[Four cases of ectopic pinealoma with ipsilateral cerebral hemiatrophy (author's transl)]

The 6 cases of brain tumor with ipsilateral cerebral hemiatrophy in which 4 cases were experienced in our institute and 2 cases were found in literature were studied in this paper. The specific character which was common in 6 cases were observed in their clinical course and the findings of clinical examinations. The histopathological study was proceeded with a autopsy case to observe the correlation between brain tumor and the ipsilateral cerebral hemiatrophy. As the result of it, we discussed the mechanism of appearance of the ipsilateral cerebral hemiatrophy due to brain tumor in the thalamic region. 1) All six cases were very young in which the age of onset was between 8 to 14 years old, 11 years 8 months old in average. 2) Their clinical course was relatively chronic. The period from onset to first admission was between 1 year 2 months to 4 years, 2 years and 1 month in average. 3) The declining of school work and hemiparesis were recognized as the primary sign of their onset. Main symptoms were hemiparesis, dementia, character and emotional change, and abnormal behavior, but sign of increased intracranial pressure was not observed. 4) The findings of carotid angiogram and pneumoence-phalogram showed ipsilateral hemiatrophy on the tumor side. 5) The brain tumor localized in the thalamic region and its surroundings which was common with all six cases. 6) Histopathological diagnosis was pinealoma, and 3 autopsy cases were ectopic pinealoma and the other 3 cases were suspected too as ectopic pinealoma. 7) In our autopsy case, ipsilateral cortical and subcortical atrophy with ectopic pinealoma was observed. As the pathological findins, degeneration and destruction of ganglion cells, demyelination in the subcortex and damage of axon were observed. These findings suggested that the ipsilateral cerebral hemiatrophy was induced by Waller's and retrograde degeneration as the result of the secondary damages of the thalamic ganglion cells and the afferent and efferent nerve fibers, due to invasive tumor into thalamic region.