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Langerhans Cell Histiocytosis
A. Carlson Merrow, Jr., MD, FAAP
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KEY FACTS

  • Terminology

    • Imaging

      • Clinical Issues

        • Diagnostic Checklist

          TERMINOLOGY

          • Synonyms

            • Histiocytosis X, eosinophilic granuloma (EG), Hand-Schüller-Christian disease, Letterer-Siwe disease
          • 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 frequently involved
                • Lung is most common SS site in adults
                • Skin is only site of involvement in 2% of cases but is most common site under 2 years of age
              • Risk organ (RO) involvement: Liver, spleen, marrow; confers worse prognosis (high risk)
                • Lung is no longer considered RO
              • 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

            • Radiographic Findings

              • CT Findings

                • MR Findings

                  • Ultrasonographic Findings

                    • Nuclear Medicine Findings

                      • Imaging Recommendations

                        DIFFERENTIAL DIAGNOSIS

                          PATHOLOGY

                          • General Features

                            • Gross Pathologic & Surgical Features

                              • Microscopic Features

                                CLINICAL ISSUES

                                • Presentation

                                  • Demographics

                                    • Natural History & Prognosis

                                      • Treatment

                                        DIAGNOSTIC CHECKLIST

                                        • Consider

                                          Selected References

                                          1. Jessop S et al: FDG PET-CT in pediatric Langerhans cell histiocytosis. Pediatr Blood Cancer. 67(1):e28034, 2020
                                          2. Hinson ARP et al: Hemophagocytic lymphohistiocytosis in Langerhans cell histiocytosis: a case report and review of the literature. J Pediatr Hematol Oncol. ePub, 2019
                                          3. 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
                                          4. Allen CE et al: Langerhans-cell histiocytosis. N Engl J Med. 379(9):856-68, 2018
                                          5. 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
                                          6. Lee SW et al: Long-term clinical outcome of spinal Langerhans cell histiocytosis in children. Int J Hematol. 106(3):441-9, 2017
                                          7. Sher AC et al: PET/MR in the assessment of pediatric histiocytoses: a comparison to PET/CT. Clin Nucl Med. 42(8):582-8, 2017
                                          8. Emile JF et al: Revised classification of histiocytoses and neoplasms of the macrophage-dendritic cell lineages. Blood. 127(22):2672-81, 2016
                                          9. Samet J et al: MRI and clinical features of Langerhans cell histiocytosis (LCH) in the pelvis and extremities: can LCH really look like anything? Skeletal Radiol. 45(5):607-13, 2016
                                          10. Allen CE et al: How I treat Langerhans cell histiocytosis. Blood. 126(1):26-35, 2015
                                          11. Gelfand MJ et al: Selective CT for PET/CT: dose reduction in Langerhans cell histiocytosis. Pediatr Radiol. 45(1):81-5, 2015
                                          12. Harmon CM et al: Langerhans cell histiocytosis: a clinicopathologic review and molecular pathogenetic update. Arch Pathol Lab Med. 139(10):1211-4, 2015
                                          13. Monsereenusorn C et al: Clinical characteristics and treatment of Langerhans cell histiocytosis. Hematol Oncol Clin North Am. 29(5):853-73, 2015
                                          14. Picarsic J et al: Nosology and pathology of Langerhans cell histiocytosis. Hematol Oncol Clin North Am. 29(5):799-823, 2015
                                          15. Delprat C et al: Blood spotlight on Langerhans cell histiocytosis. Blood. 124(6):867-72, 2014
                                          16. DiCaprio MR et al: Diagnosis and management of Langerhans cell histiocytosis. J Am Acad Orthop Surg. 22(10):643-52, 2014
                                          17. Kamath S et al: Outcomes of children younger than 24 months with langerhans cell histiocytosis and bone involvement: a report from a single institution. J Pediatr Orthop. 34(8):825-30, 2014
                                          18. Lee JW et al: Clinical characteristics and treatment outcome of Langerhans cell histiocytosis: 22 years' experience of 154 patients at a single center. Pediatr Hematol Oncol. 31(3):293-302, 2014
                                          19. Zaveri J et al: More than just Langerhans cell histiocytosis: a radiologic review of histiocytic disorders. Radiographics. 34(7):2008-24, 2014
                                          20. Jeh SK et al: Extracranial skeletal Langerhans cell histiocytosis: MR imaging features according to the radiologic evolutional phases. Clin Imaging. 36(5):466-71, 2012
                                          21. Goo HW et al: Whole-body MRI of Langerhans cell histiocytosis: comparison with radiography and bone scintigraphy. Pediatr Radiol. 36(10):1019-31, 2006
                                          22. Garg S et al: Langerhans cell histiocytosis of the spine in children. Long-term follow-up. J Bone Joint Surg Am. 86-A(8):1740-50, 2004
                                          23. Schmidt S et al: Extra-osseous involvement of Langerhans' cell histiocytosis in children. Pediatr Radiol. 34(4):313-21, 2004
                                          24. Kilborn TN et al: Paediatric manifestations of Langerhans cell histiocytosis: a review of the clinical and radiological findings. Clin Radiol. 58(4):269-78, 2003
                                          Related Anatomy
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                                          Related Differential Diagnoses
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                                          References
                                          Tables

