Trilateral retinoblastoma (TRB) is a rare condition characterized by an intracranial neuroblastic tumor associated with bilateral or unilateral retinoblastoma (RB). The outcome is almost always fatal. An 18-month-old patient with familial bilateral RB was referred for a pineal lesion detected on a screening by magnetic resonance imaging. The child, considered inoperable by 2 different neurosurgical teams, was treated with conventional chemotherapy (methotrexate, vincristine, vepeside, cyclophosphamide, and carboplatin) plus tandem transplantation (vepeside/carboplatin and thiotepa/mephalan) followed by local radiotherapy. At 80 months from the diagnosis of TRB, the patient is alive and in complete remission, with no neuropsychologic consequences. An early and aggressive treatment may improve the prognosis of TRB.

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http://dx.doi.org/10.1097/MPH.0b013e3181e90031DOI Listing

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Article Synopsis
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Follow-up of Cystic Pineal Glands in Retinoblastoma Patients Does Not Increase Detection of Pineal Trilateral Retinoblastoma.

Am J Ophthalmol

December 2024

From the European Retinoblastoma Imaging Collaboration (ERIC) (C.M.d.B., R.W.J., M.K., S.S., P.M., P.G., S.G., P.d.G., M.C.d.J.); Cancer Center Amsterdam, Imaging and Biomarkers (C.M.d.B., R.W.J., J.d.H., A.C.M., P.d.G., M.C.d.J.), Amsterdam, the Netherlands; Department of Radiology and Nuclear Medicine, Amsterdam UMC location Vrije Universiteit Amsterdam (C.M.d.B., R.W.J., J.d.H., P.d.G., M.C.d.J.), Amsterdam, the Netherlands.

Purpose: To evaluate the effectiveness of baseline screening and follow-up with magnetic resonance imaging (MRI) for detecting trilateral retinoblastoma (TRb) and assessing the risk of TRb development.

Design: Prospective multicenter cohort study.

Methods: A total of 607 retinoblastoma patients from 2012 through 2022 were included and followed up until September 1, 2023.

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