本文报告3例原发颅内黑色素瘤的临床病理分析。3例均为儿童(年龄分别为61/2、7、11岁)。尸检所见,皮肤和其它脏器未见有黑色素瘤,但大脑、小脑、脊髓整段的表面被盖一层黑纱,切面见肿瘤浸润脑实质。镜下所见瘤细胞形态大小不一,胞浆丰富,胞浆内充满黑色素颗粒,胞核圆形,核膜厚,核仁明显。 本文提示当有全身巨大黑色素痣儿童出现中枢神经系统症状时,应该注意到颅内原发黑色素瘤之可能。本文综述文献,提出本病的病理学诊断标准,并作了病理鉴别诊断。对本病的组织来源、颅内分布及预后也作了简单说明。 This article reports 3 cases of primary intracranial melanoma clinicopathological analysis. 3 cases were children (age were 61 / 2,7,11 years old). Autopsy showed no skin and other organs of melanoma, but the brain, cerebellum, spinal cord entire surface covered with a layer of black gauze, the tumor infiltration of the cut surface of brain parenchyma. Microscopic findings of tumor cells in different sizes, abundant cytoplasm, filled with melanin granules, nucleus round, thick nuclear nucleoli, nucleoli obvious. This article suggests that when there are symptoms of central nervous system in children with massive melanoma, the possibility of intracranial primary melanoma should be noted. This article reviews the literature, put forward the pathological diagnostic criteria of the disease, and made pathological differential diagnosis. The source of this disease, intracranial distribution and prognosis also made a brief description.