Melanotisch progonoma van de bovenkaak bij een kind

– At the age of one month, a girl had been subjected elsewhere to a limited enucleation of a swelling in the alveolar region beside the nose which upon resection was pitchblack. Four months later we performed radical resection because of recurrence of the tumour in the maxilla. At the time of writing, 5 years after this operation, there are no indications of recurrence.

The histological diagnosis was melanotic progonoma. This rare tumour, which occurs nearly exclusively in children younger than one year, is usually benign. A neuro-ectodermal pathogenesis is the most plausible. The localization is usually in the upper or lower jaw, although extrabuccal and extracephalic tumours have also been described.