Extraocular retinoblastoma is associated with a very poor outcome. At Children's Hospital Los Angeles, 10 of 207 patients with retinoblastoma had extraocular disease. Four patients with no histopathologic risk factors developed extraocular disease. All patients with direct extension into the central nervous system or with distant metastatic disease died. One of three patients with trilateral retinoblastoma and one patient with regional recurrence are alive after autologous bone marrow transplant. Patients with extraocular retinoblastoma who achieve remission may benefit from consolidation of their therapy with autologous bone marrow transplant.
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http://dx.doi.org/10.1097/00043426-200401000-00011 | DOI Listing |
Semin Ophthalmol
January 2025
Department of Ophthalmology, Sidney Kimmel Medical College of Thomas Jefferson University, Philadelphia, PA, USA.
Objective: Ciliary body medulloepithelioma (CBME), a pediatric intraocular tumor with potential for locally aggressive behavior and metastasis, may present with a diverse spectrum of clinical and histopathologic features leading to diagnostic and management challenges. Examination of unusual CBME cases highlights challenges and modern diagnostic techniques which facilitate accurate diagnosis and guide management.
Methods: A retrospective clinicopathologic analysis of 6 patients with unusual clinical or pathologic features of CBME was performed.
Biomedicines
January 2025
Kellogg Eye Center, Department of Ophthalmology and Visual Science, University of Michigan, Ann Arbor, MI 48105, USA.
Intraocular malignant tumors are rare; however, they can cause serious life-threatening complications. Uveal melanoma (UM) and retinoblastoma (RB) are the most common intraocular tumors in adults and children, respectively, and come with a great disease burden. For many years, several different treatment modalities for UM and RB have been proposed, with chemotherapy for RB cases and plaque radiation therapy for localized UM as first-line treatment options.
View Article and Find Full Text PDFSemin Ophthalmol
January 2025
SJD Pediatric Cancer Center Barcelona, Hospital Sant Joan de Deu, Barcelona, Spain.
Background: Metastatic retinoblastoma remains a significant challenge in pediatric oncology, with stark disparities in survival outcomes between high-income countries (HICs) and low-income countries (LICs). Delayed diagnosis and treatment, driven by socioeconomic factors and limitations in healthcare systems, contribute to poorer outcomes in LICs. Histopathological characteristics, including high-risk pathology factors (HRPFs) and the extent of ocular tumor invasion, are critical for predicting metastatic risk and guiding treatment strategies.
View Article and Find Full Text PDFOphthalmol Sci
October 2024
International Centre for Eye Health, London School of Hygiene & Tropical Medicine, London, United Kingdom.
Purpose: To study the treatment and outcomes of children with retinoblastoma (RB) with extraocular tumor extension (RB-EOE) and compare them with RB without extraocular tumor extension (RB-w/o-EOE).
Design: Multicenter intercontinental collaborative prospective study from 2017 to 2020. RB-EOE cases included those with overt orbital tumor extension in treatment-naive patients.
A 3-year-old girl treated with intravenous chemotherapy for bilateral retinoblastoma (RB) and a standard technique of intravitreal topotecan for vitreous seeds in the left eye developed a conjunctival nodule at the injection site. Ultrasound biomicroscopy showed normal underlying sclera and ciliary body. Fundus examination of the left eye showed partly calcified vitreous seeds.
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