Trilateral retinoblastoma: Ocular and pineal retinoblastomas

D. L. Johnson, R. Chandra, W. S. Fisher, M. K. Hammock, C. A. McKeown

Research output: Contribution to journalArticle

23 Scopus citations

Abstract

Trilateral retinoblastomas, the syndrome of bilateral retinoblastoma associated with ectopic retinoblastoma in the pineal gland, is rare but well recognized. In contrast to bilateral retinoblastomas alone, the ocular retinoblastomas in trilateral retinoblastoma develop before the age of 6 months, and a family history positive for retinoblastoma is usually obtained. The retinal tumors are often quiescent at the time that the pineal tumor is discovered, and show no evidence of metastatic spread after enucleation of the globes. Pathologically, the pineal tumor is indistinguishable from the ocular retinoblastoma. The pathophysiology of this syndrome is not well understood, but a germinal mutation is thought to target photoreceptor tissue for further postzygotic mutation. Eventual expression depends on the inherited host resistance to the carcinogenic manifestation of these genes. The low host resistance of trilateral retinoblastoma is evident by the early age of presentation, the multicentric occurrence of the tumor, and the high early mortality rate despite aggressive management.

Original languageEnglish (US)
Pages (from-to)367-370
Number of pages4
JournalJournal of neurosurgery
Volume63
Issue number3
DOIs
StatePublished - Jan 1 1985
Externally publishedYes

ASJC Scopus subject areas

  • Surgery
  • Clinical Neurology

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