ASTROCYTIC TUMORS OF THE RETINA - DIFFERENTIATION OF SPORADIC TUMORS FROM PHAKOMATOSIS-ASSOCIATED TUMORS

  • 1 January 1984
    • journal article
    • research article
    • Vol. 108  (2) , 160-163
Abstract
A single, 1.3 cm, intraocular tumor, composed of interlacing, spindle-shaped astrocytes, involved the retina and optic disc of a 13 mo. old girl. Neither the patient nor her family had stigmas of a phakomatosis. A total of 42 previous cases of histologically documented astrocytic tumors of the retina were found: 24 patients (57%) had tuberous sclerosis, 6 patients (14%) had neurofibromatosis, and 12 patients (29%) were otherwise normal. Patients with tuberous sclerosis usually had multiple, peripheral, retinal tumors containing giant astrocytes. Patients with neurofibromatosis and otherwise normal patients more frequently had disc-based tumors. However, 3 patients (50%) with neurofibromatosis had multiple tumors, whereas multiplicity occurred in only 1 of the patients in the sporadic group. If the tumor is single, is disc based, and lacks giant cells, the patient is not at great risk of tuberous sclerosis.

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