Melanotic neuroectodermal tumor of infancy: case report

Abstract

-Introduction:Melanotic neuro e c t o d e rmal tumor of infancy (MNTI) is a rare tumor, locally aggressive, usually originated from maxilla and mandible and rarely from the skull. A case of a 4 monthold child presenting a bulging lesion in the midline of the occipitoparietal region with pro g ressive gro w t h is re p o rt e d . Case re p o rt: The neurologic examination had normal developmental milestones. Computerized tomography scan and magnetic resonance Image showed a highly enhancing tumor, dislocating anteriorly and inferiorly the superior sinuses. In order to prevent excessive bleeding, surgical resection was performed in three stages, with complete removal. Conclusion: Based on the absence of tumor recurrence, we believe in a favorable neurological prognosis and in a possible of cure, although the patient was not submitted to any adjuvant treatment.KEY WORDS: neuroectodermic melanotic tumor, cranial tumor, tumor of infancy.Tumor melanótico neuro-ectodérmico da Infância: relato de caso RESUMO -O tumor melanótico neuro -ectodérmico da infância é raro, localmente agressivo, originado da maxila ou da mandíbula, raramente originando-se da calota craniana. Relatamos o caso de um menino de 4 meses de idade que apresentava lesão abaulada com crescimento pro g ressivo no couro cabeludo. O exame neurológico apresentava desenvolvimento neuro-psicomotor adequado. A tomografia computadorizada e a ressonância magnética do encéfalo mostravam lesão que impregnava pelo contraste, deslocando anterior e inferiormente o seio sagital superior. Com o objetivo de prevenir o risco do sangramento excessivo, o procedimento foi realizado em três tempos, com remoção completa da lesão. Baseados na ausência de recidiva do tumor, acreditamos no bom prognóstico neurológico e na possibilidade de cura, embora o paciente não tenha sido submetido a nenhum tratamento adjuvante. PALAVRAS-CHAVE: tumor melanótico neuro-ectodérmico, tumor craniano, tumor da infância. D e p a rtment

Slow-growing lambdoid melanotic neuroectodermal tumor of infancy

“…On the grounds of tissue culture and immunohistochemical and electron microscopic studies, MNTI are presently thought to arise from neural crest cells [1,6]. Histologically, these tumors consist of a mixture of cell types, including small immature cells with a hyperchromatic nucleus, scanty nonpigmented cytoplasm, and small stellated cells found in clusters.…”

“…Histologically, these tumors consist of a mixture of cell types, including small immature cells with a hyperchromatic nucleus, scanty nonpigmented cytoplasm, and small stellated cells found in clusters. Islands of large cuboidal cells with an ovoid marginated nucleus and abundant vacuolated cytoplasm are also seen [1]. MNTI cells are usually positive for cytokeratin, vimentin, epithelial membrane antigen (EMA), S100, glial fibrillary acidic protein (GFAP), and neuron-specific enolase (NSE) [1].…”

“…Islands of large cuboidal cells with an ovoid marginated nucleus and abundant vacuolated cytoplasm are also seen [1]. MNTI cells are usually positive for cytokeratin, vimentin, epithelial membrane antigen (EMA), S100, glial fibrillary acidic protein (GFAP), and neuron-specific enolase (NSE) [1]. CT scan usually shows a dense contrast-enhancing mass, with adjacent hyperostosis [4] (Fig.…”

“…In 2/3 patients, MNTI are located in the maxilla, and the skull vault is involved in 10% of cases, usually in the region of the anterior fontanelle [1,4,5]. However, temporoparietal, fronto-temporo-sphenoidal, posterior fontanelle, and mastoid locations have also been described [3,5].…”

Melanotic neuroectodermal tumour of infancy

“…This benign tumour originates from the soft tissue overlying the maxilla in more than 70% of cases, although mandibular, cranial, cerebral and genital involvement have also been described (4,5). The mean age of patients at diagnosis is 4.3 months, with a near-equal male to female ratio of 6:7 (6)(7)(8). The present report describes a case of MNTI requiring a near-total maxillectomy, along with an overview of the current pathological knowledge and therapeutic recommendations.…”