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目的探讨原发性颅内黏液型软骨肉瘤(PIMCS)的临床病理学特征及鉴别诊断要点。方法报道1例发生于右侧桥小脑角的黏液型软骨肉瘤的临床及影像学资料,观察其病理学形态及免疫表型,并复习相关文献探讨该肿瘤的病理诊断与鉴别诊断要点。结果患者39岁,女性。以右侧面部抽搐伴耳鸣1年余为主要症状。MRI示右侧桥小脑角区不规则软组织肿块。镜下可见黏液背景下,有纤维间隔分隔的多结节结构,间质血管稀少;瘤细胞相互连接成条索状或簇状;瘤细胞有中等量嗜酸性的细颗粒状至空泡状胞质,细胞核圆形至椭圆形,核分裂活性低。免疫组化示S-100和vimentin(+),Ki-67阳性指数为2%,而CK、p53、EMA、CEA、GFAP、CK7、CD10、CD117和nestin均为(-)。结论颅内原发性黏液型软骨肉瘤罕见,需与副节瘤、脊索瘤以及转移性腺癌等进行鉴别,其确诊需结合临床、影像学、组织病理学及免疫组化等信息进行综合分析。
Objective To investigate the clinicopathological features and differential diagnosis of primary intracranial mucinous chondrosarcoma (PIMCS). Methods One case of mucinous chondrosarcoma occurred in the right cerebellopontine angle was observed. The histopathological features and immunophenotypes were observed. Relevant literatures were reviewed to discuss the main points of pathological diagnosis and differential diagnosis. Results The patient was 39 years old and female. To the right side of the twitch with tinnitus more than 1 year as the main symptom. MRI showed right side of the cerebellopontine angle irregular soft tissue mass. Mucous under the microscope can be seen in the background, there are multi-nodular structure of fibrous septum, interstitial vascular sparse; tumor cells connected to each other into a cord-like or cluster; tumor cells with moderate eosinophilic fine particles to vacuolar cells Quality, nucleus round to oval, low activity of nuclear fission. Immunohistochemistry showed that the positive index of Ki-67 was 2% for S-100 and vimentin (+), and (-) for CK, p53, EMA, CEA, GFAP, CK7, CD10, CD117 and nestin. Conclusion The intracranial primary mucinous chondrosarcoma is rare and needs to be differentiated from paraneoplastic, chordoma and metastatic adenocarcinoma. The diagnosis should be based on clinical, imaging, histopathology and immunohistochemistry.