Melanotic Neuroectodermal Tumor of Infancy

Excerpt

Melanotic neuroectodermal tumor of infancy (MNTI) is a rapidly growing benign tumor that arises from the neural crest. It typically occurs in infants in the head and neck region. It was described for the first time in 1918 by Krompecher, and since the origin was unknown, it was described as congenital melanocarcinoma. Until 1966, this tumor was characterized by variable names (e.g., retinal anlage tumor, pigmented congenital epulis, melanotic progonoma, pigmented teratoma, atypical melanoblastoma, etc.). However, Borello and Gorlin found that the tumor produces vanillylmandelic acid (VMA), which is produced by other types of tumors that arise from the neural crest.

MNTI has had 500 cases reported since 1918. The highest prevalence is in the United States, followed by India. There is a slightly higher predominance in males. Since this tumor rarely metastasizes to distant locations, surgical excision is considered the best treatment. However, local recurrence is common. MNTI is considered a locally aggressive tumor because it has a high growth rate. Even though the neoplasm is considered to be of neuroblastic origin because it produces VMA, it is not a common feature. A recent systematic review of jaw lesions found that only 35% of cases had high VMA levels.