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Orbital Langerhans Cell Histiocytosis
H. Christian Davidson, MD
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KEY FACTS

  • Terminology

    • Imaging

      • Top Differential Diagnoses

        • Pathology

          • Clinical Issues

            TERMINOLOGY

            • Abbreviations

              • Langerhans cell histiocytosis (LCH)
            • Synonyms

              • Eosinophilic granuloma (EG)
                • Unifocal disease involving single location
                • Can also refer to orbit + limited bone lesions
              • Hand-Schüller-Christian (HSC)
                • Multifocal disease in single organ
                • Triad of skull lesions, proptosis, and diabetes insipidus
              • Letterer-Siwe (LS)
                • Multifocal disease involving multiple organs
                • Acute fulminant form involving multiple organ systems
              • Histiocytosis X
                • Outdated term
            • Definitions

              • Spectrum of diseases characterized by proliferation of pathological Langerhans-type histiocytes

            IMAGING

            • General Features

              • CT Findings

                • MR 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

                                      • Reporting Tips

                                        Selected References

                                        1. Bhanage AB et al: Langerhans cell histiocytosis with presentation as orbital disease. J Pediatr Neurosci. 10(2):162-5, 2015
                                        2. Esmaili N et al: Langerhans cell histiocytosis of the orbit: spectrum of disease and risk of central nervous system sequelae in unifocal cases. Ophthal Plast Reconstr Surg. ePub, 2015
                                        3. Herwig MC et al: Langerhans cell histiocytosis of the orbit: five clinicopathologic cases and review of the literature. Surv Ophthalmol. 58(4):330-40, 2013
                                        4. Giovannetti F et al: Langerhans cell histiocytosis with orbital involvement: our experience. J Oral Maxillofac Surg. 67(1):212-6, 2009
                                        5. Kempster R et al: Langerhans cell histiocytosis mimicking preseptal cellulitis. J Pediatr Ophthalmol Strabismus. 46(2):108-11, 2009
                                        6. Lee Y et al: Orbital langerhans cell histiocytosis. Int Ophthalmol Clin. 49(1):123-31, 2009
                                        7. Vosoghi H et al: Orbital involvement in langerhans cell histiocytosis. Ophthal Plast Reconstr Surg. 25(6):430-3, 2009
                                        8. Cheung N et al: Orbital Langerhans cell histiocytosis in adults. Ophthalmology. 114(8):1569-73, 2007
                                        9. Harris GJ: Langerhans cell histiocytosis of the orbit: a need for interdisciplinary dialogue. Am J Ophthalmol. 141(2):374-378, 2006
                                        10. Erly WK et al: Orbital histiocytosis X. AJNR Am J Neuroradiol. 16(6):1258-61, 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

                                                  TERMINOLOGY

                                                  • Abbreviations

                                                    • Langerhans cell histiocytosis (LCH)
                                                  • Synonyms

                                                    • Eosinophilic granuloma (EG)
                                                      • Unifocal disease involving single location
                                                      • Can also refer to orbit + limited bone lesions
                                                    • Hand-Schüller-Christian (HSC)
                                                      • Multifocal disease in single organ
                                                      • Triad of skull lesions, proptosis, and diabetes insipidus
                                                    • Letterer-Siwe (LS)
                                                      • Multifocal disease involving multiple organs
                                                      • Acute fulminant form involving multiple organ systems
                                                    • Histiocytosis X
                                                      • Outdated term
                                                  • Definitions

                                                    • Spectrum of diseases characterized by proliferation of pathological Langerhans-type histiocytes

                                                  IMAGING

                                                  • General Features

                                                    • CT Findings

                                                      • MR 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

                                                                            • Reporting Tips

                                                                              Selected References

                                                                              1. Bhanage AB et al: Langerhans cell histiocytosis with presentation as orbital disease. J Pediatr Neurosci. 10(2):162-5, 2015
                                                                              2. Esmaili N et al: Langerhans cell histiocytosis of the orbit: spectrum of disease and risk of central nervous system sequelae in unifocal cases. Ophthal Plast Reconstr Surg. ePub, 2015
                                                                              3. Herwig MC et al: Langerhans cell histiocytosis of the orbit: five clinicopathologic cases and review of the literature. Surv Ophthalmol. 58(4):330-40, 2013
                                                                              4. Giovannetti F et al: Langerhans cell histiocytosis with orbital involvement: our experience. J Oral Maxillofac Surg. 67(1):212-6, 2009
                                                                              5. Kempster R et al: Langerhans cell histiocytosis mimicking preseptal cellulitis. J Pediatr Ophthalmol Strabismus. 46(2):108-11, 2009
                                                                              6. Lee Y et al: Orbital langerhans cell histiocytosis. Int Ophthalmol Clin. 49(1):123-31, 2009
                                                                              7. Vosoghi H et al: Orbital involvement in langerhans cell histiocytosis. Ophthal Plast Reconstr Surg. 25(6):430-3, 2009
                                                                              8. Cheung N et al: Orbital Langerhans cell histiocytosis in adults. Ophthalmology. 114(8):1569-73, 2007
                                                                              9. Harris GJ: Langerhans cell histiocytosis of the orbit: a need for interdisciplinary dialogue. Am J Ophthalmol. 141(2):374-378, 2006
                                                                              10. Erly WK et al: Orbital histiocytosis X. AJNR Am J Neuroradiol. 16(6):1258-61, 1995