Cabeza y Cuello/etiología , Tumor Neuroectodérmico Melanótico/etiología , Femenino , Francia/epidemiología , Neoplasias de Cabeza y Cuello/epidemiología , Neoplasias de Cabeza y Cuello/patología , Neoplasias de Cabeza y Cuello/cirugía , Humanos , Lactante , Recién Nacido , Imagen por Resonancia Magnética , Masculino , Neoplasias Mandibulares/epidemiología , Neoplasias Mandibulares/etiología , Neoplasias Mandibulares/patología , Neoplasias Mandibulares/cirugía , Neoplasias Maxilares/epidemiología , Neoplasias Maxilares/etiología , Neoplasias Maxilares/patología , Neoplasias Maxilares/cirugía , Recurrencia Local de Neoplasia/epidemiología , Recurrencia Local de Neoplasia/etiología , Tumor Neuroectodérmico Melanótico/epidemiología , Tumor Neuroectodérmico Melanótico/patología , Tumor Neuroectodérmico Melanótico/cirugía , Neoplasias Orbitales/epidemiología , Neoplasias Orbitales/etiología , Neoplasias Orbitales/patología , Neoplasias Orbitales/cirugía , Estudios Retrospectivos , Factores de Riesgo , Tomografía Computarizada por Rayos XRESUMEN
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.
Asunto(s)
Animales , Femenino , Lactante , Tumor Neuroectodérmico Melanótico , Neoplasias Craneales/diagnóstico , Neoplasias Craneales/patología , Tumor Neuroectodérmico Melanótico/diagnóstico , Tumor Neuroectodérmico Melanótico/etiología , Maxilar , Cresta Neural , Tomografía Computarizada por Rayos XAsunto(s)
Humanos , Preescolar , Femenino , Tumor Neuroectodérmico Melanótico/cirugía , Tumor Neuroectodérmico Melanótico/diagnóstico , Tumor Neuroectodérmico Melanótico/etiología , Distribución por Edad , Chile , Diagnóstico Diferencial , Histocitoquímica/métodos , Neoplasias Maxilomandibulares , Microscopía Electrónica/métodos , Recurrencia , Distribución por Sexo , Tumor Neuroectodérmico Melanótico/epidemiología , Tumor Neuroectodérmico MelanóticoRESUMEN
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.
