Melanotic neuroectodermal tumor of infancy (progonoma)—Clinical, radiological, pathological features and literature review

SummaryProgonomas are rare neuroectodermal neoplasms of uncertain behavior. Also called melanotic neuroectodermal tumors of infancy (MNTI), they usually present themselves early in first childhood, but there have been some case reports in adults. Typically presenting local aggressive behavior, they are prone to local recurrence and rapid growth. In this paper we present a case of MNTI which arose in maxillary bones and was surgically treated without any other adjuvant treatment. Clinical, radiological and histopathological features were reviewed, as well as literature data.