MELANOTIC NEUROECTODERMAL TUMOR OF INFANCY - A MOLECULAR-GENETIC STUDY

Melanotic neuroectodermal tumor of infancy is a rare but well-recogniz ed entity in pediatric pathology. However, the relationship of this tu mor to other pediatric small cell tumors with neuroectodermal features (such as neuroblastoma, Ewing sarcoma/peripheral primitive neuroectod ermal tumor, and desmoplastic small round cell tumor) is undetermined. Molecular genetic studies of melanotic neuroectodermal tumor of infan cy have not been reported. We studied three typical cases of melanotic neuroectodermal tumor of infancy in an attempt to link this tumor to other small cell tumors with well-characterized molecular genetic chan ges. Tests performed included: detection of MYCN gene amplification an d deletion of 1p (all 3 cases), and presence of the t(11;22)(q24;q12) and the t(11;22)(p13;q12) translocations (2 of 3 cases). None of these tests yielded positive results. Thus, there is no genetic basis at pr esent to link melanotic neuroectodermal tumor of infancy to neuroblast oma, Ewing sarcoma/peripheral primitive neuroectodermal tumor, or desm oplastic small round cell tumor.