Yamashiro S, Nagahiro S, Mimata C, Kuratsu J, Ushio Y
Department of Neurosurgery, Kumamoto University School of Medicine.
Neurol Med Chir (Tokyo). 1994 Dec;34(12):817-20. doi: 10.2176/nmc.34.817.
A 46-year-old male with xeroderma pigmentosum developed an intracranial malignant schwannoma originating from the second branch of the left trigeminal nerve. The tumor was subtotally removed and postoperative radiation therapy given, but the tumor recurred twice over 3 years, and extended to the third branch of the left trigeminal nerve and the ipsilateral facial nerve. Radical surgery and radiation therapy finally achieved a cure. This is the first case of malignant trigeminal schwannoma with xeroderma pigmentosum, although various other internal neoplasms including central nervous system tumors have been reported in xeroderma pigmentosum patients. Radical surgery and radiation therapy are effective for treating intracranial malignant schwannoma. Radiation therapy is considered safe for xeroderma pigmentosum patients.
一名46岁患有着色性干皮病的男性罹患起源于左侧三叉神经第二支的颅内恶性神经鞘瘤。肿瘤次全切除并给予术后放疗，但肿瘤在3年内复发两次，并扩展至左侧三叉神经第三支及同侧面神经。根治性手术和放疗最终实现了治愈。这是首例伴有着色性干皮病的恶性三叉神经鞘瘤，尽管在着色性干皮病患者中已报道过包括中枢神经系统肿瘤在内的各种其他内部肿瘤。根治性手术和放疗对治疗颅内恶性神经鞘瘤有效。放疗被认为对着色性干皮病患者是安全的。
