Subependymal giant cell astrocytoma in tuberous sclerosis complex (with report of 9 cases)

Objective To explore the clinical, pathologic and imaging features of subependymal giant cell astrocytoma (SEGA) in tuberous sclerosis complex. Methods From 1998 to 2005, 9 patients with subependymal giant cell astrocytoma and tuberous sclerosis complex were treated in our department. Clinical and imaging presentations, pathologic features, treatment principle, and follow-up findings of these patients were analysed retrospectively. Results All of the tumors located around the foramen of Monro and conformed with the clinical diagnosis criteria of tuberous sclerosis complex. Incidence ratio of male and female was 8 ∶ 1. In CT scanning, the solid part of tumor showed iso-density or slightly higher density, the tumor border was clear, and calcification was seen in the tumor. On MRI T1WI, the solid part of tumor mostly presented iso-signal while slightly lower signal was lesser and on T2WI iso-signal or slightly higher signal was shown. On enhanced imaging, the solid part of tumor showed uniform intensification. Subependymal tuber was found in 8 cases and cortical tuber in 5 cases. All the tumors were resected. Frontal transcortical approach was performed in 6 cases and transcallosal-interfornix approach in 3 cases. Total resection was achieved in 7 cases, subtotal resection ( 90%) in 2 cases. No operative death occurred. Followed up 2-8 years. There were no recurrences among the total resected tumors. Conclusion Subependymal giant cell astrocytoma is a characteristic lesion of tuberous sclerosis complex, and it has a good prognosis after total resection. If the subependymal lesion of tuberous sclerosis complex locates around the foramen of Monro and induces hydrocephalus or trends to progressive growing, it should be considered as subependymal giant cell astrocytoma and must be treated earlier.