Melanotic neuroectodermal tumor of infancy

Definitions: A rare, biphasic, neuroblastic, and pigmented epithelial neoplasm of craniofacial sites, usually involving the oral cavity or gums.

[1] By imaging studies, there is usually a large expansive radiolucent (clear) mass without well defined borders.

The reason for this postulation is based on the expression of melanotransferrin (melanoma-specific peptide that may play role in iron metabolism).

These cells have a fibrillary cytoplasm surrounding round nuclei with coarse and heavy nuclear chromatin.

[1][4] The lesional cells will show cross reactivity with several different families of immunohistochemistry markers (polyphenotypic), including neural, melanocytic, and epithelial.

[1][4] Even though there is often a very dramatic and rapidly enlarging, destructive tumor, there is usually a benign clinical course after complete local excision (usually by partial maxillectomy) with clear or free surgical margins.

A high power of melanotic neuroectodermal tumor of infancy showing pigmented large epithelioid cells and smaller primitive cells (hematoxylin and eosin stain).
A high power of melanotic neuroectodermal tumor of infancy showing pigmented large epithelioid cells and smaller primitive cells in alveolar nests (hematoxylin and eosin stain).