原発性異種多発性脳腫瘍

MULTIPLE DIVERSE PRIMARY BRAIN TUMORS

I．はじめに

　組織学的に異なつた種類の原発性腫瘍が多発することは比較的まれなことであるが，von Recklinghausen病（以下vR病）においては中枢神経系に多発性腫瘍が出現する頻度が高いことが知られている。vR病に伴つた多発性脳腫瘍例の報告はRodrigeuzら^15）が文献上から収集したようにかなりの数にのぼつている。本邦では，百井ら^10），石原ら^5），直田ら^13），新井ら^1），寺尾ら^19）の報告がみられる。寺尾らはvR病に脳腫瘍を合併した27症例について報告したが，そのうち1例は神経鞘腫と髄膜腫の合併した異種多発性脳腫瘍の例である^19）。原発性異種多発性脳腫瘍の報告は，vR病にみられた例（中枢型vR病）^{1,5,10,13,15,19）}。vR病などの奇型的色彩の濃厚ないわゆるPhacomatosisを除外した例の報告^6,11），あるいは1つの脳腫瘍が組織学的に異なつた種類の脳腫瘍を誘発したであろうとの見解をとつている例の報告^8）などさまざまである。

　当科においては，最近までに12例の原発性異種多発性脳腫瘍を経験したが，これは全脳腫瘍症例の0.9％にあたつている（新井ら^2）の異種多発性脳腫瘍例9例に3例を追加した）。ここでは，1例の聴神経腫瘍と脂肪腫の合併例を除外し，中枢型vR病と考えられた11症例について，剖検の有無にかかわらず，その臨床的な面に考察を加えて報告する。

The occurrence of multiple primary intracranialneoplasms of diverse histologic variety is rather common in von Recklinghausen's disease. Eleven cases with multiple diverse primary brain tumors associated with von Recklinghausen's disease (central form of von Recklinghausen's disease) are presented. The average age of the patients at onset was 16.5 years. Females and males were almost equally affected. No family history of von Recklinghausen's disease was found in all cases. All patients had less striking skin manifestation than patients with peripheral form of von Recklinghausen's disease. Three cases had no cafe au lait spot, and one case had neither cafe au lait spot nor subcutaneous tumor. Subcutaneous tumors were not neurofibromas histologically but neurinomas in 7 cases. It may be said that central form of von Recklinghausen's disease had neurinomatosis of peripheral and cranial nerves in contrast to neurofibromatosis in peripheral form. Subcutaneous tumors had a tendency of rapidly growing simultaneously with brain tumors at some time in clinical course of 5 cases.

Eleven cases had various combinations of multiple diverse primary brain tumors. Six cases had multiple neurinomas and meningiomas. Two cases had multiple neurinomas and gliomas. Three cases had multiple neurinomas, meningiomas and gliomas. All cases had acoustic neurinomas, which were bilateral in 10 cases. Autopsies revealed multipleneurinomas involving 5th, 3rd, 7th, 9-12th cranial nerves, spinal nerve roots, cauda equina and other peripheral nerves in addition to acoustic neurinomas. Meningiomas were also multiple in intracranial and intraspinal region of 4 cases. Small multiple nodules of meningiomas were revealed on subdural surface in 2 cases. Intraventricular meningiomas were revealed in 4 cases. Combinations of neurinomas and meningiomas were most common in von Recklinghausen's disease. Our 9 cases had multiple neurinomas and meningiomas. Combi-nations of neurinomas and gliomas were found in 5 cases. Gliomas were histologically varified as spongioblastoma, and it was found that multiple aggregations of anaplastic glial cells were scattered in cerebral and cerebellar cortex. Angiomatous proliferations were found in cerebral cortex of 2 cases.

At admission, almost all cases had intracranial hypertension, hearing disturbance, visual dis-turbance, choked disc, facial palsy and high CSF protein. Almost all cases had poor prognosis. The average period of survival was 8.5 years, but 4 cases survived for less than 5 years.

Computed tomography may be useful in the diagnosis of multiple diverse primary brain tumors.