Melanotic neuroectodermal tumor of the neurocranium in infancy

Melanotic neuroectodermal tumors of the neurocranium are a rare but life-threatening disorder of infancy. 11 previously reported cases are reviewed in terms of clinical presentation, radiological diagnosis, and management. A twelfth case, a 4-month-old infant who developed three discrete sites of tumor unilaterally in the neurocranium is presented. Several hypotheses for the mechanism of formation of these tumors are reviewed. The authors propose that the mechanism of formation involves a dysontogenesis of neural crest tissue and that these tumors form, at least in part, from fragments of melanin-containing arachnoid villi which are displaced during embryonic development.