Almomani Mohammad H., Rentea Rebecca M.
University of texas medical branch
Children's Mercy
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.
婴儿黑色素性神经外胚层肿瘤（MNTI）是一种起源于神经嵴的快速生长的良性肿瘤。它通常发生于头颈部区域的婴儿。1918年由克伦佩彻首次描述，由于其起源不明，被描述为先天性黑色素癌。直到1966年，这种肿瘤有多种不同的命名（如视网膜原基肿瘤、色素性先天性龈瘤、黑色素性原神经瘤、色素性畸胎瘤、非典型成黑色素细胞瘤等）。然而，博雷洛和戈林发现该肿瘤会产生香草扁桃酸（VMA），而其他起源于神经嵴的肿瘤也会产生这种物质。自1918年以来，已报告了500例MNTI病例。发病率最高的是美国，其次是印度。男性的发病率略高。由于这种肿瘤很少转移到远处，手术切除被认为是最佳治疗方法。然而，局部复发很常见。MNTI被认为是一种局部侵袭性肿瘤，因为它生长速度快。尽管该肿瘤因产生VMA而被认为起源于成神经细胞，但这并非常见特征。最近一项关于颌骨病变的系统评价发现，只有35%的病例VMA水平较高。
