The melanotic neuroectodermal tumor of infancy.

Background: The melanotic neuroectodermal tumor of infancy is a rare and so far as benigne classified neoplasm with a high rate of recurrence for one year after diagnosis. Since Krompecher descriped 1918 the tumor at first, o nly about 200 cases are reported until today, mostly with manifestation in the maxillary region. Case-reports: The authors present two infants at the age of six and eight weeks with first clinical manifestation of the tumor i n the maxillary region. Although there were no other common signs, the tumo r destructed wide areas of the mid-face. In spite of a treatment with radic al surgery, recurrences occur rapidly in the first living year. Conclusions : Our clinical and histological findings show characteristica of local mali gnant growth. For these facts the radical resections of the primarius tumor and its recurrences are individually the therapeutical concequences. A fol low up of seven years of one infant shows a hypoplasm of the mid-face as a result of the inhibition of further growth by the loss of germs after maxil lary hemisection.