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).