Maxillary Melanotic Neuroectodermal Tumor of Infancy Management

Abstract

Melanotic neuroectodermal tumor of infancy (MNTI), an uncommon neoplasm that involves predominantly the maxilla, is classically benign but clinically aggressive. Surgery is the mainstay of the treatment with a proven efficacy, but its optimal method is not entirely clear. A case of maxillary MNTI in a 4-month-old male infant treated by radical surgery without recurrence after more than 3 years of follow-up is presented. On the basis of this experience and a review of the literature, the optimal method of MNTI surgery is discussed. To improve the prognostic of this neoplasm, the role of conservative surgery should be limited to the treatment of tumors where clear surgical margins excision should require sacrifice of functional or vital structures.

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

“…[17][18][19] Further, our literature review demonstrated that 92.5% of tumors affected the maxilla and 7.5% affected the mandible. 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 melanotic epithelioid cells generally surround nests of small cells but may form solid and tubuloglandular structures.…”

“…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. MNTI of the jaw bones has not previously been described as presenting an association with regional lymph node metastasis.…”

“…In previous studies, researchers observed that MNTI of the jaw bones is found mainly in children below 1 year of age. [15][16][17] We found a mean age of 6.5 months, with a higher age among males than females. Males were slightly more affected than females (ratio male:female 1.32:1).…”

“…In addition, some papers reported the concomitant use of chemotherapy and radiotherapy. 14,[16][17][18]23 The use of chemotherapy and radiotherapy is not well established for the treatment of MNTI of the jaw bones, as there was no evidence of a significant increase in survival when they were used.…”

BRAFV600E Mutation in Melanotic Neuroectodermal Tumor of Infancy: Toward Personalized Medicine?

“…2 The recurrent tumors grow more aggressively and can invade other anatomic structures, such as the nasal cavity, the orbit, and the skull base. 4 The Raf-MEK-ERK mitogen-activated protein kinase pathway is activated by growth factors, hormones, and cytokines and regulates proliferation, differentiation, survival, senescence, and migration of cells. 5 Among other isoforms of the Raf serine/threonine kinases, B-Raf (encoded by the BRAF gene) has a dominant role in signaling to the ERK pathway.…”