Abstract
ABSTRACTMelanotic neuroectodermal tumor of infancy is a rare and fast-growing neoplasm. In this study, we describe the case of a 6-month-old female patient, who presented swelling in the anterior maxilla. Tomographic reconstruction showed an unilocular hypodense and expansive area associated with the upper right central primary incisor. The presumptive diagnoses were dentigerous cyst, adenomatoid odontogenic tumor, melanotic neuroectodermal tumor of infancy and rhabdomyosarcoma, and an incisional biopsy was performed. Microscopically, the lesion revealed a biphasic cell population, consisting of small, ovoid, neuroblastic-like cells and epithelioid cells containing melanin. Immunohistochemically, the melanocyte-like component was strongly and diffusely positive for HMB-45 and Melan-A, but weakly positive for S100. Based on these findings, definitive diagnosis of melanotic neuroectodermal tumor of infancy was established. Then, enucleation of the lesion was performed by careful curettage. After 2 year follow-up, no clinical or radiographical evidence of recurrence was verified. The present case highlights the importance of early diagnosis and therapeutic intervention at the appropriate time to achieve a favorable outcome for the patient.
Highlights
Despite being classically a benign tumor, in most cases, the Melanotic neuroectodermal tumor of infancy (MNTI) grows rapidly, presents locally destructive invasion, and can cause deformities in adjacent structures.(1-3) a recurrence rate of about 60% has been reported for these tumors, some undergo malignant transformation, and 5% to 10% produce metastases.(1,3) early diagnosis minimizes the difficulties and risks associated with treatment, favoring an optimal outcome and normal infant development.(3) We report a case of MNTI, emphasizing its clinical, imaging, histological and immunohistochemical characteristics
❚❚DISCUSSION Melanotic neuroectodermal tumor of infancy is a rare, fast growing, benign tumor originating from the neural crest that usually occurs during the first year of life.(1-4) In a comprehensive systematic review of 472 MNTI cases, from 1918 to 2013, Rachidi et al,(4) found that most cases occurred in the head and neck region, quite frequently in the maxilla (62.2%), skull (15.6%), and mandible (7.8%)
As seen in the present case, MNTI appears as a painless, expansive, lobulated, partly pigmented tumor mass.(1,2) well defined, it is usually an unencapsulated lesion that primarily affects the jaws of newborn children, frequently causing bone destruction and displacement of dental follicles.(1,2) Some patients have high levels of urinary vanillylmandelic acid, which supports the neural crest origin of the tumor.(4,5)
Summary
Estudo clínico e imuno-histoquímico de tumor neuroectodérmico melanocítico da infância em maxila. Clinical and immunohistochemical study of melanotic neuroectodermal tumor of infancy in the maxilla. The presumptive diagnoses were dentigerous cyst, adenomatoid odontogenic tumor, melanotic neuroectodermal tumor of infancy and rhabdomyosarcoma, and an incisional biopsy was performed. The melanocyte-like component was strongly and diffusely positive for HMB-45 and Melan-A, but weakly positive for S100. Based on these findings, definitive diagnosis of melanotic neuroectodermal tumor of infancy was established. O tumor neuroectodérmico melanocítico da infância é uma neoplasia rara e de crescimento rápido. Foi estabelecido o diagnóstico definitivo de tumor neuroectodérmico melanocítico da infância. Descritores: Tumor neuroectodérmico melanótico/diagnóstico; Neoplasias maxilares/diagnóstico; Imuno-histoquímica; Microscopia; Criança; Relatos de casos
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