[Melanotic ectodermal tumor of infancy (melanotic progonoma)--an unusual soft tissue tumor]

In rare cases an utmost uncommonly pigmented lesion is found in young infants which is mostly located in the anterior maxilla. The histogenesis of this unusual soft tissue tumor has provoked a long-lasting debate, which is reflected in many synonyms. There is no anatomical precursor and the possibility of a phylogenetic ancestral form is discussed. Therefore, the term melanotic progonoma was proposed. Because of the derivation from neural crest cells the designation melanotic neuroectodermal tumor of infancy was introduced. This name is now generally accepted. In this study, two typical cases of this rare tumor are described. The tumors are composed of large epithelial-like melanin-producing cells and small nonpigmented cells, so-called lymphocyte- like cells resembling neuroblasts. The diagnostic relevant histological pattern is characterized by intensely pigmented cells arranged either in strands or clusters often forming the lining of small cleft-like or tubular spaces, or by alveolar structures surrounded by a fibrovascular stromal component. At ultrastructural level, the pigment corresponds to the cutaneous type of neural crest type of melanin. The histogenesis of these lesions and the classification of pigmented benign and malignant neuroectodermal tumors of the soft tissues are discussed especially taking into consideration the concept of the soft tissue variant of melanomas. The melanotic neuroectodermal tumor of infancy is a benign growth Only in very few cases a fatal outcome is reported in the literature. The melanotic neuroectodermal tumor of infancy must be distinguished from other types of benign and malignant neuroectodermal tumors. From histological point of view and with regard to its biological behaviour this lesion is a particular entity of pigmented neuroectodermal tumors of the soft tissues, and for subclassification the term melanotic progonoma should be maintained, too.