link
Bookmarks
 (0)
Pediatrics
Diagnosis
Musculoskeletal
Focal, Multifocal, and Diffuse Bone Lesions
Langerhans Cell Histiocytosis
Langerhans Cell Histiocytosis
A. Carlson Merrow, Jr., MD, FAAP
To access 4,300 diagnoses written by the world's leading experts in radiology, please log in or subscribe.Log inSubscribe
0
32
0
12

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-positive dendritic cells
            • Single system (SS) (unifocal or multifocal) vs. multisystem (MS) disease
              • Bone (80-90%), skin (50%) most frequently involved
                • Lung most common SS site in adults
                • Skin only involvement in 2%
              • Risk organ (RO) involvement: Liver, spleen, marrow; confers worse prognosis ("high risk")
                • Lung no longer considered RO
              • Other organs: Lymph nodes, pituitary, thymus, GI tract
              • CNS-risk lesion: Skull base & many facial lesions
                • At risk for diabetes insipidus & 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. Allen CE et al: How I treat Langerhans cell histiocytosis. Blood. 126(1):26-35, 2015
                                          2. Demellawy DE et al: Langerhans cell histiocytosis: a comprehensive review. Pathology. 47(4):294-301, 2015
                                          3. Gelfand MJ et al: Selective CT for PET/CT: dose reduction in Langerhans cell histiocytosis. Pediatr Radiol. 45(1):81-5, 2015
                                          4. Harmon CM et al: Langerhans cell histiocytosis: a clinicopathologic review and molecular pathogenetic update. Arch Pathol Lab Med. 139(10):1211-4, 2015
                                          5. Picarsic J et al: Nosology and pathology of Langerhans cell histiocytosis. Hematol Oncol Clin North Am. 29(5):799-823, 2015
                                          6. Atkin KL et al: The role of whole-body MRI in pediatric oncology. J Pediatr Hematol Oncol. 36(5):342-52, 2014
                                          7. Delprat C et al: Blood spotlight on Langerhans cell histiocytosis. Blood. 124(6):867-72, 2014
                                          8. DiCaprio MR et al: Diagnosis and management of Langerhans cell histiocytosis. J Am Acad Orthop Surg. 22(10):643-52, 2014
                                          9. 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
                                          10. 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
                                          11. Zaveri J et al: More than just Langerhans cell histiocytosis: a radiologic review of histiocytic disorders. Radiographics. 34(7):2008-24, 2014
                                          12. 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
                                          13. Goo HW et al: Whole-body MRI of Langerhans cell histiocytosis: comparison with radiography and bone scintigraphy. Pediatr Radiol. 36(10):1019-31, 2006
                                          14. 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
                                          15. Schmidt S et al: Extra-osseous involvement of Langerhans' cell histiocytosis in children. Pediatr Radiol. 34(4):313-21, 2004
                                          16. 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
                                          Loading...
                                          Related Differential Diagnoses
                                          Loading...
                                          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-positive dendritic cells
                                                    • Single system (SS) (unifocal or multifocal) vs. multisystem (MS) disease
                                                      • Bone (80-90%), skin (50%) most frequently involved
                                                        • Lung most common SS site in adults
                                                        • Skin only involvement in 2%
                                                      • Risk organ (RO) involvement: Liver, spleen, marrow; confers worse prognosis ("high risk")
                                                        • Lung no longer considered RO
                                                      • Other organs: Lymph nodes, pituitary, thymus, GI tract
                                                      • CNS-risk lesion: Skull base & many facial lesions
                                                        • At risk for diabetes insipidus & 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. Allen CE et al: How I treat Langerhans cell histiocytosis. Blood. 126(1):26-35, 2015
                                                                                  2. Demellawy DE et al: Langerhans cell histiocytosis: a comprehensive review. Pathology. 47(4):294-301, 2015
                                                                                  3. Gelfand MJ et al: Selective CT for PET/CT: dose reduction in Langerhans cell histiocytosis. Pediatr Radiol. 45(1):81-5, 2015
                                                                                  4. Harmon CM et al: Langerhans cell histiocytosis: a clinicopathologic review and molecular pathogenetic update. Arch Pathol Lab Med. 139(10):1211-4, 2015
                                                                                  5. Picarsic J et al: Nosology and pathology of Langerhans cell histiocytosis. Hematol Oncol Clin North Am. 29(5):799-823, 2015
                                                                                  6. Atkin KL et al: The role of whole-body MRI in pediatric oncology. J Pediatr Hematol Oncol. 36(5):342-52, 2014
                                                                                  7. Delprat C et al: Blood spotlight on Langerhans cell histiocytosis. Blood. 124(6):867-72, 2014
                                                                                  8. DiCaprio MR et al: Diagnosis and management of Langerhans cell histiocytosis. J Am Acad Orthop Surg. 22(10):643-52, 2014
                                                                                  9. 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
                                                                                  10. 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
                                                                                  11. Zaveri J et al: More than just Langerhans cell histiocytosis: a radiologic review of histiocytic disorders. Radiographics. 34(7):2008-24, 2014
                                                                                  12. 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
                                                                                  13. Goo HW et al: Whole-body MRI of Langerhans cell histiocytosis: comparison with radiography and bone scintigraphy. Pediatr Radiol. 36(10):1019-31, 2006
                                                                                  14. 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
                                                                                  15. Schmidt S et al: Extra-osseous involvement of Langerhans' cell histiocytosis in children. Pediatr Radiol. 34(4):313-21, 2004
                                                                                  16. 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