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.

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2. Balaji Babu Bangi and AvinashTejasvi M. L. Melanotic neuroectodermal tumor of infancy: A rare case report with differential diagnosis and review of literature. Contemporary Clinical Dentistry, Jan-Mar 2012, Volume 3, Issue 1.
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4. Agrawal, V. Agrawal and V. K. Raina. Melanotic neuroectodermal tumor of infancy: Case report of an unusual tumor. Indian J. Plastic Surg, July-December 2003, Vol. 36 Issue 2.
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8. Krompecher E. Zur Histogenese and morphologic der adimantinome and sonstiger keifergeschwulste. Beitr Path anat 1918; 64: 165-97.
9. Nathanson NR, Tedeschi LLG. Melanotic progonoma- A tumor of infancy. Oral Surg Oral Med Oral Pathol 1967; 23 (3): 354-361.
10. Allen MS Jr, Harrison W, Jahrsdoerfer RA. “Retinal analage” tumors. Melanotic progonoma, melanotic adamantinoma, pigmented epulis. Melanotic neuroectodermal tumor of infancy. Am J Clin Pathol 1969;(51)3 : 309-314.
11. Borello ED, Gorlin RJ. Melanotic neuroectodermal tumor of infancy: a neoplasm of neural crest origin.1966; 19: 196-206.
12. Galera-Ruiz H, Gomez-Angel D et al. FNAC in the pre-operative diagnosis of melanotic neuroectodermal tumor of infancy. J Laryngol Otol 1999; 113: 581-4.

Dr Karuna Gupta

Professor, Pathology, SMS Medical College, Jaipur