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Trilateral retinoblastoma.

Célia B G Antoneli1, Karina de Cássia B Ribeiro, Luis Henrique Sakamoto

  • 1Pediatric Oncology Department, Centro de Tratamento e Pesquisa Hospital do Câncer A C Camargo, São Paulo, Brazil. cantoneli@terra.com.br

Pediatric Blood & Cancer
|March 31, 2006
PubMed
Summary

Trilateral retinoblastoma (TRB), a rare hereditary cancer, has a poor prognosis. Early neuroimaging screening is crucial for detecting TRB in children with retinoblastoma, but outcomes remain challenging.

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Area of Science:

  • Pediatric Oncology
  • Ophthalmology
  • Neuro-oncology

Background:

  • Trilateral retinoblastoma (TRB) is a rare hereditary syndrome combining retinoblastoma (Rb) with intracranial neuroblastic tumors.
  • This condition has a very poor prognosis despite its low incidence.

Observation:

  • The study reviewed four cases of TRB diagnosed between 1986 and 2003.
  • Patients were diagnosed with Rb at ages 4-24 months and TRB at ages 10-72 months.
  • Two of the four patients had a positive family history.

Findings:

  • All four patients with TRB, including those with unilateral or bilateral Rb, died within 7-12 months of diagnosis.
  • Treatment included enucleation, chemotherapy, and/or external beam radiation therapy (EBRT).

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Implications:

  • Early diagnosis through neuroimaging screening is vital for TRB management.
  • New therapeutic strategies are urgently needed to improve survival rates for children with TRB.