脑膜瘤伴黑色素细胞移殖性增生

doi:10.3969/j.issn.1672-6731.2021.06.011

摘要/Abstract

摘要：

目的总结1例脑膜瘤伴黑色素细胞移殖性增生患者的临床特点及诊断与鉴别诊断要点。方法与结果 女性患者，24岁，头部CT和MRI提示左侧枕叶占位性病变，T₁WI呈高和低混杂信号，T₂WI呈等和低混杂信号，增强扫描明显强化。临床诊断为左侧枕叶肿瘤性病变，行左侧枕后入路开颅肿瘤切除术。术中可见肿瘤表面有部分光滑包膜，色泽黑，血供极其丰富。组织学形态，肿瘤细胞呈团片状分布，部分围绕血管呈乳头状排列；肿瘤细胞中等大小，胞质丰富，可见瘤巨细胞和核内包涵体以及散在的砂粒体形成；较多色素细胞胞质内可见大量黑色素颗粒。免疫组化染色，肿瘤细胞波形蛋白、上皮型钙黏附蛋白、孕激素受体呈阳性，生长抑素受体2、胶质纤维酸性蛋白、S-100蛋白部分阳性，黑色素相关抗原45（HMB45）、黑色素-A、上皮膜抗原、细胞角蛋白、低分子细胞角蛋白和高分子细胞角蛋白、SOX10、L1细胞黏附分子、D2-40、少突胶质细胞转录因子2呈阴性，而色素细胞HMB45、SOX10呈阳性。基因检测提示NF2基因缺失。最终诊断为脑膜瘤伴黑色素细胞移殖性增生。术后未行放化疗，复查头部MRI未见肿瘤复发。结论脑膜瘤伴黑色素细胞移殖性增生临床罕见，其诊断依靠组织学形态、免疫组化染色和分子病理学检查，应注意与其他中枢神经系统含有黑色素细胞的肿瘤相鉴别。

关键词: 脑膜瘤, 黑素细胞, 细胞移植, 细胞增殖, 免疫组织化学, 病理学

Abstract:

Objective To summarize the clinical features, diagnosis and differential diagnosis of a case of meningioma associated with reactive hyperplasia and colonization of melanocytes. Methods and Results A 24-years-old female was discovered a left occipital lobe occupying occasionally by the head CT and MRI. The tumor revealed heterogeneous hyperintense and hypointense signals on T₁WI and heterogeneous isointense and hypointense signals on T₂WI with enhancement obviously. The left occipital lobe tumor was diagnosed clinically, and the left occipital posterior approach craniotomy was performed under general anesthesia. During operation, there was a smooth capsule on the surface of the tumor partially, which was dark in color and extremely rich in blood supply. Histological findings revealed the tumor cells were distributed in patches, and some of them were around blood vessels characterized by a prominent pseudopapillary architecture. Tumor cells were medium in size and eosinophilic cytoplasm was abundant. Giant tumor cells, intranuclear inclusion and scattered psammoma bodies were visible. A large number of pigmented cells consisted of dark, fine pigment granules. Immunohistochemical staining showed tumor cells expressed vimentin (Vim), E-cadherin, progesterone receptor (PR). Somatostatin receptor 2 (SSTR2), glial fibrillary acidic protein (GFAP), S-100 protein (S-100) presented partially positive. Melanosome-associated antigen (HMB45), Melan-A, epithelial membrane antigen (EMA), cytokeratin (CK), low molecular weight cytokeratin (LCK), high molecular weight cytokeratin (HCK), SOX10, L1-cell adhesion molecule (L1CAM), D2-40 and oligodendrocyte transcription factor-2 (Olig-2) were negative, but the pigmented cells were immunopositive for HMB45, SOX10. Genetic test revealed deletion of NF2 gene. The final diagnosis was meningioma associated with reactive hyperplasia and colonization of melanocytes. No chemoradiotherapy was performed after operation, and no recurrence of tumor was found by MRI. Conclusions Meningioma associated with reactive hyperplasia and colonization of melanocytes is a rare neoplasm. The diagnosis relies on its morphological characteristics, immunophenotype and genetic test. It should be differentiated from other tumors with melanocytes in the central nervous system.

Key words: Meningioma, Melanocytes, Cell transplantation, Cell proliferation, Immunohistochemistry, Pathology

宋坤, 王娟, 柴学, 张玉梅, 严春燕, 周金宝, 朱海青. 脑膜瘤伴黑色素细胞移殖性增生[J]. 中国现代神经疾病杂志, 2021, 21(6): 485-492.

SONG Kun, WANG Juan, CHAI Xue, ZHANG Yu-mei, YAN Chun-yan, ZHOU Jin-bao, ZHU Hai-qing. Meningioma associated with reactive hyperplasia and colonization of melanocytes[J]. Chinese Journal of Contemporary Neurology and Neurosurgery, 2021, 21(6): 485-492.

http://journal11.magtechjournal.com/cjcnn/CN/Y2021/V21/I6/485

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