Trilateral retinoblastoma with suprasellar tumor and associated pineal cyst

J Pediatr Hematol Oncol. 2007 Jan;29(1):53-6. doi: 10.1097/MPH.0b013e3180308782.

Abstract

Trilateral retinoblastoma (TRb) is a well-known syndrome associating hereditary retinoblastoma (Rb) with an intracranial neuroblastic tumor arising usually in the pineal region, rarely at the suprasellar or parasellar site. It develops in most cases after diagnosis of Rb. The outcome is usually fatal because of secondary spinal dissemination. Pineal cysts have recently been reported as a benign variant of TRb. We report the unusual presentation of a TRb in a 12-month-old boy with extensive bilateral Rb, a voluminous suprasellar tumor, pineal cyst, and leptomeningeal disease. The special features of this "quadrilateral" Rb are discussed.

Publication types

  • Case Reports

MeSH terms

  • Antineoplastic Combined Chemotherapy Protocols / administration & dosage
  • Carboplatin / administration & dosage
  • Central Nervous System Cysts / diagnostic imaging*
  • Central Nervous System Cysts / drug therapy
  • Central Nervous System Cysts / secondary
  • Child
  • Cyclophosphamide / administration & dosage
  • Eye Neoplasms / diagnostic imaging*
  • Eye Neoplasms / drug therapy
  • Humans
  • Male
  • Meningeal Neoplasms / diagnostic imaging*
  • Meningeal Neoplasms / drug therapy
  • Meningeal Neoplasms / secondary
  • Pineal Gland / diagnostic imaging*
  • Radiography
  • Retinoblastoma / diagnostic imaging*
  • Retinoblastoma / drug therapy
  • Thiotepa / administration & dosage

Substances

  • Cyclophosphamide
  • Thiotepa
  • Carboplatin