title: Aggressive retinal astrocytomas in four patients with tuberous sclerosis complex.
abstract: OBJECTIVE
  To report the clinical and histopathologic findings of retinal astrocytic tumors that showed progressive growth in four patients with tuberous sclerosis complex (TSC).
  
  
  METHODS
  Four young children each developed an enlarging retinal neoplasm that eventually necessitated enucleation of the affected eye. The systemic findings, clinical course, and histopathologic findings were reviewed.
  
  
  RESULTS
  Each patient had a progressively enlarging retinal mass associated with a total exudative retinal detachment and neovascular glaucoma. Enucleation was necessary in each case because the affected eye became blind and painful. The mean patient age at enucleation was 7 years, and the median age was 3 years. At the time of enucleation the tumors ranged from 10 to 20 mm in basal diameter and from 10 to 25 mm in thickness. Histopathologic studies of each eye revealed a giant cell astrocytoma that had produced a total exudative retinal detachment. The tumor cells showed positive immunoreactivity to neuron-specific enolase and glial fibrillary acidic protein. The retinal neoplasms in these cases were identical histopathologically to the subependymal giant cell astrocytoma that typifies TSC in the brain. One tumor filled the entire eye and perforated the globe. Although the lesions simulated retinoblastoma clinically, each patient had ocular and systemic findings of TSC, supporting the diagnosis of astrocytic hamartoma.
  
  
  CONCLUSIONS
  Although retinal astrocytic lesions of TSC generally are stationary, they can sometimes grow relentlessly and cause severe ocular complications. Patients with retinal astrocytic hamartomas should have serial ophthalmic evaluations because of this possibility.
authors.affiliation: Oncology Service, Wills Eye Hospital, Thomas Jefferson University, Philadelphia, Pennsylvania, USA.
authors.forename: Jerry A
authors.initials: JA
authors.lastname: Shields
authors.forename: Ralph C
authors.initials: RC
authors.lastname: Eagle
authors.forename: Carol L
authors.initials: CL
authors.lastname: Shields
authors.forename: Brian P
authors.initials: BP
authors.lastname: Marr
mesh_terms: D001254:Astrocytoma / Q000150:complications / Q000209:etiology* / Q000473:pathology* / Q000601:surgery; D001766:Blindness / Q000209:etiology; D002648:Child; D002675:Child, Preschool; D015353:Eye Enucleation; D005260:Female; D015355:Glaucoma, Neovascular / Q000209:etiology; D006801:Humans; D007223:Infant; D008297:Male; D010146:Pain / Q000209:etiology; D012163:Retinal Detachment / Q000209:etiology; D019572:Retinal Neoplasms / Q000150:complications / Q000209:etiology* / Q000473:pathology* / Q000601:surgery; D014402:Tuberous Sclerosis / Q000150:complications*