颅内原发组织细胞肉瘤一例临床分析

Clinical analysis of primary central nervous system histiocytic sarcoma： one case report

目的提高对中枢神经系统原发组织细胞肉瘤（HS）的临床表现、组织病理学特征、诊断与治疗方法的认识。方法报道1例颅内多发HS患者的临床表现、辅助检查及诊治过程。结果患者女性，55岁，急性起病，主要表现为行走不稳及记忆力减退。头颅MRI示脑实质深部多发边界清楚的肿块，增强后明显强化。予肿瘤活体组织检查术，显微镜下见肿瘤细胞核多形性，胞质丰富，呈嗜伊红或透明，背景空泡样，部分肿瘤细胞核仁明显。免疫组织化学染色示Vimentin、CD68、CD163、S-100、CD45、Ki67均阳性，雌激素受体、孕激素受体、上皮细胞膜抗原、P63、平滑肌肌动蛋白、细胞角蛋白（CK）18、CK5／6、CD20、CD79a、CD3、抗黑素瘤特异性单抗、胶质纤维酸性蛋白、抗黑色素A均阴性。虽经积极放疗，患者仍于放疗2个月后死亡。结论对于颅内多发及单发不明原因肿块，需考虑HS可能，需尽早行病理活体组织检查，以期早期诊断并治疗。目前尚无有效治疗手段，总体预后不佳。

Objective To provide insight into the clinical manifestation, histopathology characteristics, diagnostic and therapeutic methods of primary histiocytic sarcoma （HS） of central nervous system. Methods The clinical, auxiliary examination and pathological data of one patient with intracranial primary histiocytie sarcoma were presented, and relevant literature were reviewed. Results A 55-year-old female had an 2-month history of hypomnesia and unsteady gait. MRI scans showed multiple mass lesions in brain. Biopsies of the brain lesion showed the tumor cells had polymorphism, and abundant cytoplasm was eosinophilic or clear, with large, pleomorphic nuclei. Immunohistochemically, the tumor cells stained positively with CD45, CD68, CD163, Ki67 and Vimentin, but negatively with pan-cytokeratin, epithelial membrane antigen, CD3, CD20, CD79 a, estrogen receptor, progesterone receptor, Melan-A and glial fibrillary acidic protein. Though she had received radiotherapy, the disease progressed and her consciousness deteriorated within 2 months. Conclusions HS should be considered if multiple or single intracranial unexplained lumps are found and the earliest pathological biopsy is required for early diagnosis. There are no effective therapeutic methods for HS and the prognosis is very poor.