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Pediatrics
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Head and Neck
Orbit
Retinoblastoma
Retinoblastoma
Bernadette L. Koch, MD
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KEY FACTS

  • Terminology

    • Imaging

      • Top Differential Diagnoses

        • Pathology

          • Clinical Issues

            • Diagnostic Checklist

              TERMINOLOGY

              • Abbreviations

                • Retinoblastoma (RB)

              • Definitions

                • Malignant primary retinal neoplasm
                • Trilateral RB: Bilateral ocular tumors + midline intracranial neuroblastic tumor, typically pineal
                • Quadrilateral/tetralateral RB: Bilateral disease + pineal & suprasellar tumors
                • Retinocytoma: Rare benign variant with similar genetics

              IMAGING

              • General Features

                • CT Findings

                  • MR Findings

                    • Ultrasonographic Findings

                      • Imaging Recommendations

                        DIFFERENTIAL DIAGNOSIS

                          PATHOLOGY

                          • General Features

                            • Staging, Grading, & Classification

                              • Gross Pathologic & Surgical Features

                                • Microscopic Features

                                  CLINICAL ISSUES

                                  • Presentation

                                    • Demographics

                                      • Natural History & Prognosis

                                        • Treatment

                                          DIAGNOSTIC CHECKLIST

                                          • Consider

                                            • Image Interpretation Pearls

                                              Selected References

                                              1. Li Z et al: Diagnosis of postlaminar optic nerve invasion in retinoblastoma with MRI features. J Magn Reson Imaging. 51(4):1045-52, 2020
                                              2. Chen Q et al: Comparison between intravenous chemotherapy and intra-arterial chemotherapy for retinoblastoma: a meta-analysis. BMC Cancer. 18(1):486, 2018
                                              3. Wyse E et al: A review of the literature for intra-arterial chemotherapy used to treat retinoblastoma. Pediatr Radiol. 46(9):1223-33, 2016
                                              4. Yousef YA et al: Intra-arterial chemotherapy for retinoblastoma: a systematic review. JAMA Ophthalmol. 134(5):584-91, 2016
                                              5. Abramson DH et al: Treatment of retinoblastoma in 2015: agreement and disagreement. JAMA Ophthalmol. 133(11):1341-7, 2015
                                              6. Delhiwala KS et al: Retinoblastoma: an update. Semin Diagn Pathol. 33(3):133-40, 2015
                                              7. Learned KO et al: Imaging of the postoperative orbit. Neuroimaging Clin N Am. 25(3):457-76, 2015
                                              8. Sirin S et al: High-resolution MRI using orbit surface coils for the evaluation of metastatic risk factors in 143 children with retinoblastoma: Part 1: MRI vs. histopathology. Neuroradiology. 57(8):805-14, 2015
                                              9. Barnwell JD et al: MR imaging of progressive enhancement of a bioceramic orbital prosthesis: an indicator of fibrovascular invasion. AJNR Am J Neuroradiol. 32(1):E8-9, 2011
                                              10. de Graaf P et al: Contrast-enhancement of the anterior eye segment in patients with retinoblastoma: correlation between clinical, MR imaging, and histopathologic findings. AJNR Am J Neuroradiol. 31(2):237-45, 2010
                                              11. Dunkel IJ et al: Trilateral retinoblastoma: potentially curable with intensive chemotherapy. Pediatr Blood Cancer. 54(3):384-7, 2010
                                              12. Mallipatna AC et al: Management and outcome of unilateral retinoblastoma. J AAPOS. 13(6):546-50, 2009
                                              13. Wilson MW et al: Lack of correlation between the histologic and magnetic resonance imaging results of optic nerve involvement in eyes primarily enucleated for retinoblastoma. Ophthalmology. 116(8):1558-63, 2009
                                              14. Yu CL et al: Cause-specific mortality in long-term survivors of retinoblastoma. J Natl Cancer Inst. 101(8):581-91, 2009
                                              15. de Graaf P et al: Eye size in retinoblastoma: MR imaging measurements in normal and affected eyes. Radiology. 244(1):273-80, 2007
                                              16. Murphree L: Staging & grouping of retinoblastoma. In Singh A et al: Clinical Ophthalmic Oncology. Philadelphia: Saunders Elsevier. 422-7, 2007
                                              17. Shields CL et al: The International Classification of Retinoblastoma predicts chemoreduction success. Ophthalmology. 113(12):2276-80, 2006
                                              18. de Graaf P et al: Retinoblastoma: MR imaging parameters in detection of tumor extent. Radiology. 235(1):197-207, 2005
                                              19. Galluzzi P et al: Retinoblastoma: Abnormal gadolinium enhancement of anterior segment of eyes at MR imaging with clinical and histopathologic correlation. Radiology. 228(3):683-90, 2003
                                              20. Tateishi U et al: CT and MRI features of recurrent tumors and second primary neoplasms in pediatric patients with retinoblastoma. AJR Am J Roentgenol. 181(3):879-84, 2003
                                              Related Anatomy
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                                              Related Differential Diagnoses
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                                              References
                                              Tables

