Glioblastoma multiformeの頭蓋外広範転移—その症例報告と文献症例の臨床病理学的考察

WIDESPREAD EXTRACRANIAL METASTASES OF GLIOBLASTOMA MULTIFORME. REPORT OF A CASE AND CLILICOPATHOLOGICAL REVIEW OF THE CASES IN THE LITERATURES

はじめに

　脳腫瘍の頭蓋外転移は従来稀であると考えられていたが，そのうち間葉性起源の悪性腫瘍，medulloblastoma，ependymomaやいわゆるpineal germinomaなどについては，次第に転移症例の報告が増加している。しかし，原発性頭蓋内腫瘍のうち，最も悪性度の高いものの中で，最高の頻度をもつglioblastoma multiformeについては，誰しもがその頭蓋外転移について当然考慮はしているものの，実際上は文献的にもそれらの症例報告は極めて稀であり，また充分に調査されてはいない。

　脳神経外科学の進歩・普及とともに，glioblastomamultiformeの治療がますます重要な課題となつてきつつあるが，その再発問題と共に，その転移については，今後大きな問題となることが予想される。

Extracranial metastasis of glioblastoma multifor-me has been said to extramely rare. We presented a case of glioblastoma multiforme which widely metastasized to cervical lymph nodes, scalp, peri-cranium, spinal cord and cauda equina.

A 18 year-old female of right handed was admit-ted to our clinic with complaints of headache, vomiting and visual disturbance. Decompression craniectomy was done with a diagnosis of a left parieto-occipital glioma. Selective arterial-infusion of 5-FU and MMC and irradiation were followed. Seven months after craniectomy, left cervical lymph nodes enlarged to the size of pigeon-egg, biopsy of which revealed a malignant neurogenic neoplasm. The tumor extensively infiltrated through the scalp and pericranium to the left lateral neck, and sub-total removal of the tumor was performed, inclu-ding the three fourth of the intracranial tumor mass, which weighed 788 g. Immediate postopera-tive cource was uneventful, but 10 days later, suddenly she went into coma after severe vomiting, and Froin's syndrome was noted by spinal tap. She expired 17 days after operation.

At autopsy, the primary tumor was found in the left parieto-occipital region and metastasized widely to the left cervical lymph nodes, scalp, pericranium, dura mater, spinal subarachnoid space and cauda equina. Microscopically there is great variation in size and shape of cells, with giant and multinuclea-ted and numerous bizarre mitoses. There is fre-quently an endothelial hypertrophy and prolifera-tion. Areas of necrosis and hemorrhage are frequent. Silver impregnation for reticulum fiber is negative. Microscopical diagnosis is glioblastoma multiforme.

Although there are many reports on extracranial metastasis of brain tumors, such an extensive metastasis of glioblastoma multiforme as this seems extremely rare.

52 cases of extracranial metastasis of glioblastoma multiforme in the literatures of the world and our case were analyzed, and 42 cases were males and 11 cases were females. The highest incidence is in the fifth decade. Only two cases were in the absence of operation and the others underwent the operation previously. Metastatic sites were found in limph node (26%), lung (22%), bone (19%), liver (9%), operative flap (8%), and dural vein system (3%).

We suggested that systemic treatment would had been necessary in these cases in addition to local application of chemotherapeutic agents via infusion and/or irradiation.

We discussed about immunological aspects of glioma through CSF in our case, considering the extremely rapid growing of the metastatic tumors ensuing after the operative removal of the larger part of the primary tumor and its extracranial cervical extension.