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Category: Volume 06 Issue 11 November 2018
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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

  1. David R. Mirich, Susan I Blaser, Derek C. Harwood-Nash, Derek C. Armstrong, Larry E. Becker, Jeffrey C. Posnick. Melanotic neuroectodermal tumor of infancy: Clinical, radiological and pathologic findings in five cases.AJMR : 12 July/ August 1991.
  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.
  3. Agrawal, S. Saxena, S. Kumar and R. Gupta. Melanotic neuroectodermal tumor of infancy: Presentation of a case affecting the maxilla. Journal of oral and maxillofacial pathology, Jan-Jun 2010, Vol. 14, Issue 1.
  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.
  5. Chaudhary, N. Manuja, C. T. Ravishankar, and A. Sinha. Oral melanotic neuroectodermal tumor of infancy. JISPPD Jan-Mar 2014, Vol. 32, Issue 1.
  6. Madrid C, Aziza J, Hlali A, Bouferrache K, Abarca M. Melanotic neuroectodermal tumor of infancy: A case report and review of the aetiopathogenic hypotheses. Med Oral Pathol Oral Cir Bucal 2010; 15; e 739-42.
  7. Maria Gaiger de Oliveria, Thompson LD, Chaves AC et al. Management of Melanotic neuroectodermal tumor of infancy. Ann Diagn Pathol 2004:8:207-12.
  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.

Corresponding Author

Dr Karuna Gupta

Professor, Pathology, SMS Medical College, Jaipur