OPTIC DISK ASTROCYTOMA UNASSOCIATED WITH TUBEROUS SCLEROSIS COMPLEX MANAGED WITH SURGICAL EXCISION AND A 7-YEAR FOLLOW-UP.

PURPOSE: Optic disk astrocytoma is a rare variant of congenital hamartoma, which is usually associated with tuberous sclerosis complex. Generally, it is stationary but in rare cases, it can show progressive growth and cause severe local complications.

METHODS

Clinical-pathologic correlation.

RESULTS

A 10-year-old boy presented a unilateral optic disk mass with vitreous hemorrhage that eventually necessitated vitrectomy of the affected eye. Visual acuity was hand movements at presentation. Pars plana vitrectomy was performed because of sudden loss of vision. At the time of vitrectomy, a yellow tumor that measured 1.5 mm in basal diameter and 6 mm in thickness was surgically removed. Cytological analysis revealed benign giant cells. The cells were negative for HMB-45 but stained positively for glial fibrillary acidic protein and neuron-specific enolase compatible with an astrocytic lesion. At the follow-up of 7 years, his vision was 20/80 and there has been no recurrence of the tumor.

CONCLUSION

Optic disk astrocytoma can be managed with local surgical excision and vision can be preserved if treated timely. Histopathologic features can help evaluate the degree of nuclear pleomorphism and cell morphology. Special immunohistochemical studies of the tumor's coexpressed neuronal markers will help differentiate astrocytoma from other neoplasms.