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Langerhans Cell Histiocytosis
B.J. Manaster, MD, PhD, FACR
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KEY FACTS

  • Terminology

    • Imaging

      • Clinical Issues

        TERMINOLOGY

        • Abbreviations

          • Langerhans cell histiocytosis (LCH)
        • Synonyms

          • Eosinophilic granuloma (EG), Langerhans cell granulomatosis, histiocytosis X
          • Clinical variants: Hand-Schüller-Christian disease, Letterer-Siwe (LS) disease
        • Definitions

          • Neoplastic proliferation of Langerhans cells

        IMAGING

        • General Features

          • Radiographic Findings

            • CT Findings

              • MR 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

                                      • Image Interpretation Pearls

                                        Selected References

                                        1. Amini B et al: Soft tissue Langerhans cell histiocytosis with secondary bone involvement in extremities: evolution of lesions in two patients. Skeletal Radiol. 42(9):1301-9, 2013
                                        2. Song YS et al: Radiologic findings of adult pelvis and appendicular skeletal Langerhans cell histiocytosis in nine patients. Skeletal Radiol. 40(11):1421-6, 2011
                                        3. Arkader A et al: Primary musculoskeletal Langerhans cell histiocytosis in children: an analysis for a 3-decade period. J Pediatr Orthop. 29(2):201-7, 2009
                                        4. Diederichs G et al: Case 147: langerhans cell histiocytosis of the femur. Radiology. 252(1):309-13, 2009
                                        5. Muramatsu K et al: Langerhans cell histiocytosis of the acetabulum in adults. Orthopedics. 32(4), 2009
                                        6. Phillips M et al: Comparison of FDG-PET scans to conventional radiography and bone scans in management of Langerhans cell histiocytosis. Pediatr Blood Cancer. 52(1):97-101, 2009
                                        7. Ruggieri P et al: Tumours and tumour-like lesions of the hip in the paediatric age: a review of the Rizzoli experience. Hip Int. 19 Suppl 6:S35-45, 2009
                                        8. Denaro L et al: Eosinophilic granuloma of the pediatric cervical spine. Spine (Phila Pa 1976). 33(24):E936-41, 2008
                                        9. Gasent Blesa JM et al: Langerhans cell histiocytosis. Clin Transl Oncol. 10(11):688-96, 2008
                                        10. Schmidt S et al: Extraosseous langerhans cell histiocytosis in children. Radiographics. 28(3):707-26; quiz 910-1, 2008
                                        11. Hoover KB et al: Langerhans cell histiocytosis. Skeletal Radiol. 36(2):95-104, 2007
                                        12. Azouz EM et al: Langerhans' cell histiocytosis: pathology, imaging and treatment of skeletal involvement. Pediatr Radiol. 35(2):103-15, 2005
                                        13. DeYoung BR et al: Langerhans Cell Histiocytosis. In Fletcher CDM et al: World Health Organization Classification of Tumors: Tumours of Soft Tissue and Bone. Lyon: IARC Press. 345-6, 2002
                                        14. Hung PC et al: From normal to abnormal MR findings within three weeks in a solitary pelvic Langerhans histiocytosis. Skeletal Radiol. 32(8):481-4, 2003
                                        15. Stull MA et al: Langerhans cell histiocytosis of bone. Radiographics. 12(4):801-23, 1992
                                        16. Sartoris DJ et al: Histiocytosis X: rate and pattern of resolution of osseous lesions. Radiology. 152(3):679-84, 1984
                                        Related Anatomy
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                                        Related Differential Diagnoses
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                                        References
                                        Tables

                                        Tables

                                        KEY FACTS

                                        • Terminology

                                          • Imaging

                                            • Clinical Issues

                                              TERMINOLOGY

                                              • Abbreviations

                                                • Langerhans cell histiocytosis (LCH)
                                              • Synonyms

                                                • Eosinophilic granuloma (EG), Langerhans cell granulomatosis, histiocytosis X
                                                • Clinical variants: Hand-Schüller-Christian disease, Letterer-Siwe (LS) disease
                                              • Definitions

                                                • Neoplastic proliferation of Langerhans cells

                                              IMAGING

                                              • General Features

                                                • Radiographic Findings

                                                  • CT Findings

                                                    • MR 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

                                                                            • Image Interpretation Pearls

                                                                              Selected References

                                                                              1. Amini B et al: Soft tissue Langerhans cell histiocytosis with secondary bone involvement in extremities: evolution of lesions in two patients. Skeletal Radiol. 42(9):1301-9, 2013
                                                                              2. Song YS et al: Radiologic findings of adult pelvis and appendicular skeletal Langerhans cell histiocytosis in nine patients. Skeletal Radiol. 40(11):1421-6, 2011
                                                                              3. Arkader A et al: Primary musculoskeletal Langerhans cell histiocytosis in children: an analysis for a 3-decade period. J Pediatr Orthop. 29(2):201-7, 2009
                                                                              4. Diederichs G et al: Case 147: langerhans cell histiocytosis of the femur. Radiology. 252(1):309-13, 2009
                                                                              5. Muramatsu K et al: Langerhans cell histiocytosis of the acetabulum in adults. Orthopedics. 32(4), 2009
                                                                              6. Phillips M et al: Comparison of FDG-PET scans to conventional radiography and bone scans in management of Langerhans cell histiocytosis. Pediatr Blood Cancer. 52(1):97-101, 2009
                                                                              7. Ruggieri P et al: Tumours and tumour-like lesions of the hip in the paediatric age: a review of the Rizzoli experience. Hip Int. 19 Suppl 6:S35-45, 2009
                                                                              8. Denaro L et al: Eosinophilic granuloma of the pediatric cervical spine. Spine (Phila Pa 1976). 33(24):E936-41, 2008
                                                                              9. Gasent Blesa JM et al: Langerhans cell histiocytosis. Clin Transl Oncol. 10(11):688-96, 2008
                                                                              10. Schmidt S et al: Extraosseous langerhans cell histiocytosis in children. Radiographics. 28(3):707-26; quiz 910-1, 2008
                                                                              11. Hoover KB et al: Langerhans cell histiocytosis. Skeletal Radiol. 36(2):95-104, 2007
                                                                              12. Azouz EM et al: Langerhans' cell histiocytosis: pathology, imaging and treatment of skeletal involvement. Pediatr Radiol. 35(2):103-15, 2005
                                                                              13. DeYoung BR et al: Langerhans Cell Histiocytosis. In Fletcher CDM et al: World Health Organization Classification of Tumors: Tumours of Soft Tissue and Bone. Lyon: IARC Press. 345-6, 2002
                                                                              14. Hung PC et al: From normal to abnormal MR findings within three weeks in a solitary pelvic Langerhans histiocytosis. Skeletal Radiol. 32(8):481-4, 2003
                                                                              15. Stull MA et al: Langerhans cell histiocytosis of bone. Radiographics. 12(4):801-23, 1992
                                                                              16. Sartoris DJ et al: Histiocytosis X: rate and pattern of resolution of osseous lesions. Radiology. 152(3):679-84, 1984