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Melanotic neuroectodermal tumour of infancy: A report of two cases.

Moussa SA, ElSayed M, Mansour S, Mobarak FA

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  • Journal International journal of surgery case reports

  • Published 15 Nov 2018

  • Volume 53

  • Pagination 337-344

  • DOI 10.1016/j.ijscr.2018.11.004

Abstract

Melanotic neuroectodermal tumour of infancy (MNTI) is a benign tumour of infancy, most commonly affecting the head and neck region. First described in 1918, less than 500 cases have been reported in the literature. MNTI is aggressive in nature & has a high rate of recurrence.

In this retrospective case series, we report two cases of MNTI that presented at our unit; both cases were managed by wide excision and have been followed up uneventfully for over two years.

MNTI has a recurrence rate of up to 20%. Patient's age can play a significant role in recurrence rate. Although this neural crest tumour is somewhat rare in the literature, there is a consensus with regards to surgical management; the gold standard remains to be wide excision with safety margin. Select cases may benefit from adjuvant and neoadjuvant therapy.

Owing to its locally aggressive nature and high recurrence rate, prompt diagnosis and surgical intervention is advised in cases of MNTI. Further understanding of this tumour is needed on a microscopic level in order to determine clear prognostic factors.