Department of Neurosurgery, West China Hospital, Sichuan University, Chengdu, Sichuan Province, China.
Department of Neurosurgery, West China Hospital, Sichuan University, Chengdu, Sichuan Province, China.
World Neurosurg. 2019 Oct;130:170-178. doi: 10.1016/j.wneu.2019.07.017. Epub 2019 Jul 8.
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).
婴儿黑色素神经外胚层肿瘤（MNTI）是一种罕见的婴儿颅面色素性肿瘤。本研究旨在分析起源于颅骨和大脑的 MNTI 文献。进行了系统的文献复习，从 1918 年至 2019 年，从 78 篇文章中确定了 91 例患者的报告。颅骨和大脑的 MNTI 在男孩中略为多见。颅内 MNTI 患者的诊断年龄主要>1 岁，而颅骨 MNTI 患者大多在出生后第一年被诊断（P<0.001）。颅骨 MNTI 多表现为快速生长的肿块，而颅内 MNTI 则表现为颅内压增高和神经功能障碍。手术切除是主要治疗方法，平均随访 25.6 个月。总生存率与 1 年内的诊断年龄（P=0.001）、肿瘤位置（P<0.001）、肿瘤大小（P=0.010）、治疗（P<0.001）和转移（P<0.001）以及恶性程度（P<0.001）有关，而复发率与年龄（P<0.001）、肿瘤大小（P=0.010）、完全手术切除（P=0.011）、转移（P=0.003）和恶性行为（P=0.001）显著相关。
