Title: Melanotic Neuroectodermal Tumor of Infancy: A Rare Case Report

Authors: Dr Karuna Gupta, Dr Priyanka Sharma, Dr Deepika Hemrajani, Dr Krishna Sireesha Sundaragiri, Dr Ajay Yadav

 DOI:  https://dx.doi.org/10.18535/jmscr/v6i11.88

Abstract

Introduction

Melanotic Neuroectodermal Tumor Of Infancy (MNTI) is a rare, benign, pigmented neoplasm arising from neural crest.1,2 Clinical presentation is often painless, locally aggressive, rapidly expansile growth causing facial disfigurement.2 In the literature, neoplasm has been known by various synonyms like congenital melanocarcinoma, retinal analage tumor, pigmented congenital epulis and melanotic progonoma.1,3 This lesion is found mainly in children below 1 year of age. Rare cases have been reported in older children and adults also.2,3 Gender predilection is not seen through literature.4

93% of MNTI occurred in head and neck region with unique predilection for anterior maxilla, although cases including brain, skull, epididymis have also been reported.5 MNTI has a recurrence rate of 15% and show malignant differentiation in 6.6% cases, hence long term follow up after surgical excision is required.6 Plain radiographs show expansile areas of translucency with poor circumscription probably as result of rapid tumor growth and local aggressiveness.5 The present case report describes a case of MNTI in a 1.5 month old male child along with brief review of literature and pathological differential diagnosis.

References

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Corresponding Author

Dr Karuna Gupta

Professor, Pathology, SMS Medical College, Jaipur