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

Abstract

Melanotic neuroectodermal tumor of infancy (MNTI) is a rare, fast-growing, benign tumor originating from the neural crest. The tumor most often occurs during the first year of life. The predilection site of MNTI is the anterior maxilla, whereas lesions of the mandible are uncommon and account for only 6% of all cases. At present, the most common treatment for MNTI is surgical resection, however, tumor recurrence arises in 10–60% of cases. The optimal extent of surgical resection is a matter of debate; rapid growth and the possibilities of malignant transformation and metastasis indicate aggressive surgical resection. However, extensive resection may interfere with post-operative growth and development. The procedure should therefore preserve as much of the surrounding tissue as possible. The present case study examines a rare case of right-sided mandibular MNTI in a two-month-old female. The association between the tumor and the surrounding sclerotin, affected dental germ and the condition of the inferior alveolar nerve, were observed during the operative and post-operative periods. In addition, previous cases of MNTI were reviewed to evaluate the optimal scope of surgical resection.

Melanotic neuroectodermal tumor of infancy of the jaw bones: Update on the factors influencing survival and recurrence

“…We also observed that lesions was seen as a rapidly growing asymptomatic bluish swelling, which corroborates the information previously published in the literature. 11,14,21 We observed that radical surgery is the best treatment approach, consistent with other previous studies. 13,18 Histologically, the tumor consists of a biphasic population of small neuroblast-like cells and larger melaninproducing epithelioid cells arranged in an alveolar architecture, with cords and trabeculae intersected by dense fibroblastic stroma.…”

“…The tumor cells are typically negative for chromogranin, neurofilaments, S100 protein, and desmin. 11,14,21 We observed that radical surgery is the best treatment approach, consistent with other previous studies. 3,11,16,22 In our analysis, five cases reported regional lymph node metastasis and two were confirmed to be affected by the lesion.…”

“…The same result was observed in other recent studies, which found that the local recurrence rate following distant metastasis was 6.5%. 14,21 Metastasis mainly occurs due to invasion of the tumor edge into the bone and the difficulty in achieving complete resection as the tumor has no envelope or multicenter growth.…”

Tumor neuroectodérmico melanótico de la infancia. Presentación de un caso clínico

“…El tumor neuroectodérmico melanótico de la Infancia (TNMI), es benigno infrecuente, originado en la cresta neural y compuesto por células productoras de pigmentos (melanina). 1 Fue descrito por primera vez en 1918 por Krompecher quien le dio el nombre de melanocarcinoma congénito; otros sinónimos incluyen: tumor neuroectodérmico pigmentado de la infancia, progonoma melanótico y odontoma epitelial melanótico. En 1966, Gorlin y Borello registraron niveles altos de ácido vanilmandélico (AVM), un marcador de tumores neurogénicos en estos pacientes por lo que se lo clasificó dentro del grupo de tumores de origen neurogénico.…”

“…3 Se estima que cerca del 92.8% se presentan en la cabeza y el cuello, con mayor frecuencia en el maxilar superior (60-80%), en el cráneo a nivel de la fontanela anterior más comúnmente (10,8%), mandíbula (6%) y en el cerebro (4,3%). 1 En raras ocasiones surgen en meninges, seno transverso, retina, mediastino, huesos, útero y epidídimo. Se presenta con mayor frecuencia (95%) durante el primer año de vida, con una edad pico entre 2 y 6 meses de edad y se han reportado pocos casos en adultos.…”