Recurrent melanotic neuroectodermal tumour of infancy. Case report and survey of 16 cases

A case of melanotic neuroectodermal tumour of infancy (MNTI) with rapid recurrence is described, together with a survey of 16 reported recurrent cases. The recurrent lesion was successfully treated by wide excision of the maxilla. The recurrent tumour had two lesions of different natures, one consisting of a lobular mass indistinguishable from the primary tumour but located remote from the original site and the other showing diffuse infiltration of tumour cells deep in the medulla of the maxilla. A survey of the recurrent cases indicated that, although incomplete removal of the primary tumour, dissemination of tumour cells during surgery and a multicentric origin could be the causes of a recurrence, the presence of a silent lesion which may express an aggressive growth on stimulation may also explain the complex behaviour of the tumour.