                                              Tables

                                              KEY FACTS

                                              • Terminology

                                                • Imaging

                                                  • Top Differential Diagnoses

                                                    • Pathology

                                                      • Clinical Issues

                                                        • Diagnostic Checklist

                                                          TERMINOLOGY

                                                          • Abbreviations

                                                            • Retinoblastoma (RB)

                                                          • Definitions

                                                            • Malignant primary retinal neoplasm
                                                            • Trilateral RB: Bilateral ocular tumors + midline intracranial neuroblastic tumor, typically pineal
                                                            • Quadrilateral/tetralateral RB: Bilateral disease + pineal & suprasellar tumors
                                                            • Retinocytoma: Rare benign variant with similar genetics

                                                          IMAGING

                                                          • General Features

                                                            • CT Findings

                                                              • MR Findings

                                                                • Ultrasonographic Findings

                                                                  • Imaging Recommendations

                                                                    DIFFERENTIAL DIAGNOSIS

                                                                      PATHOLOGY

                                                                      • General Features

                                                                        • Staging, Grading, & Classification

                                                                          • Gross Pathologic & Surgical Features

                                                                            • Microscopic Features

                                                                              CLINICAL ISSUES

                                                                              • Presentation

                                                                                • Demographics

                                                                                  • Natural History & Prognosis

                                                                                    • Treatment

                                                                                      DIAGNOSTIC CHECKLIST

                                                                                      • Consider

                                                                                        • Image Interpretation Pearls

                                                                                          Selected References

                                                                                          1. Li Z et al: Diagnosis of postlaminar optic nerve invasion in retinoblastoma with MRI features. J Magn Reson Imaging. 51(4):1045-52, 2020
                                                                                          2. Chen Q et al: Comparison between intravenous chemotherapy and intra-arterial chemotherapy for retinoblastoma: a meta-analysis. BMC Cancer. 18(1):486, 2018
                                                                                          3. Wyse E et al: A review of the literature for intra-arterial chemotherapy used to treat retinoblastoma. Pediatr Radiol. 46(9):1223-33, 2016
                                                                                          4. Yousef YA et al: Intra-arterial chemotherapy for retinoblastoma: a systematic review. JAMA Ophthalmol. 134(5):584-91, 2016
                                                                                          5. Abramson DH et al: Treatment of retinoblastoma in 2015: agreement and disagreement. JAMA Ophthalmol. 133(11):1341-7, 2015
                                                                                          6. Delhiwala KS et al: Retinoblastoma: an update. Semin Diagn Pathol. 33(3):133-40, 2015
                                                                                          7. Learned KO et al: Imaging of the postoperative orbit. Neuroimaging Clin N Am. 25(3):457-76, 2015
                                                                                          8. Sirin S et al: High-resolution MRI using orbit surface coils for the evaluation of metastatic risk factors in 143 children with retinoblastoma: Part 1: MRI vs. histopathology. Neuroradiology. 57(8):805-14, 2015
                                                                                          9. Barnwell JD et al: MR imaging of progressive enhancement of a bioceramic orbital prosthesis: an indicator of fibrovascular invasion. AJNR Am J Neuroradiol. 32(1):E8-9, 2011
                                                                                          10. de Graaf P et al: Contrast-enhancement of the anterior eye segment in patients with retinoblastoma: correlation between clinical, MR imaging, and histopathologic findings. AJNR Am J Neuroradiol. 31(2):237-45, 2010
                                                                                          11. Dunkel IJ et al: Trilateral retinoblastoma: potentially curable with intensive chemotherapy. Pediatr Blood Cancer. 54(3):384-7, 2010
                                                                                          12. Mallipatna AC et al: Management and outcome of unilateral retinoblastoma. J AAPOS. 13(6):546-50, 2009
                                                                                          13. Wilson MW et al: Lack of correlation between the histologic and magnetic resonance imaging results of optic nerve involvement in eyes primarily enucleated for retinoblastoma. Ophthalmology. 116(8):1558-63, 2009
                                                                                          14. Yu CL et al: Cause-specific mortality in long-term survivors of retinoblastoma. J Natl Cancer Inst. 101(8):581-91, 2009
                                                                                          15. de Graaf P et al: Eye size in retinoblastoma: MR imaging measurements in normal and affected eyes. Radiology. 244(1):273-80, 2007
                                                                                          16. Murphree L: Staging & grouping of retinoblastoma. In Singh A et al: Clinical Ophthalmic Oncology. Philadelphia: Saunders Elsevier. 422-7, 2007
                                                                                          17. Shields CL et al: The International Classification of Retinoblastoma predicts chemoreduction success. Ophthalmology. 113(12):2276-80, 2006
                                                                                          18. de Graaf P et al: Retinoblastoma: MR imaging parameters in detection of tumor extent. Radiology. 235(1):197-207, 2005
                                                                                          19. Galluzzi P et al: Retinoblastoma: Abnormal gadolinium enhancement of anterior segment of eyes at MR imaging with clinical and histopathologic correlation. Radiology. 228(3):683-90, 2003
                                                                                          20. Tateishi U et al: CT and MRI features of recurrent tumors and second primary neoplasms in pediatric patients with retinoblastoma. AJR Am J Roentgenol. 181(3):879-84, 2003