Multifaceted Chemotherapy for Trilateral Retinoblastoma | Pediatric Cancer | JAMA Ophthalmology | JAMA Network
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Small Case Series
March 14, 2011

Multifaceted Chemotherapy for Trilateral Retinoblastoma

Author Affiliations

Author Affiliations: Division of Hematology and Oncology (Drs Dimaras, Doyle, and Chan), and Departments of Pediatrics (Drs Dimaras, Doyle, and Chan), Ophthalmology and Visual Science (Drs Héon and Gallie), Pathology (Dr Halliday), and Diagnostic Imaging (Dr Babyn), The Hospital for Sick Children, Toronto, Ontario, Canada, and Division of Hematology and Oncology (Dr Strahlendorf), Departments of Pediatrics (Dr Strahlendorf) and Ophthalmology (Dr Paton), Children's and Women's Health Centre of British Columbia and University of British Columbia, Vancouver, Canada.

Arch Ophthalmol. 2011;129(3):360-371. doi:10.1001/archophthalmol.2011.17

Trilateral retinoblastoma (TRB) occurs in 3% of patients with unilateral or bilateral germline retinoblastoma.1 This midline malignant neuroectodermal tumor arises commonly in the pineal gland (77%-83% of patients) and less frequently in the parasellar region (17%-23% of patients).2,3

Trilateral retinoblastoma is difficult to treat and usually fatal.3 Complete resection is seldom possible for tumors in the pineal or parasellar locations.4 Craniospinal irradiation is too damaging to the growth, intellectual, cognitive, and endocrine functions, particularly for children younger than 3 years of age.5-7 Chemotherapy alone rarely cures young children with other intracranial neuroectodermal tumors, such as medulloblastoma.8 Trilateral retinoblastoma often presents with dissemination in the cerebrospinal fluid (CSF) (leptomeningeal TRB or neoplastic meningitis) and is extremely difficult to cure because most intrathecal drugs are ineffective for solid-tumor CSF metastases.9