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Orbital Langerhans Cell Histiocytosis
Bernadette L. Koch, MD; H. Christian Davidson, MD
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KEY FACTS

  • Terminology

    • Imaging

      • Top Differential Diagnoses

        • Clinical Issues

          TERMINOLOGY

          • Abbreviations

            • Langerhans cell histiocytosis (LCH)
          • Synonyms

            • Historic terminology/classification: 3 subsets of histiocytosis X
              • Eosinophilic granuloma (EG): Unifocal disease involving single location
              • Hand-Schüller-Christian (HSC)
                • Multifocal disease in single organ
                • Triad of skull lesions, proptosis, & diabetes insipidus
              • Letterer-Siwe (LS)
                • Multifocal disease involving multiple organs
                • Acute fulminant form involving multiple organ systems
          • Definitions

            • Langerhans cell histiocytosis (LCH): Spectrum of disorders caused by neoplastic clonal proliferations of CD1a, CD207, & S100 protein (+) dendritic cells
            • Single system (SS) (unifocal or multifocal) vs. multisystem (MS) disease
              • Bone (80-90%) & skin (40-50%) are most frequent
              • Risk organ (RO) involvement: Liver, spleen, marrow; confers worse prognosis (high risk)
              • Other organs: Lymph nodes, pituitary, thymus, GI tract
              • CNS-risk lesion: Skull base & many facial lesions
                • Diabetes insipidus (25% overall, 50% with MS disease), growth hormone deficiency (10%), parenchymal mass lesions (1%), & delayed neurodegenerative changes

          IMAGING

          • General Features

            • CT Findings

              • MR Findings

                • Nuclear Medicine 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. Jessop S et al: FDG PET-CT in pediatric Langerhans cell histiocytosis. Pediatr Blood Cancer. 67(1):e28034, 2020
                                        2. Kim JR et al: Comparison of whole-body MRI, bone scan, and radiographic skeletal survey for lesion detection and risk stratification of Langerhans cell histiocytosis. Sci Rep. 9(1):317, 2019
                                        3. Krooks J et al: Langerhans cell histiocytosis in children: history, classification, pathobiology, clinical manifestations, and prognosis. J Am Acad Dermatol. 78(6):1035-44, 2018
                                        4. Bhanage AB et al: Langerhans cell histiocytosis with presentation as orbital disease. J Pediatr Neurosci. 10(2):162-5, 2015
                                        5. 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. 32(1):28-34, 2015
                                        6. 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
                                        7. Giovannetti F et al: Langerhans cell histiocytosis with orbital involvement: our experience. J Oral Maxillofac Surg. 67(1):212-6, 2009
                                        8. Kempster R et al: Langerhans cell histiocytosis mimicking preseptal cellulitis. J Pediatr Ophthalmol Strabismus. 46(2):108-11, 2009
                                        9. Lee Y et al: Orbital langerhans cell histiocytosis. Int Ophthalmol Clin. 49(1):123-31, 2009
                                        10. Vosoghi H et al: Orbital involvement in langerhans cell histiocytosis. Ophthal Plast Reconstr Surg. 25(6):430-3, 2009
                                        11. Cheung N et al: Orbital Langerhans cell histiocytosis in adults. Ophthalmology. 114(8):1569-73, 2007
                                        12. Harris GJ: Langerhans cell histiocytosis of the orbit: a need for interdisciplinary dialogue. Am J Ophthalmol. 141(2):374-378, 2006
                                        13. 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

                                              • Clinical Issues

                                                TERMINOLOGY

                                                • Abbreviations

                                                  • Langerhans cell histiocytosis (LCH)
                                                • Synonyms

                                                  • Historic terminology/classification: 3 subsets of histiocytosis X
                                                    • Eosinophilic granuloma (EG): Unifocal disease involving single location
                                                    • Hand-Schüller-Christian (HSC)
                                                      • Multifocal disease in single organ
                                                      • Triad of skull lesions, proptosis, & diabetes insipidus
                                                    • Letterer-Siwe (LS)
                                                      • Multifocal disease involving multiple organs
                                                      • Acute fulminant form involving multiple organ systems
                                                • Definitions

                                                  • Langerhans cell histiocytosis (LCH): Spectrum of disorders caused by neoplastic clonal proliferations of CD1a, CD207, & S100 protein (+) dendritic cells
                                                  • Single system (SS) (unifocal or multifocal) vs. multisystem (MS) disease
                                                    • Bone (80-90%) & skin (40-50%) are most frequent
                                                    • Risk organ (RO) involvement: Liver, spleen, marrow; confers worse prognosis (high risk)
                                                    • Other organs: Lymph nodes, pituitary, thymus, GI tract
                                                    • CNS-risk lesion: Skull base & many facial lesions
                                                      • Diabetes insipidus (25% overall, 50% with MS disease), growth hormone deficiency (10%), parenchymal mass lesions (1%), & delayed neurodegenerative changes

                                                IMAGING

                                                • General Features

                                                  • CT Findings

                                                    • MR Findings

                                                      • Nuclear Medicine 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. Jessop S et al: FDG PET-CT in pediatric Langerhans cell histiocytosis. Pediatr Blood Cancer. 67(1):e28034, 2020
                                                                              2. Kim JR et al: Comparison of whole-body MRI, bone scan, and radiographic skeletal survey for lesion detection and risk stratification of Langerhans cell histiocytosis. Sci Rep. 9(1):317, 2019
                                                                              3. Krooks J et al: Langerhans cell histiocytosis in children: history, classification, pathobiology, clinical manifestations, and prognosis. J Am Acad Dermatol. 78(6):1035-44, 2018
                                                                              4. Bhanage AB et al: Langerhans cell histiocytosis with presentation as orbital disease. J Pediatr Neurosci. 10(2):162-5, 2015
                                                                              5. 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. 32(1):28-34, 2015
                                                                              6. 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
                                                                              7. Giovannetti F et al: Langerhans cell histiocytosis with orbital involvement: our experience. J Oral Maxillofac Surg. 67(1):212-6, 2009
                                                                              8. Kempster R et al: Langerhans cell histiocytosis mimicking preseptal cellulitis. J Pediatr Ophthalmol Strabismus. 46(2):108-11, 2009
                                                                              9. Lee Y et al: Orbital langerhans cell histiocytosis. Int Ophthalmol Clin. 49(1):123-31, 2009
                                                                              10. Vosoghi H et al: Orbital involvement in langerhans cell histiocytosis. Ophthal Plast Reconstr Surg. 25(6):430-3, 2009
                                                                              11. Cheung N et al: Orbital Langerhans cell histiocytosis in adults. Ophthalmology. 114(8):1569-73, 2007
                                                                              12. Harris GJ: Langerhans cell histiocytosis of the orbit: a need for interdisciplinary dialogue. Am J Ophthalmol. 141(2):374-378, 2006
                                                                              13. Erly WK et al: Orbital histiocytosis X. AJNR Am J Neuroradiol. 16(6):1258-61, 1995