Bulky subependymal giant cell astrocytoma with profuse blood supply without tuberous sclerosis.

S giant cell astrocytoma (SEGA) is a rare, slow-growing, benign tumor (WHO grade I), typically seen in young patients under 20 years,1 but can be found in infants and fetuses. It usually occurs in patients with tuberous sclerosis (TS). The SEGA is typically seen as a soft, less-vascularized tumor, located in the wall of the lateral ventricle, or near the foramen of Monro. Calcifications, cysts, and spontaneous hemorrhage have been observed. Although it rarely extends to the neighboring brain tissues, a large SEGA may cause increased intracranial pressure, seizures, and focal neurological signs. The SEGA often have a good prognosis and low recurrence.2 Herein, we present a rare case of an adult patient with a bulky SEGA with profuse blood supply, but without TS, who died due to postoperative massive intraventricular hemorrhage originating from the tumor. A 25-year-old man complained of daytime sleepiness and fatigue for one month with visual disturbance, dizziness without headache, and vomiting for 9 days. The physical examination showed that both pupils were enlarged up to 6 mm, and direct and indirect pupillary light reflexes were retarded, accompanied by bilateral papilledema. He had no significant medical history, or family history of major diseases such as cancer, cardiovascular, and hematological diseases. A CT and MRI performed on admission revealed an 8.0×6.0×5.5cm solid-cystic tumor mass upon the third ventricle and moderate obstructive hydrocephalus without cortical nodules (Figure 1). The lesion was heterogeneous containing cysts and enlarged blood vessels. Both lateral cerebral ventricles were enlarged. The tumor was partially excised through the transcallosal approach. The tumor was fuchsia with abundant vascularization. External ventricular drainage was installed post-operatively. The patient died of massive left intraventricular hemorrhage 4 days after the operation. Microscopic examination showed that most of the tumor tissue was composed of cells arranged in compact bundles, trabeculae, and nests, with some of the cells being multi-nuclei. Perivascular palisading/pseudorosettes were also observed (Figure 2). Approximately 40% of the tumor vessels were significantly hyperplastic, which were hyperemic and angio-ectatic. No focal calcifications were found. Immunohistochemical studies showed a limited reaction for glial fibrillary acid protein in plump astrocytes positivity for S-100 protein, vimentin and neuron specific enolase in cluster-like arrangements of Clinical Notes