Dorwal Pranav, Mohapatra Ishani, Gautam Dheeraj, Gupta Aditya
Department of Pathology, Medanta The Medicity, Gurgaon, India.
Department of Neurosurgery, Medanta The Medicity, Gurgaon, India.
Asian J Neurosurg. 2014 Jan;9(1):36-9. doi: 10.4103/1793-5482.131068.
Melanocytomas are present in leptomeninges and arise from neural crest during early embryonic development. They are a rare entity and usually occur in the thoracic spine and infratentorial region. We report a 32-year-old female with meningeal melanocytoma of D9-10. Magnetic resonance imaging revealed an intramedullary spinal tumor at D9-D10. Intraoperatively, the tumor was greyish-black in color with moderate vascularity, and was adherent to the cord. The clinical differential diagnoses included cavernoma and melanocytoma. On microscopic examination, the lesion showed sheets of cells with marked pigment deposition, which was obscuring the cellular morphology. The pigment was confirmed to be melanin by Masson's Fontana stain. Immunohistochemistry was performed, which showed positivity for HMB-45, S-100, Vimentin and Melan-A. The cells were negative for cytokeratin, epithelial membrane antigen, Glial fibrillary acidic protein and neuron-specific enolase. Mib-1 labeling index was less than 1%. In view of the lack of nuclear atypia, mitoses, necrosis and low Mib-1-labeling index along with immunohistochemistry profile, the diagnosis of Melanocytoma was made. Melanocytomas are rare pigmented tumors of the spinal cord and posterior cranial fossa. They are benign in nature, but can also be locally aggressive. Melanocytic lesions of the nervous system are to be differentiated from metastatic melanomas and also tumors showing melanin pigment deposition like schwanomma, paraganglioma, medulloblastoma and various gliomas.
黑色素细胞瘤存在于软脑膜,起源于胚胎早期发育过程中的神经嵴。它们是一种罕见的病变,通常发生在胸椎和幕下区域。我们报告一名32岁女性,患有D9 - 10节段的脑膜黑色素细胞瘤。磁共振成像显示D9 - D10节段髓内脊髓肿瘤。术中,肿瘤呈灰黑色,血管中等,与脊髓粘连。临床鉴别诊断包括海绵状血管瘤和黑色素细胞瘤。显微镜检查显示,病变有大量细胞片,伴有明显的色素沉积,掩盖了细胞形态。通过Masson氏Fontana染色证实色素为黑色素。进行了免疫组织化学检查,结果显示HMB - 45、S - 100、波形蛋白和Melan - A呈阳性。细胞角蛋白、上皮膜抗原、胶质纤维酸性蛋白和神经元特异性烯醇化酶呈阴性。Mib - 1标记指数小于1%。鉴于缺乏核异型性、有丝分裂、坏死以及低Mib - 1标记指数,同时结合免疫组织化学特征,做出了黑色素细胞瘤的诊断。黑色素细胞瘤是脊髓和后颅窝罕见的色素性肿瘤。它们本质上是良性的,但也可能具有局部侵袭性。神经系统的黑色素细胞病变需要与转移性黑色素瘤以及显示黑色素沉积的肿瘤如神经鞘瘤、副神经节瘤、髓母细胞瘤和各种胶质瘤相鉴别。