Melanotic neuroectodermal tumour of infancy: Report of two cases and review of the literature

Extensive Melanotic Neuroectodermal Tumor of Infancy

“…Pigmentation may or may not be seen in the overlying soft tissue. This condition is generally found in patients under the age of 1 year, usually without predilection for sex [1,8,10,11], although in some review studies of cases described in the literature, we observed a slight predilection for the male sex [1,8,[12][13][14]. In spite of being locally invasive, these tumors are generally described as being benign.…”

“…The immunohistochemical profile of MNTI is similar in all reported studies. The main characteristic cells are: small cells of neural origin, confirmed by NSE, synaptophysin, and chromogranin positivity; large cells of ectodermal origin, confirmed by EMA, CK, and HMB-45 positivity [10].…”

“…Since the first description by Krompecher [2], who called the lesion congenital melanocarcinoma, studies have proposed various theories for the origin of this tumor [10]. Some researchers believed that it originated from the retinal anlage, whereas others believed it to be of odontogenic origin [10,18].…”

“…Some researchers believed that it originated from the retinal anlage, whereas others believed it to be of odontogenic origin [10,18]. One of the theories proposed arises from the fact that the tumor is reminiscent of the initial stages of retina development (retinal anlage tumor) [19,20].…”

“…Furthermore, no tissue similar to that of the sclera was identified, and the eyes of these patients are generally normal. Therefore, the majority of authors believe that the tumor originates from neural crest cells [1,3,5,[8][9][10].…”