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Head and Neck
Diagnosis
Orbit
Malignant Tumors
Retinoblastoma
Retinoblastoma
Daniel E. Meltzer, 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 plus midline intracranial neuroblastic tumor, typically pineal
                • Quadrilateral (tetralateral) RB: Bilateral disease plus pineal and suprasellar tumors
                • Retinocytoma: Rare benign variant, 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. Wyse E et al: A review of the literature for intra-arterial chemotherapy used to treat retinoblastoma. Pediatr Radiol. 46(9):1223-33, 2016
                                              2. 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
                                              3. 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
                                              4. Dunkel IJ et al: Trilateral retinoblastoma: potentially curable with intensive chemotherapy. Pediatr Blood Cancer. 54(3):384-7, 2010
                                              5. Mallipatna AC et al: Management and outcome of unilateral retinoblastoma. J AAPOS. 13(6):546-50, 2009
                                              6. 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
                                              7. Yu CL et al: Cause-specific mortality in long-term survivors of retinoblastoma. J Natl Cancer Inst. 101(8):581-91, 2009
                                              8. de Graaf P et al: Eye size in retinoblastoma: MR imaging measurements in normal and affected eyes. Radiology. 244(1):273-80, 2007
                                              9. Kim JW et al: Current management strategies for intraocular retinoblastoma. Drugs. 67(15):2173-85, 2007
                                              10. Murphree L: Staging and grouping of retinoblastoma. In Singh A et al: Clinical Ophthalmic Oncology. Philadelphia, PA: Saunders Elsevier, 422-7, 2007
                                              11. Aerts I et al: Retinoblastoma. Orphanet J Rare Dis. 1:31, 2006
                                              12. Shields CL et al: The International Classification of Retinoblastoma predicts chemoreduction success. Ophthalmology. 113(12):2276-80, 2006
                                              13. de Graaf P et al: Retinoblastoma: MR imaging parameters in detection of tumor extent. Radiology. 235(1):197-207, 2005
                                              14. Shields CL et al: Continuing challenges in the management of retinoblastoma with chemotherapy. Retina. 24(6):849-62, 2004
                                              15. 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
                                              16. Schueler AO et al: High resolution magnetic resonance imaging of retinoblastoma. Br J Ophthalmol. 87(3):330-5, 2003
                                              17. 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
                                              18. De Potter P: Current treatment of retinoblastoma. Curr Opin Ophthalmol. 13(5):331-6, 2002
                                              19. Brisse HJ et al: Sonographic, CT, and MR imaging findings in diffuse infiltrative retinoblastoma: report of two cases with histologic comparison. AJNR Am J Neuroradiol. 22(3):499-504, 2001
                                              20. Shields CL et al: Recent developments in the management of retinoblastoma. J Pediatr Ophthalmol Strabismus. 36(1):8-18; quiz 35-6, 1999
                                              21. Kaufman LM et al: Retinoblastoma and simulating lesions. Role of CT, MR imaging and use of Gd-DTPA contrast enhancement. Radiol Clin North Am. 36(6):1101-17, 1998
                                              22. Barkhof F et al: MR imaging in retinoblastoma. Eur Radiol. 7(5):726-31, 1997
                                              23. Wong FL et al: Cancer incidence after retinoblastoma. Radiation dose and sarcoma risk. JAMA. 278(15):1262-7, 1997
                                              24. Provenzale JM et al: Radiologic-pathologic correlation. Bilateral retinoblastoma with coexistent pinealoblastoma (trilateral retinoblastoma). AJNR Am J Neuroradiol. 16(1):157-65, 1995
                                              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 plus midline intracranial neuroblastic tumor, typically pineal
                                                            • Quadrilateral (tetralateral) RB: Bilateral disease plus pineal and suprasellar tumors
                                                            • Retinocytoma: Rare benign variant, 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. Wyse E et al: A review of the literature for intra-arterial chemotherapy used to treat retinoblastoma. Pediatr Radiol. 46(9):1223-33, 2016
                                                                                          2. 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
                                                                                          3. 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
                                                                                          4. Dunkel IJ et al: Trilateral retinoblastoma: potentially curable with intensive chemotherapy. Pediatr Blood Cancer. 54(3):384-7, 2010
                                                                                          5. Mallipatna AC et al: Management and outcome of unilateral retinoblastoma. J AAPOS. 13(6):546-50, 2009
                                                                                          6. 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
                                                                                          7. Yu CL et al: Cause-specific mortality in long-term survivors of retinoblastoma. J Natl Cancer Inst. 101(8):581-91, 2009
                                                                                          8. de Graaf P et al: Eye size in retinoblastoma: MR imaging measurements in normal and affected eyes. Radiology. 244(1):273-80, 2007
                                                                                          9. Kim JW et al: Current management strategies for intraocular retinoblastoma. Drugs. 67(15):2173-85, 2007
                                                                                          10. Murphree L: Staging and grouping of retinoblastoma. In Singh A et al: Clinical Ophthalmic Oncology. Philadelphia, PA: Saunders Elsevier, 422-7, 2007
                                                                                          11. Aerts I et al: Retinoblastoma. Orphanet J Rare Dis. 1:31, 2006
                                                                                          12. Shields CL et al: The International Classification of Retinoblastoma predicts chemoreduction success. Ophthalmology. 113(12):2276-80, 2006
                                                                                          13. de Graaf P et al: Retinoblastoma: MR imaging parameters in detection of tumor extent. Radiology. 235(1):197-207, 2005
                                                                                          14. Shields CL et al: Continuing challenges in the management of retinoblastoma with chemotherapy. Retina. 24(6):849-62, 2004
                                                                                          15. 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
                                                                                          16. Schueler AO et al: High resolution magnetic resonance imaging of retinoblastoma. Br J Ophthalmol. 87(3):330-5, 2003
                                                                                          17. 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
                                                                                          18. De Potter P: Current treatment of retinoblastoma. Curr Opin Ophthalmol. 13(5):331-6, 2002
                                                                                          19. Brisse HJ et al: Sonographic, CT, and MR imaging findings in diffuse infiltrative retinoblastoma: report of two cases with histologic comparison. AJNR Am J Neuroradiol. 22(3):499-504, 2001
                                                                                          20. Shields CL et al: Recent developments in the management of retinoblastoma. J Pediatr Ophthalmol Strabismus. 36(1):8-18; quiz 35-6, 1999
                                                                                          21. Kaufman LM et al: Retinoblastoma and simulating lesions. Role of CT, MR imaging and use of Gd-DTPA contrast enhancement. Radiol Clin North Am. 36(6):1101-17, 1998
                                                                                          22. Barkhof F et al: MR imaging in retinoblastoma. Eur Radiol. 7(5):726-31, 1997
                                                                                          23. Wong FL et al: Cancer incidence after retinoblastoma. Radiation dose and sarcoma risk. JAMA. 278(15):1262-7, 1997
                                                                                          24. Provenzale JM et al: Radiologic-pathologic correlation. Bilateral retinoblastoma with coexistent pinealoblastoma (trilateral retinoblastoma). AJNR Am J Neuroradiol. 16(1):157-65, 1995