[Melanotic neuroectodermal tumor of infancy]

The melanotic neuroectodermal tumor of infancy is a rare and so far as being classified neoplasm with a high rate of recurrence for one year after diagnosis. Since Krompecher described 1918 the tumor at first, only about 200 cases are reported until today, mostly with manifestation in the maxillary region.

The authors present two infants at the age of six and eight weeks with first clinical manifestation of the tumor in the maxillary region. Although there were no other common signs, the tumor destroyed wide areas of the mid-face. In spite of a treatment with radical surgery, recurrences occur rapidly in the first living year.

Our clinical and histological findings show characteristics of local malignant growth. For these facts the radical resections of the primary tumor and its recurrences are individually the therapeutical consequences. A follow 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 maxillary hemisection.