Melanotic Neuroectodermal Tumor of Infancy: a Clinicopathological Case Report

Abstract

Melanotic neuroectodermal tumor of infancy (MNTI) is a rare neoplasm that affects mainly children under 1 year of age. A 4-month-old boy was referred for evaluation of a lesion with 1 month of evolution. Intra-oral examination detected a firm upon palpation submucosal nodular mass, measuring 1.5 cm in diameter, affecting the anterior maxillary alveolar ridge and covered by a slightly blue mucosa with evident telangiectasia. The patient underwent an incisional biopsy and histological and immunohistochemical analyses revealed nests of AE1/AE3 positive epithelioid cells with abundant melanin pigmentation. Other cell types, resembling neuroblasts, were also present and positive for CD56, synaptophysin and enolase. The diagnosis of MNTI was established and the patient was referred for treatment. Conservative surgical resection was performed along with 3 adjacent teeth under general anesthesia. The patient is in follow-up for 1,5 year without recurrence. Conservative surgical management of MNTI may be an alternative to maxillectomy, contributing to the patient´s quality of life.

Melanotic Neuroectodermal Tumor of Infancy: the Use of Immunohistochemical Analysis

“…Thus, with the rapid growth of MNTI, its malignant potential, in addition to recurrence rates, an early diagnosis is essential ( 12 ). Therefore, immunohistochemical analyzes have become important because they can help confirm a melanotic neuroectodermal tumor of infancy diagnosis ( 12 , 19 , 20 ).…”

A case report of melanotic neuroectodermal tumor of infancy complicated with congenital heart disease and hypothyroidism

“…The prognosis for MNTI is generally good with a postoperative recurrence rate of approximately 15–45% mostly due to incomplete resection of the primary lesion ( 2 ). In 3% of MNTI cases, metastasis to the lymph nodes or the central nervous system has been documented ( 15 , 16 ). Some studies suggest that the resection of the primary lesion should be expanded to remove the majority of the tumor that invades the surrounding tissues to decrease the rates of recurrence.…”