[The melanotic neuroectodermal tumor of childhood. Tumor identification with immunohistochemical methods. A case...

The authors report a case of a melanotic neuroectodermal tumor of infancy occurring in the maxilla of a 6-month-old female infant. The tumor displayed a biphasic cellular pattern with small neuroblast-like cells and large melanocytic cells showing a highly characteristic immunophenotype. Both tumor cell types immunostained for neuron specific enolase (NSE) and vimentin. The melanocytoid cells reacted additionally with the anti-keratin antibody Kl 1 and with HMB 45, an antibody specific for melanocytic antigens. The melanotic neuroectodermal tumor of infancy usually runs a benign course, however, local recurrence and metastasis may occur. The course of the disease cannot be predicted from morphological findings.