A 40-Year-Old Female with Leptomeningeal Lesions.
A 40-Year-Old Female with Leptomeningeal Lesions.
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一名 40 岁女性，患有软脑膜病变。
DOI：
10.1111/bpa.12270
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发表时间：
2015
期刊：
影响因子：
--
通讯作者：
Kohmura E.
中科院分区：
文献类型：
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作者：
Mizowaki T;Sasayama T;Semba S;Sasaki R;Yasuo K;Nakamizo S;Tanaka K;Mizukawa K;Uchihashi Y;Kohmura E.
原发性软脑膜肉瘤（PLS）是一种非常罕见的中枢神经系统肿瘤，占所有颅内脑膜肉瘤的10%。PLS表现为弥漫性软脑膜受累或大的离散病变，通常发生在儿童和年轻人1，2，3，4。大约10%的病例存在脑膜的弥漫性受累，并被归类为原发性软脑膜肉瘤病4。PLS是高度侵袭性的肿瘤，预后不良，切除后50%的患者平均仅存活1年。因此，尽早诊断脑膜肉瘤可能很重要3。到目前为止，只有少数病例报告1、2、3、4。临床病史通常很短，由于病变的难治性，病情恶化很快。放疗剂量为50戈伊，分25次，5周内完成。然而，发病后6个月的MRI显示弥漫性软脑膜对比增强肿瘤遍布大脑和整个脊髓，以及侧脑室和第三脑室的脑室扩大。她在发病7个月后去世。
DiscussionPrimary leptomeningeal sarcoma (PLS) is an extremely rare neoplasm of the central nervous system, representing 10% of all intracranial meningeal sarcomas. PLS presents either with diffuse leptomeningeal involvement or large discrete lesions, and it usually occurs in children and young adults 1, 2, 3, 4. Diffuse involvement of the meninges is present in approximately 10% of the cases and is classified under the heading of primary leptomeningeal sarcomatosis 4. PLSs are highly aggressive tumors with a poor outcome with 50% averaging only 1‐year of survival after resection. It may therefore be important to diagnose meningeal sarcomas as early as possible 3. Only a few cases have been reported to date 1, 2, 3, 4. The clinical history is usually short, and deterioration is rapid on account of either the intractable character of the lesion. Radiotherapy was performed to our patient with a 50 Gy by 25 fractions in 5 weeks. However, an MRI 6 months after onset demonstrated diffuse leptomeningeal contrast enhancing tumors over the brain and entire spinal cord, as well as ventriculomegaly of the lateral ventricle and the third ventricle. She died 7 months after the onset of the disease.