Melanotic neuroectodermal tumor of infancy: a rare case report

Öz

Melanotic neuroectodermal tumor of infancy (MNTI) is a rare, rapidly growing, and pigmented neoplasm of neural crest origin. It predominantly affects the maxilla of infants during the first year of life. A seven-month-old boy presented with a mass approximately 5 cm in diameter in the right oral cavity. On computerized tomography, a lytic expansile lesion was detected in the right maxilla. Microscopically, the tumor consisted of two different neoplastic cell proliferation, located peripherally and centrally, arranged in alveolar clusters within the fibrous connective tissue. Immunohistochemically, peripheral tumor cells showed diffuse staining for Pancytokeratin and HMB-45, the central cells were positive for CD56 and Synaptophysin. MNTI is a rare tumor that can be easily confused with malign small round cell tumors, especially in small biopsies. It has characteristic histomorphological and immunohistochemical findings. Its biological behavior is not fully understood. These tumors can present locally aggressive behavior and a high recurrence rate.

Anahtar Kelimeler

• Melanin
• Neuroectoderm
• Infant
• Tumor

Kaynakça

1. 1. Emmerling MR, York TA, Caccamese JF. Melanotic Neuroectodermal Tumor of Infancy: Case Report and Review of Management. J Oral Maxillofac Surg. 2019;77:315-320.
2. 2. Krombecher E. Zur Histogenese und Morphologie der Adamantinome und sonstiger Kiefergeschwulste. Beitr Pathol Anat Allg Pathol. 1918;64:165–197.
3. 3. Ebel F, Thieringer FM, Kunz C, et al. Melanotic neuroectodermal tumor of infancy to the skull: case-based review. Childs Nerv Syst. 2020;36:679-688.
4. 4. Sharma P, Yadav AK, Goyal S, Mandal AK. Melanotic neuroectodermal tumor of infancy: A rare entity. J Oral Maxillofac Pathol. 2019;23:134-7.
5. 5. Tan O, Atik B, Ugras S. Melanotic Neuroectodermal Tumor in a Newborn. Int J Pediatr Otorhinolaryngol. 2005;69:1441-4.
6. 6. Soles BS, Wilson A, Lucas DR, Heider A. Melanotic Neuroectodermal Tumor of Infancy. Arch Pathol Lab Med. 2018;142:1358-1363.
7. 7. Andrade NN, Mathai PC, Sahu V, Aggarwal N, Andrade T. Melanotic Neuroectodermal Tumour of Infancy - A Rare Entity. J Oral Biol Craniofac Res. 2016;6:237-240.
8. 8. Borello KD, Gorlin RJ. Melanotic neuroectodermal tumor of infancy – A neoplasm of neural crest origin. Report of a case associated with high urinary excretion of vanilmandelic acid. Cancer 1966;19:196-206.

9. 9. Ünsal HY, Yalçin M. Melanotic Neuroectodermal Tumor of Infancy in the Maxilla. J Craniofac Surg. 2018;29:28-30.
10. 10. Kumar A, Deepthi V, Aggarwal R, Aiyer HM. Melanotic Neuroectodermal Tumor of Infancy: A Rare Case Report. J Oral Maxillofac Pathol. 2018;22:44-7.
11. 11. Agarwal P, Saxena S, Kumar S, Gupta R. Melanotic Neuroectodermal Tumor of Infancy: Presentation of a Case Affecting the Maxilla. J Oral Maxillofac Pathol. 2010;14:29-32.
12. 12. Tan AP, Jacques TS, Mankad K, et al. Melanotic Neuroectodermal Tumour of Infancy: A Case Report and Differential Diagnosis. Neuroradiol J. 2018;31:434-9.
13. 13. Mengide JP, Jaimovich SG, Lubieniecki FJ, Rugilo C, Lamas G, Mantese B. Melanotic neuroectodermal tumor of infancy arising from the skull: report of an unusual case, review of the literature, and a diagnostic approach. Childs Nerv Syst. 2020 Mar;36(3):469-475.