“…In the literature it has been known by a variety of synonyms including retinal anlage tumour, pigmented congenital epulis, melanotic progonoma, pigmented ameloblastoma, melanotic epithelial odontoma, pigmented teratoma, and melanotic adamantinoma, retinal ameloblastoma (Clarke and Parsons, 1951;Lurie, 1961;Stowens and Lin, 1974;Fletcher, 1995). This reflects great controversy about the cell of origin and pathophysiology of this tumour (Heba et al, 2008).…”
“…In the literature it has been known by a variety of synonyms including retinal anlage tumour, pigmented congenital epulis, melanotic progonoma, pigmented ameloblastoma, melanotic epithelial odontoma, pigmented teratoma, and melanotic adamantinoma, retinal ameloblastoma (Clarke and Parsons, 1951;Lurie, 1961;Stowens and Lin, 1974;Fletcher, 1995). This reflects great controversy about the cell of origin and pathophysiology of this tumour (Heba et al, 2008).…”
“…The authors also present a case report of a 5-month-old girl diagnosed with MNTI and review her clinical presentation and imaging and histopathologic findings. Ó 2015 American Association of Oral and Maxillofacial Surgeons J Oral Maxillofac Surg -: [1][2][3][4][5][6][7][8][9][10][11]2015 Melanotic neuroectodermal tumor of infancy (MNTI) is a rare neoplasm arising mainly in infants, although it also has been reported in older children and adults. It was first described by Krompecher 1 in 1918, and other names have been used to describe this lesion, such as melanotic progonoma, pigmented epulis, congenital melanocarcinoma, pigmented adamantinoma, retinal anlage tumor, and pigmented tumor of the jaw of infants.…”
“…Borello and Gorlin suggested the term "melanotic neurectodermal tumor of infancy." This tumor is also known as melanotic prognoma and has a predeliction for the head and neck region [8,9].…”
MNTI is an uncommon tumor mainly of infants. Surgery is the primary modality of treatment. Chemotherapy has been tried for recurrent/residual tumors. The chemotherapeutic response of 3 infants treated at a center is discussed here. All 3 infants showed good response to chemotherapy. In 2 of them complete residual tumor resection became possible; 2 of them are alive and tumor regression continues. One child had local recurrence later and was lost for follow-up.
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