Subependymal Giant-Cell Astrocytoma in Tuberous Sclerosis: Endoscopic Images and the Implications for Therapy

Supratentorial intraventricular tumors are not freqently encountered in childhood. One of the most frequent intraventricular glial tumors is the subependymal giant-cell astrocytoma, mostly associated with tuberous sclerosis. These tumors are diagnosed on computed tomography (CT) or magnetic resonance imaging (MRI) scans. They can occur isolated or multiple and operative resection is advised if these tumors cause symptoms, usually raised intracranial pressure due to obstructive hydrocephalus. However, the number of tumors can be much higher than seen on radiological examination making total resection of all tumors impossible. We demonstrate this with the endoscopic images derived during the endoscopic removal of a subependymal giant-cell astrocytoma obstructing a foramen of Monro in a 15-year-old boy with tuberous sclerosis.

Key words:

Endoscopy - Operative treatment - Subependymal giant-cell astrocytoma - Tuberous sclerosis

References

• 1 Braffman B H, Bilaniuk L T, Naidich T P, Altman N R, Post M J, Quencer R M, Zimmerman R A. MR imaging of tuberous sclerosis: pathogenesis of this phakomatosis, use of gadopentetate dimeglumine and literature review.  Radiology. 1992;  183 227-238
• 2 Chow C W, Klug G L, Lewis E A. Subependymal giant-cell astrocytoma in children. An unusual discrepancy between histological and clinical features.  J Neurosurg. 1988;  68 880-883
• 3 Conzen M, Oppel F. Tuberous sclerosis in neurosurgery. An analysis of 18 patients.  Acta Neurochir (Wien). 1990;  106 106-109
• 4 Fujiwara S, Takaki T, Nishio S. Subependymal giant-cell astrocytoma associated with tuberous sclerosis. Do subependymal nodules grow?.  Child's Nerv Syst. 1989;  5 43-44
• 5 Gomez M R. Varieties of expression of tuberous sclerosis.  Neurofibromatosis. 1988;  1 330-338
• 6 Hirsch J R, Sainte-Rose C. A new surgical approach to subcortical lesions: balloon inflation and cortical gluing.  J Neurosurg. 1991;  74 1014-1017
• 7 McLaurin R L, Towbin R B. Tuberous sclerosis: diagnostic and surgical considerations.  Pediatr Neurosci. 1986;  12 43-48
• 8 Menor F, Marti-Bonmati L, Mulas F, Poyatos C, Cortina H. Neuroimaging in tuberous sclerosis: a clinicoradiological evaluation in pediatric patients.  Pediatr Radiol. 1992;  22 485-489
• 9 Morimoto K, Mogami H. Sequential CT study of subependymal giant-cell astrocytoma associated with tuberous sclerosis.  J Neurosurg. 1986;  65 874-877
• 10 Roszkowski M, Drabik K, Barszcz S, Jozwiak S. Surgical treatment of intraventricular tumors associated with tuberous sclerosis.  Child's Nerv Syst. 1995;  11 335-339
• 11 Russell D S, Rubinstein L J. Pathology of tumors of the nervous system. Baltimore: Williams & Wilkins, 5th edition 1989: 114-120
• 12 Schut L, Duhaime A C, Sutton L N. Phakomatoses: surgical considerations. In: American Association of Neurological Surgeons (eds). Pediatric Neurosurgery: surgery of the developing nervous system Philadelphia: Saunders 1989: 453-462
• 13 Sheperd C W, Gomez M R, Lie J T, Crowson C S. Causes of death in patients with tuberous sclerosis.  Mayo Clin Proc. 1991;  66 792-796
• 14 Shitara N, Yoshida J, Kageyama N, Takakura K. Clinical aspects of tuberous sclerosis and associated brain tumors. In: Ishibash Y, Hori Y (eds). Tuberous sclerosis and neurofibromatosis: epidemiology, pathophysiology, biology and management Amsterdam: Elsevier 1990: 323-330

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