多発性の神経膠腫・髄膜腫および神経鞘腫を併発した1剖検例とその発生について

Triple Diverse Multiple Primary Tumors in the Nervous System:Report of an Autopsy Case with Special Reference to Their Origins

I．はじめに

　異なつた種類の原発性脳腫瘍が，しかも多発している症例を経験することは比較的まれなことから，すでにこうした腫瘍についてはおびただしい報告が繰り返されてきた。しかしながら，本報告例のごとく，多能性の神経鞘腫と髄膜腫に加え，さらに，びまん性な拡がりを示す神経膠腫，あるいは少なくとも神経膠細胞の腫瘍性増殖像を全神経系に併発したものはきわめてまれであるだけでなく，脳腫瘍の発生に多くの示唆を与えると考えられるので，症例を報告しその発生につき考察を加えたい。

An autopsy case having multiple meningiomas, neurinomas and diffusely involved gliomas was re-ported.

The female patient expired at the age of 21 had no family history of von Recklinghausen's disease. At the age of 14, a neurinoma of 3 cm in diameter was extirpated from the subcutaneous tissue of her right orbit. The right peripheral facial palsy was noted at the age of 15. It was also noted that since about 7 years old, she had a mass on her left frontoparietal portion and at the age of 20 its size suddenly began to develop up to 17×20 cm, and there developed cafe-au-lait spots representing gene-ralized neurofibromatosis. Neurological examination revealed choked disc, loss of visual activity, right lagophthalmos, right exophthalmos, horizontal nys-tagmus, right peripheral facial paralysis, tinnitus and loss of hearing on the right side, slight paresis of the right upper extremity, right dysdiadochokine-sis and slight impairment of the right finger-nose-test. Left carotid arteriogram suggested the presence of meningioma on the left cerebral convexity. The patient was diagnosed to have right acoustic neu-rinoma and meningioma in von Recklinghausen's disease. Then, meningothlial meningioma (Fig. 19) involving the left frontoparietal region measuring 8 cm in diameter was totally removed. However, she expired 2 days after the procedures.

An autopsy revealed triple diverse multiple pri-mary tumors, such as multiple meningiomas, neu-rinomas and diffusely involved gliomas. Huge mul-tiple neoplastic glial cells, which were predominantly protoplasmic astrocytes and occasionally confluented as multiple tiny nodular gliomas, were diffusely scattered mainly in the normally configurated gray matter of the frontal to occipital lobes of the bi-lateral cerebral hemispheres, the basal ganglia, cere-bellar gray matter, left substantia nigra, optic chiasm and in the gray matter of the spinal cord (Figs 4, 5, 6-17). Multiple meningiomas were noted at the meninges covering the gasserian ganglion, optic chiasm, and the spinal cord, as well as in the oho-roid plexus of the left lateral ventricle (Fig. 18). Multiple neurinomas were also observed at the left HI, bilateral V, all of the bilateral VII to XII cranial nerve roots, spinal nerve roots of the cer-vical, thoracic and lumbar spinal cord, cauda equina, lumbar plexus, coeliac plexus, subserosal portion of the lower lobe of the left lung and the stomach, and at the paraaortic tissue of the aortic arch (Figs. 1, 2, 3, 20).

One-hundred twenty-six cases of primary multiple intracranial tumors in the literature were reviewed including the cases with von Recklinghausen's dis-ease. Nearly half (58 cases) of these 126 cases com-bined generalized neurofibromatosis, and all of the 58 cases also combined intracranial or spinal neu-rinomas and 56 out of the 58 cases associated with meningiomas. Twenty-five out of 26 cases, which combined three different kinds of intracranial tumors whether with or without von Recklinghausen's dis-ease, were combined with neurinomas, meningiomas and gliomas, although all of such gliomas, except a few cases, were noted as massed as contract to this particular case.

In reviewing the recent experimental studies on the neoplasms with the carcinogenic substances and viruses as well as on the malformations, it was suggested that in cases of phacomatosis, these mul-tiple diverse neoplasms might be originally deve-loped multicentrically from preexisting different kinds of cells, and from the same certain unknown stimulation.