                                          Tables

                                          KEY FACTS

                                          • Terminology

                                            • Imaging

                                              • Clinical Issues

                                                • Diagnostic Checklist

                                                  TERMINOLOGY

                                                  • Synonyms

                                                    • Histiocytosis X, eosinophilic granuloma (EG), Hand-Schüller-Christian disease, Letterer-Siwe disease
                                                  • 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 frequently involved
                                                        • Lung is most common SS site in adults
                                                        • Skin is only site of involvement in 2% of cases but is most common site under 2 years of age
                                                      • Risk organ (RO) involvement: Liver, spleen, marrow; confers worse prognosis (high risk)
                                                        • Lung is no longer considered RO
                                                      • 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

                                                    • Radiographic Findings

                                                      • CT Findings

                                                        • MR Findings

                                                          • Ultrasonographic Findings

                                                            • Nuclear Medicine Findings

                                                              • Imaging Recommendations

                                                                DIFFERENTIAL DIAGNOSIS

                                                                  PATHOLOGY

                                                                  • General Features

                                                                    • Gross Pathologic & Surgical Features

                                                                      • Microscopic Features

                                                                        CLINICAL ISSUES

                                                                        • Presentation

                                                                          • Demographics

                                                                            • Natural History & Prognosis

                                                                              • Treatment

                                                                                DIAGNOSTIC CHECKLIST

                                                                                • Consider

                                                                                  Selected References

                                                                                  1. Jessop S et al: FDG PET-CT in pediatric Langerhans cell histiocytosis. Pediatr Blood Cancer. 67(1):e28034, 2020
                                                                                  2. Hinson ARP et al: Hemophagocytic lymphohistiocytosis in Langerhans cell histiocytosis: a case report and review of the literature. J Pediatr Hematol Oncol. ePub, 2019
                                                                                  3. 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
                                                                                  4. Allen CE et al: Langerhans-cell histiocytosis. N Engl J Med. 379(9):856-68, 2018
                                                                                  5. 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
                                                                                  6. Lee SW et al: Long-term clinical outcome of spinal Langerhans cell histiocytosis in children. Int J Hematol. 106(3):441-9, 2017
                                                                                  7. Sher AC et al: PET/MR in the assessment of pediatric histiocytoses: a comparison to PET/CT. Clin Nucl Med. 42(8):582-8, 2017
                                                                                  8. Emile JF et al: Revised classification of histiocytoses and neoplasms of the macrophage-dendritic cell lineages. Blood. 127(22):2672-81, 2016
                                                                                  9. Samet J et al: MRI and clinical features of Langerhans cell histiocytosis (LCH) in the pelvis and extremities: can LCH really look like anything? Skeletal Radiol. 45(5):607-13, 2016
                                                                                  10. Allen CE et al: How I treat Langerhans cell histiocytosis. Blood. 126(1):26-35, 2015
                                                                                  11. Gelfand MJ et al: Selective CT for PET/CT: dose reduction in Langerhans cell histiocytosis. Pediatr Radiol. 45(1):81-5, 2015
                                                                                  12. Harmon CM et al: Langerhans cell histiocytosis: a clinicopathologic review and molecular pathogenetic update. Arch Pathol Lab Med. 139(10):1211-4, 2015
                                                                                  13. Monsereenusorn C et al: Clinical characteristics and treatment of Langerhans cell histiocytosis. Hematol Oncol Clin North Am. 29(5):853-73, 2015
                                                                                  14. Picarsic J et al: Nosology and pathology of Langerhans cell histiocytosis. Hematol Oncol Clin North Am. 29(5):799-823, 2015
                                                                                  15. Delprat C et al: Blood spotlight on Langerhans cell histiocytosis. Blood. 124(6):867-72, 2014
                                                                                  16. DiCaprio MR et al: Diagnosis and management of Langerhans cell histiocytosis. J Am Acad Orthop Surg. 22(10):643-52, 2014
                                                                                  17. Kamath S et al: Outcomes of children younger than 24 months with langerhans cell histiocytosis and bone involvement: a report from a single institution. J Pediatr Orthop. 34(8):825-30, 2014
                                                                                  18. Lee JW et al: Clinical characteristics and treatment outcome of Langerhans cell histiocytosis: 22 years' experience of 154 patients at a single center. Pediatr Hematol Oncol. 31(3):293-302, 2014
                                                                                  19. Zaveri J et al: More than just Langerhans cell histiocytosis: a radiologic review of histiocytic disorders. Radiographics. 34(7):2008-24, 2014
                                                                                  20. Jeh SK et al: Extracranial skeletal Langerhans cell histiocytosis: MR imaging features according to the radiologic evolutional phases. Clin Imaging. 36(5):466-71, 2012
                                                                                  21. Goo HW et al: Whole-body MRI of Langerhans cell histiocytosis: comparison with radiography and bone scintigraphy. Pediatr Radiol. 36(10):1019-31, 2006
                                                                                  22. Garg S et al: Langerhans cell histiocytosis of the spine in children. Long-term follow-up. J Bone Joint Surg Am. 86-A(8):1740-50, 2004
                                                                                  23. Schmidt S et al: Extra-osseous involvement of Langerhans' cell histiocytosis in children. Pediatr Radiol. 34(4):313-21, 2004
                                                                                  24. Kilborn TN et al: Paediatric manifestations of Langerhans cell histiocytosis: a review of the clinical and radiological findings. Clin Radiol. 58(4):269-78, 2003