[Subependymal giant cell astrocytoma associated with tuberous sclerosis exhibiting rapid regrowth after 17 years: a case report].
1999
: A 17-year-follow-up case of subependymal giant cell astrocytoma (SGCA) is reported. In 1979, when aged 28 years, the patient first presented obstructive hydrocephalus caused by a tumor in the right lateral ventricle close to the foramen of Monro. It was partially removed by a transcallosal approach. Pathological examinations showed gemistocytic astrocytoma or SGCA associated with tuberous sclerosis. A ventriculo-peritoneal shunt was carried out and 36Gy of radiation therapy was administered. Eight months later, the patient suffered from an intraventricular hemorrhage originating from SGCA, but he responded to conservative therapy. He was followed-up by CT scans over 17 years. In 1996, because of rapid regrowth of the tumor, total removal was performed by a transcortical approach via the right frontal horn. The pathological diagnosis was SGCA. The greater part of the recurrent tumor was composed of blood vessels. The tumor cells were grouped into two morphological types, large cells and spindle cells. We compared the tumor in 1996 with that in 1979, each revealing immunohistochemical stainability for glial fibrillary acidic protein (GFAP), neuron specific enolase (NSE) and S-100 protein (S-100). The large cells in 1979 were negative for GFAP, NSE and S-100, but were positive for NSE and S-100 in 1996. The spindle cells in 1979 were positive for GFAP, NSE and S-100, but were negative for GFAP in 1996. The pathological origin of SGCA remains a subject of controversy. These results suggest that the origin of SGCA could be variably differentiated cells like the germinal matrix cells.
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