Melanotic neuroectodermal tumour of infancy (progonoma) treated by radical maxillary surgery

Abstract

Two cases of melanotic neuroectodermal tumour of infancy (MNTI), otherwise known as melanotic progonoma, both arising from the maxilla are presented here. The two infants both presented within three months of
each other and though clinically Burkitt’s lymphoma was stated as a possible diagnosis, the correct diagnosis was made on biopsy specimens. Radical surgery consisting of wide resection of the tumour with margins of
healthy tissue via hemi-maxillectomy was performed in each case as an initial method of treatment. At follow up four months later, both infants appeared clinically well. One infant was then lost to follow up thereafter
but the other was reviewed three years later, and apart from slight facial asymmetry, there was no evidence of tumour recurrence.