Melanotic Neuroectodermal Tumor of Infancy Arising in the Skull and Brain: A Systematic Review.

Melanotic neuroectodermal tumor of infancy (MNTI) is a rare pigmented craniofacial tumor of infants. This study aimed to analyze the literature on MNTI arising from the skull and brain. A systematic literature review was performed and 91 patients reported in 78 articles were identified from 1918 to 2019. MNTI of the skull and brain was slightly more prevalent among boys. The age at diagnosis of patients with intracranial MNTI was mainly >1 year, and most patients with skull MNTI were diagnosed within the first year of life (P < 0.001). MNTI of the skull mostly presented as a rapidly growing mass, whereas intracranial MNTI presented with increasing intracranial pressure and neurologic dysfunction. Surgical resection was the primary treatment, with an average follow-up of 25.6 months. Overall survival was related to age at diagnosis within 1 year (P = 0.001), tumor location (P < 0.001), tumor size (P = 0.010), treatment (P < 0.001), and metastasis (P < 0.001) and malignancy (P < 0.001), whereas recurrence rate was significantly associated with age (P < 0.001), tumor size (P = 0.010), complete surgical resection (P = 0.011), metastasis (P = 0.003), and malignant behavior (P = 0.001).

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

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.