The aim of the present study was to integrate the available data published on melanotic neuroectodermal tumor of infancy (MNTI) of the jaw bones. An electronic search was undertaken in April 2018. Hundred forty-seven publications and 371 patients were included. The lesion was more prevalent in males and in the second to sixth months of life. The lesions mostly presented as a rapidly growing bluish swelling and the most commonly involved was the anterior maxilla. The mean follow-up was 51.1 months (range 1-408 months). Age (P </= .0001), location (P = .007), occurrence of lymph node metastasis (P </= .0001), treatment (P = .001), recurrence (P </= .0001), and distant metastasis (P = .0001) were independently associated with survival. Recurrence was significantly correlated with age (P = .0001), distant metastasis (P = .0001), and treatment (P = .0001). Patients older than 12 months, with lesions in the mandible, positive regional lymph node metastasis, treated with chemotherapy, recurrence, or distant metastasis presented the worst prognosis.
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