脑膜恶性黑色素瘤病三例临床病理及神经影像学特点

Meningeal malignant melanosis： 3 cases report with clinicopathological and neurohnaging

目的探讨脑膜恶性黑色素瘤病的临床、病理及神经影像学特点。方法对我院收治的经病理确诊的3例脑膜恶性黑色素瘤病患者进行观察，总结其临床、脑脊液细胞学、神经影像学、脑膜组织病理等方面特点。结果3例患者均以头痛起病，随后出现脑膜刺激征。其中1例皮肤有巨大黑色素痣，1例额部黑痣破溃经久不愈，另一例无皮肤及内脏黑色素瘤，为原发性。3例患者颅脑MRI强化后显示软脑膜及蛛网膜弥漫性较均匀强化并增厚。脑脊液可见大量的异形细胞。脑膜呈黑褐色或深棕色，光镜下肿瘤细胞呈多形性，核大而圆或呈不规则形，胞质丰富，核分裂象可见，胞质内黑色素颗粒聚集，细胞排列紊乱。免疫组织化学分析S-100蛋白、波形蛋白、黑色素瘤抗体HMB-45等胞质反应阳性。结论脑膜恶性黑色素瘤病临床主要表现为头痛及脑膜刺激征，脑脊液可见大量的异形肿瘤细胞。脑膜病理见肿瘤细胞胞质内黑色素颗粒聚集、瘤细胞排列紊乱。颅脑强化MRI对该病的诊断具有一定价值。

Objective To investigate clinicopathological and neuroimaging features of meningeal malignant melanosis, Methods The cytologic study of cerebrospinal fluid （CSF） and neureimaging examination and meningeal histochemistry were performed in 3 cases of meningeal malignant melanosis. Results Three patients were found to have initial onsets of headache, followed by meningeal irritation. One case had huge malignant melanoma on skin. The second patient had diabretic malignant melanoma on her face, which did not healed for a long time. The third patient had no malignant melanoma on skin and viscera. The MRI enhanced scanning of all 3 cases showed abnormal enhancement of meninge. There were a large number of abnormal shape cells in CSF. The meninxes were blackbrewn or brown. The tumor cells were in various shapes with big and round irregular nucleus and rich cytoplasm. There were a great quantity of melanosomes. The tumor cells were disarranged. Immunohistochemistry analyses found S-100 protein and Vim and HMB-45 were positive reaction. Conclusions The patients with meningeal malignant melanosis have an initial onset of headache, followed by meningeal irritation, and MRI enhanced scanning plays a valueble role in the diagnosis. A lage number of tumor cells in abnormal shape have been found in CSF. The tumour cells Of meninx presents different shapes with big and round or irregular nucleus. There are a great quantity of mellanosomes, and the tumour cells are arranged in a state of chaos.