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目的研究原发性颅内滑膜肉瘤的临床表现、病理、治疗与预后的特点。方法回顾性分析1例原发性颅内滑膜肉瘤11岁患儿的临床资料、影像学表现、病理、治疗和预后。结果 CT检查示右颞枕大片混杂密度影,肿瘤大小为3.5 cm×3.0 cm×2.0 cm。接受右颞枕叶肿瘤根治性切除术,术后病理示镜下小圆核瘤细胞呈片巢状或围绕血管密集排列,细胞异型,可见核分裂相,部分区域见胶原纤维增生,部分区域黏液变性;免疫组化染色示CK(部分+)、Vim(部分+)、Syn(+)、EMA(-/+)、CK7(-)、CK19(+)、INI-1(+)、AFP(-)、Oct-4(-)、Des(-),GFAP(-),CD99(-),CgA(-),PLAP(-),NF(-)、CD117(-/+),MIB-1 10%,诊断为(右颞枕叶)间叶组织来源的恶性肿瘤——滑膜肉瘤。术后在当地医院行放射治疗,总剂量为50 Gy,共25次。术后11个月复查头颅MRI见肿瘤复发累及右侧小脑半球。结论原发性颅内滑膜肉瘤是一种非常罕见的恶性肿瘤,目前采用手术切除肿瘤结合术后放、化疗的方案,但预后不良。
Objective To study the clinical features, pathology, treatment and prognosis of primary intracranial synovial sarcoma. Methods The clinical data, imaging findings, pathology, treatment and prognosis of a 11-patient primary intracranial synovial sarcoma were retrospectively analyzed. Results CT examination showed a large mixed density of the right temporal occipital shadow, the tumor size of 3.5 cm × 3.0 cm × 2.0 cm. The patients with right temporal occipital lobectomy underwent radical resection. The pathological examination showed that the nucleus pulposus cells were nested or encircled with densely arranged blood vessels. The cells were abnormal and the mitotic phase was seen. In some areas, collagen fibers proliferated and mucoid degeneration Immunohistochemical staining showed that CK (partial +), Vim (partial +), Syn (+), EMA (- / +), CK7 (-), CK19 (+), INI- (-), CD (-), CD99 (-), CgA (-), PLAP %, Diagnosed as (right temporal occipital lobe) mesenchymal-derived synovial sarcoma. Radiotherapy was performed at a local hospital after surgery with a total dose of 50 Gy for a total of 25 times. Eleven months after the review of cranial MRI see the tumor recurrence involving the right cerebellar hemisphere. Conclusions Primary intracranial synovial sarcoma is a very rare malignant tumor. At present, surgical resection combined with postoperative chemotherapy and radiotherapy is adopted, but the prognosis is poor.