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Treatment of Extracranial Metastatic Retinoblastoma
Source: Retinoblastoma Treatment (PDQ®)–Health Professional Version, National Cancer Institute.
Source updated: April 3, 2025 · Captured 2026-09-09.
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Context: Treatment of Extraocular Retinoblastoma
Treatment options for extracranial metastatic retinoblastoma include the following:
Systemic chemotherapy followed by myeloablative chemotherapy with stem cell rescue and radiation therapy.
Hematogenous metastases may develop in the bones, bone marrow, and, less frequently, the liver. The COG conducted a prospective international trial (ARET0321 [NCT00554788]) for patients with extraocular retinoblastoma. The study showed that intensified therapy improved the outcomes of patients with stage IVa disease.[1][Level of evidence B4] Patients with stage IVa retinoblastoma (n = 18) were treated with four cycles of induction therapy (vincristine, cisplatin, cyclophosphamide, and etoposide).
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The patients who had more than a partial response then received one cycle of high-dose carboplatin, thiotepa, and etoposide with autologous hematopoietic stem cell support. Patients with residual tumor after chemotherapy received radiation therapy.
With a median follow up of 7.3 years, the 3-year EFS and OS rates were 76.7%.
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