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[一例患有冯雷克林霍增氏病的两种不同脊髓肿瘤(脑膜瘤和神经鞘瘤)]

[Two different spinal tumors (meningioma and schwannoma) with von Recklinghausen's disease in a case].

作者信息

Honda E, Hayashi T, Goto S, Oshima Y, Kikuchi N, Utsunomiya H, Ogasawara T, Honda Y, Sato Y

机构信息

Department of Neurosurgery, St. Mary's Hospital.

出版信息

No Shinkei Geka. 1990 May;18(5):463-8.

PMID:2117259
Abstract

Von Recklinghausen's neurofibromatosis is a congenital anomaly due to maldevelopment of the neurectoderm and mesoderm. The disease is known to be commonly associated with other tumors of the neural system. We experienced a case in which two different types of spinal tumors (meningioma and schwannoma) appeared together with Von Recklinghausen's disease. The patient was a 54 year-old female. Her chief complaint was slowly progressive lumbar back pain of about a 10-year duration. She was admitted to our hospital after developing such clinical symptoms as paraparesis, bladder and rectal incontinence, and pain in the soles of her feet. The CT study demonstrated a massive tumor at the TH12-L2 levels accompanied with marked scalloping of the posterior vertebral body. The tumor removed was, histologically, meningioma. During surgery, another small bean-size tumor was incidentally found originating from the dorsal root, and it was identified by histological study as typical schwannoma. It has been reported that the incidence rate of spinal tumors in von Recklinghausen's disease is approximately 4%. Although a case has been reported in which different types of tumors developed in both the cranium and the spinal canal, our case as described above is considered extremely rare as far as we can find in reference literature. The pathogenic mechanism of vertebral scalloping as encountered in our case was thought to be based on interactions between hypoplasia of the supporting tissue and pressure generated by the tumor and CSF.

摘要

冯雷克林霍增氏神经纤维瘤病是一种由于神经外胚层和中胚层发育异常引起的先天性疾病。已知该疾病常与其他神经系统肿瘤相关。我们遇到了一例患者,其同时出现了两种不同类型的脊柱肿瘤(脑膜瘤和神经鞘瘤)以及冯雷克林霍增氏病。患者为一名54岁女性。她的主要诉求是持续约10年的缓慢进展性腰背痛。在出现诸如双下肢轻瘫、膀胱和直肠失禁以及足底疼痛等临床症状后,她被收治入院。CT检查显示在胸12至腰2水平有一个巨大肿瘤,伴有椎体后缘明显的扇贝样改变。切除的肿瘤经组织学检查为脑膜瘤。手术过程中,偶然发现另一个小豆大小的肿瘤起源于背根,经组织学检查确定为典型的神经鞘瘤。据报道,冯雷克林霍增氏病中脊柱肿瘤的发病率约为4%。尽管曾有报道在颅骨和椎管内均出现不同类型肿瘤的病例,但就我们在参考文献中所能查到的情况而言,我们上述的病例被认为极为罕见。我们病例中所见到的椎体扇贝样改变的致病机制被认为是基于支持组织发育不全与肿瘤及脑脊液产生的压力之间的相互作用。

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