Mandibular Melanotic Neuroectodermal Tumor of Infancy Treated Conservatively With Enucleation

Abstract

Melanotic neuroectodermal tumor of infancy (MNTI) is an uncommon, rapidly growing neoplasm of neural crest origin that primarily develops in the maxilla of infants during their first year of life. Mandibular lesions are rare and account for about 6% of all cases. Radical surgical excision is usually curative, but patients should be followed up closely because recurrence may occur in approximately 10% to 20% of cases. In this study, we report a case of mandibular MNTI in a 4-month-old male patient that was conservatively treated with enucleation and curettage and has shown no recurrence 11 years after surgery. Review of the English-language literature revealed that of more than 350 cases of MNTI reported so far, only 23, including the present one, have been encountered in the mandible. Most patients (91.3%) were younger than 1 year, whereas the male-to-female ratio was 1.3:1. Most lesions were treated with wide surgical excision, with only 2 cases being conservatively treated with enucleation. Recurrence was noted in 36.8% of cases at less than 3 months postoperatively. In conclusion, MNTI lesions in the mandible, albeit rare, show high recurrence rate. However, small-size mandibular MNTI lesions may be successfully treated with conservative enucleation. Close follow-up is highly recommended, in particular during the first 6 postoperative months.

Clinical and immunohistochemical study of melanotic neuroectodermal tumor of infancy in the maxilla

“…Melanotic neuroectodermal tumor of infancy may share a common histological and immunophenotypic expression with other lesions, such as cellular blue nevus, melanoma, neuroblastoma, and rhabdomyosarcoma, but MNTI commonly does not express diffuse reactivity with S-100, ( 2 , 3 , 6 , 8 ) as observed in our case. Other markers, such as HMB45, Melan A, cytokeratin, and neuroblastic markers, such as synaptophysin and neuron-specific enolase, can help with the diagnosis.…”

“…This treatment modality is curative when tumors are easily detached from the bone, as in this case. ( 8 ) Moreover, esthetic sequelae and alteration in normal face development may be prevented by this conservative surgical approach. ( 10 ) Rachidi et al, ( 4 ) reported that recurrence usually occurs within 6 months after treatment and in patients younger than 4.5 months.…”

Melanotic neuroectodermal tumor of infancy: A case report and review of the surgical treatment

“…Recurrence may occur due to invasion of the tumor edge into the bone, difficulty in complete resection due to a tumor with no envelope ( 15 ) or multicenter growth. In previous studies, high recurrence rates and no recurrence despite incomplete resection have been reported ( 16 ). The lack of recurrence without complete resection may stem from a compaction effect that triggers an immune response to destroy the remaining tumor cells.…”

The use of proton radiation therapy in a patient with melanotic neuroectodermal tumor of infants of the anterior fontanel: description of a clinical case

“…В мировой литературе описано около 500 случаев МНЭО [1]. Встречается преимущественно у детей первого года жизни, возрастной пик заболевания -5 месяцев [2]. У 7 % больных могут определяться отдаленные метастазы [3].…”