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Neurological Surgery No Shinkei Geka Volume 20, Issue 1 （January 1992）

Neurological Surgery 脳神経外科 20巻1号（1992年1月発行）

Japanese English

症例

von Recklinghausen氏病にみられた多発性脳・脊髄腫瘍の2症例 Two Cases of von Recklinghausen's Disease with Multiple Brain and Spinal Tumors 阪井田博司 ¹ , 花北順哉 ¹ , 諏訪英行 ¹ , 長安慎二 ¹ , 西正吾 ¹ , 太田文人 ¹ Hiroshi SAKAIDA ¹ , Junya HANAKITA ¹ , Hideyuki SUWA ¹ , Shinji NAGAYASU ¹ , Shogo NISHI ¹ , Fumito OHTA ¹ ¹静岡県立総合病院脳神経外科 ¹Department of Neurosurgery, Shizuoka General Hospital キーワード： von Recklinghausen's disease , Multiple brain tumors , Multiple spinal tumors Keyword: von Recklinghausen's disease , Multiple brain tumors , Multiple spinal tumors pp.51-56

発行日 1992年1月10日 Published Date 1992/1/10

DOI https://doi.org/10.11477/mf.1436900383

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Abstract 文献概要
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I．はじめに

　von Recklinghausen氏病は，皮膚・末梢神経の多発性腫瘍とCafe-au-lait spotを伴う家族性・遺伝性疾患として理解されてきた．また中枢神経系に各種の腫瘍が多発することもよく知られており，いわゆるcentralneurofibromatosisとして，脳神経外科領域でしばしば取り上げられてきた．しかしこれらの報告のなかで，異種多発性脳腫瘍と多発性脊髄腫瘍を合併したものは，われわれが渉猟したかぎりで22例のみであった^{2,4,7-8,10,12，14-21,25-27）}．今回われわれは，異種多発性脳腫瘍と多発性脊髄腫瘍を合併したvon Recklinghausen氏病の2症例を経験したので，若干の文献的考察を加え報告する．

The authors reported two cases of von Reckling-hausen's disease with multiple brain tumors and multi-ple spinal tumors.

The first case, a 21-year-old man who had a past his-tory of optic gliomas was admitted because of gait dis-turbance. Computed tomography (CT) and magnetic resonance imaging (MRI) showed calcification of the basal ganglia, bilateral C-P angle tumors, cystic cerebel-lar tumor and arachnoid cyst in the quadrigeminal cist-ern. Myelography and MRI revealed multiple spinal tumors. Surgical management was performed and cere-bellar tumor was histologically confirmed to be apilocytic astrocytoma. Spinal tumors were also astrocy-tomas.

The second patient, a 56-year-old woman suffered from right iliac pain, right hemiparesis and motor apha-sia. CT revealed two round tumors in the left cerebral hemisphere and bilateral C-P angle tumors. Myelogra-phy and MRI demonstrated multiple intradural-extramedullary spinal tumors. Histologically, supra-tentorial tumors were transitional meningiomas and spinal tumors were neurinomas.

It is well known that von Recklinghausen's disease isoften associated with brain or spinal tumors. But, in the literature, only 22 cases of von Recklinghausen's dis-ease combined with multiple brain tumors with diffe-rent histological types and multiple spinal tumors have been reviewed. With our two cases, the average age of these 24 cases was 28.6 years old, nine cases were male and 15 cases were female. All patients had C-P angle tumors and 23 cases were combined with intracranial meningiomas. In this paper, the clinical features and dia-gnostic aspects were discussed.