Hamlat Abderrahmane, Hua Zi Fen, Saikali Stephan, Egreteau Joelle, Guegan Yvon
Department of Neurosurgery, CHRU Pontchaillou, Rennes, France.
Acta Neurol Belg. 2003 Dec;103(4):221-4.
Although rare, the malignant degeneration of epidermoid cysts is well documented. Its occurrence with leptomeningeal dissemination and malignant cells in CSF is scarce. To the best of our knowledge only four cases have been reported.
The authors report the case of malignant transformation of an epidermoid cyst with leptomeningeal carcinomatosis (which simulates aseptic meningitis) in a 54 year-old woman. Changes in CSF and radiological features are not correlated with the clinical course. Despite improvement of both the lesions on the scan images and the malignant cells in the CSF under chemotherapy the patient died.
The differential diagnosis of malignant degeneration of epidermoid cyst, and leptomeningeal carcinomatosis are discussed. The diagnosis of malignant transformation should be retained when MRI (or CT scan) shows contrast enhancement at the epidermoid site, and malignant cells are detected in CSF The prognosis is generally poor.
尽管表皮样囊肿的恶性变罕见，但已有充分文献记载。其伴有软脑膜播散及脑脊液中出现恶性细胞的情况则更为罕见。据我们所知，仅有4例相关病例报告。
作者报告了1例54岁女性表皮样囊肿发生恶性转化并伴有癌性脑膜炎（类似无菌性脑膜炎）的病例。脑脊液变化和影像学特征与临床病程不相关。尽管化疗后扫描图像上的病变及脑脊液中的恶性细胞均有所改善，但患者仍死亡。
讨论了表皮样囊肿恶性变及癌性脑膜炎的鉴别诊断。当磁共振成像（或计算机断层扫描）显示表皮样囊肿部位有强化且脑脊液中检测到恶性细胞时，应考虑恶性转化的诊断。其预后通常较差。
