Melanotic Neuroectodermal Tumor of Infancy of the Femur

Abstract

Melanotic neuroectodermal tumor of infancy (MNTI) is a rare neoplasm that often occurs during the first year of life. More than 90% of MNTIs occur in the head and neck region, with most on the anterior part of the maxillary ridge. MNTI has also been reported in the mediastinum, thigh, foot, shoulder, and gonads. Here the authors report a rare case of MNTI of the femur in a 3-month-old child.

Melanotic Neuroectodermal Tumor of Infancy: A Systematic Review

“…7 Infants typically present with a rapidly growing, painless, nonulcerative mass affecting the maxilla or other craniofacial structures, 4 destroying adjacent bone and causing esthetic deformities and feeding disruption. The mass is typically firm, with smooth swelling and bluish-black coloration.…”

“…This yielded a total of 69 publications, which included the 116 cases (Supplementary Table 1). 4,7,[12][13][14][15][16] In addition, data were collected on 356 additional cases that were reported in the literature from 1918 through 2004 from 3 major systematic reviews. 18,81,82 These cases were combined with the 116 cases published from January 2005 through December 2013 (Supplementary Table 1) to constitute a total of 472 cases for analysis.…”

“…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.…”

Melanotic Neuroectodermal Tumor of Infancy

“…3,4 Rare, non-head and neck sites include epididymis, [5][6][7] testis, 8,9 ovaries, 10 and soft tissue and bones of the extremities. [11][12][13][14] The largest retrospective study 2 that reviewed 472 reported cases within the literature showed mainly maxillary involvement in 279 cases (62.2%), followed by the skull (70; 15%), mandible (35; 7%), male reproductive system (23; 5%), central nervous system (20; 4%), soft tissue (7; 1%), and female reproductive system (3; ,1%). They also determined that the median age of diagnosis was 5 months.…”

Melanotic neuroectodermal tumor of infancy: a description of the experience of Dmitry Rogachev National Medical Research Center of Pediatric Hematology, Oncology and Immunology and literature review

“…В большинстве случаев МНЭО локализуется в области головы и шеи, однако описаны случаи локализации процесса в центральной нервной системе (ЦНС), мягких тканях, яичке, придатке яичка, средостении, бедренной кости и т. д. [3][4][5]. Исторически для описания МНЭО использовались различные синонимы, такие как пигментная амелобластома, опухоль сетчатки, меланотическая эпителиальная одонтома.…”