von Recklinghausen病を伴ないRosenthal線維に富む視神経交叉部神経膠腫の1例

OPTIC GLIOMA WITH ABOUNDANT ROSENTHAL FIBERS ASSOCIATED WITH VON RECKLINGHAUSEN'S DISEASE: A CASE REPORT

はじめに

　von Recklinghausen病（以下R病と略す）は頭蓋内の種々の腫瘍を合併することがあり，母斑症の中でも特異な地位を占めている。特に神経鞘腫，髄膜腫，神経膠腫などを伴なうことが多い。そのうち視神経および視交叉部神経膠腫の合併率は，外国の文献では37.6％（Bürki^4）），21.8％（Marshall^15）），10％（Davis^6））などと高率にみられるのに反し，本邦での合併報告例はきわめてまれである。われわれはR病を伴ない，手術により視交叉部神経膠腫を確認し，いわゆる"Rosenthal線維"を豊富に認めた興味ある1例を経験したので症例を報告し本疾患とこの線維との関連について考察を加えたい。

Optic glioma associated with von Recklinghau-sen's disease has been rarely reported in Japan.

A 13-year-old girl was admitted to our clinic, because of visual disturbance on four years duration. Café-au-lait spots and subcutaneous nodules had been noticed since her childhood.

The findings on physical examinations were nor-mal except for bitemporal hemianopia and atrophy of the optic discs. Carotid angiography and pneumoencephalography revealed the presence of a space-occupying lesion in the suprasellar region.

By the right fronto-temporal craniotomy, a large fleshy tumor was observed in the chiasmal region, extending posteriorly to the interpeduncular fossa, and involved both optic nerves and optic chiasm. Subtotal removal of the tumor including the right optic nerve was followed by radiation therapy of ⁶⁰Co.

Microscopically, the tumor composed of spongio-blasts and astrocytes admixed with abundant eosino-philic globules and rods. On histochemical exami-nations, these bodies were corresponded to so-called "Rosenthal fibers". The origin and nature of this fiber were discussed.

Review of 63 gliomas of the optic nerve and chiasm in Japanese literatures disclosed 3 cases (4.7%) associating with von Recklinghausea's dis-ease. Of 70 autopsy cases of von Recklinghausen's disease between 1958 and 1967 in our country, only one case (1.4%) was associated with glioma of the optic nerve and chiasm.