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

  • Terminology

    • Imaging

      • Clinical Issues

        • Diagnostic Checklist

          TERMINOLOGY

          • Abbreviations

            • Langerhans cell histiocytosis (LCH)
          • Synonyms

            • Not recommended: eosinophilic granuloma, Langerhans cell granulomatosis, histiocytosis X, Hand-Schüller-Christian disease, Letterer-Siwe disease
          • Definitions

            • Part of spectrum of histiocytic disorders: accumulation of macrophages, dendritic cells, or monocyte-derived cells in tissues
              • > 100 subtypes with wide range of clinical behavior
                • Including Rosai-Dorfman disease, Erdheim-Chester disease, adult xanthogranuloma, benign cephalic histiocytosis, generalized eruptive histiocytosis, hemophagocytic lymphohistiocytosis, indeterminate cell histiocytosis, juvenile xanthogranuloma, multicentric reticulohistiocytosis, necrobiotic xanthogranuloma, progressive nodular histiocytosis, solitary reticulohistiocytoma, xanthoma disseminatum, and xanthogranuloma
            • LCH: clonal neoplastic proliferation of myeloid dendritic cells with Langerhans cell phenotype
              • Single system: unifocal or multifocal
                • Predominantly skeletal
                • Less commonly lymph node, skin, and lung
              • Multisystem
                • Skin, bone, liver, spleen, and bone marrow preferentially involved

          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. Ferrell J et al: Discrepancies between F-18-FDG PET/CT findings and conventional imaging in Langerhans cell histiocytosis. Pediatr Blood Cancer. 68(4):e28891, 2021
                                          2. Rajakulasingam R et al: Skeletal staging in Langerhans cell histiocytosis: a multimodality imaging review. Skeletal Radiol. 50(6):1081-93, 2021
                                          3. Tang X et al: Clinical and prognostic characteristics of 95 cases of Langerhans cell histiocytosis in children: a single-institute experience from 2013 to 2020. Ann Med. 53(1):1537-46, 2021
                                          4. Pileri SA et al: Langerhans cell histiocytosis. In Fletcher CDM et al: World Health Organization Classification of Tumours: Tumours of Soft Tissue and Bone. IARC Press, 2020
                                          5. Goyal G et al: The Mayo Clinic Histiocytosis Working Group consensus statement for the diagnosis and evaluation of adult patients with histiocytic neoplasms: Erdheim-Chester disease, Langerhans cell histiocytosis, and Rosai-Dorfman disease. Mayo Clin Proc. 94(10):2054-71, 2019
                                          6. Emile JF et al: Revised classification of histiocytoses and neoplasms of the macrophage-dendritic cell lineages. Blood. 127(22):2672-81, 2016
                                          7. 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
                                          8. Khung S et al: Skeletal involvement in Langerhans cell histiocytosis. Insights Imaging. 4(5):569-79, 2013
                                          9. 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
                                          10. 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
                                          11. Diederichs G et al: Case 147: langerhans cell histiocytosis of the femur. Radiology. 252(1):309-13, 2009
                                          12. Muramatsu K et al: Langerhans cell histiocytosis of the acetabulum in adults. Orthopedics. 32(4), 2009
                                          13. 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
                                          14. 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
                                          15. Denaro L et al: Eosinophilic granuloma of the pediatric cervical spine. Spine (Phila Pa 1976). 33(24):E936-41, 2008
                                          16. Gasent Blesa JM et al: Langerhans cell histiocytosis. Clin Transl Oncol. 10(11):688-96, 2008
                                          17. Schmidt S et al: Extraosseous langerhans cell histiocytosis in children. Radiographics. 28(3):707-26; quiz 910-1, 2008
                                          18. Hoover KB et al: Langerhans cell histiocytosis. Skeletal Radiol. 36(2):95-104, 2007
                                          19. Azouz EM et al: Langerhans' cell histiocytosis: pathology, imaging and treatment of skeletal involvement. Pediatr Radiol. 35(2):103-15, 2005
                                          20. 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
                                          21. Stull MA et al: Langerhans cell histiocytosis of bone. Radiographics. 12(4):801-23, 1992
                                          22. 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

                                                • Diagnostic Checklist

                                                  TERMINOLOGY

                                                  • Abbreviations

                                                    • Langerhans cell histiocytosis (LCH)
                                                  • Synonyms

                                                    • Not recommended: eosinophilic granuloma, Langerhans cell granulomatosis, histiocytosis X, Hand-Schüller-Christian disease, Letterer-Siwe disease
                                                  • Definitions

                                                    • Part of spectrum of histiocytic disorders: accumulation of macrophages, dendritic cells, or monocyte-derived cells in tissues
                                                      • > 100 subtypes with wide range of clinical behavior
                                                        • Including Rosai-Dorfman disease, Erdheim-Chester disease, adult xanthogranuloma, benign cephalic histiocytosis, generalized eruptive histiocytosis, hemophagocytic lymphohistiocytosis, indeterminate cell histiocytosis, juvenile xanthogranuloma, multicentric reticulohistiocytosis, necrobiotic xanthogranuloma, progressive nodular histiocytosis, solitary reticulohistiocytoma, xanthoma disseminatum, and xanthogranuloma
                                                    • LCH: clonal neoplastic proliferation of myeloid dendritic cells with Langerhans cell phenotype
                                                      • Single system: unifocal or multifocal
                                                        • Predominantly skeletal
                                                        • Less commonly lymph node, skin, and lung
                                                      • Multisystem
                                                        • Skin, bone, liver, spleen, and bone marrow preferentially involved

                                                  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. Ferrell J et al: Discrepancies between F-18-FDG PET/CT findings and conventional imaging in Langerhans cell histiocytosis. Pediatr Blood Cancer. 68(4):e28891, 2021
                                                                                  2. Rajakulasingam R et al: Skeletal staging in Langerhans cell histiocytosis: a multimodality imaging review. Skeletal Radiol. 50(6):1081-93, 2021
                                                                                  3. Tang X et al: Clinical and prognostic characteristics of 95 cases of Langerhans cell histiocytosis in children: a single-institute experience from 2013 to 2020. Ann Med. 53(1):1537-46, 2021
                                                                                  4. Pileri SA et al: Langerhans cell histiocytosis. In Fletcher CDM et al: World Health Organization Classification of Tumours: Tumours of Soft Tissue and Bone. IARC Press, 2020
                                                                                  5. Goyal G et al: The Mayo Clinic Histiocytosis Working Group consensus statement for the diagnosis and evaluation of adult patients with histiocytic neoplasms: Erdheim-Chester disease, Langerhans cell histiocytosis, and Rosai-Dorfman disease. Mayo Clin Proc. 94(10):2054-71, 2019
                                                                                  6. Emile JF et al: Revised classification of histiocytoses and neoplasms of the macrophage-dendritic cell lineages. Blood. 127(22):2672-81, 2016
                                                                                  7. 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
                                                                                  8. Khung S et al: Skeletal involvement in Langerhans cell histiocytosis. Insights Imaging. 4(5):569-79, 2013
                                                                                  9. 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
                                                                                  10. 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
                                                                                  11. Diederichs G et al: Case 147: langerhans cell histiocytosis of the femur. Radiology. 252(1):309-13, 2009
                                                                                  12. Muramatsu K et al: Langerhans cell histiocytosis of the acetabulum in adults. Orthopedics. 32(4), 2009
                                                                                  13. 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
                                                                                  14. 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
                                                                                  15. Denaro L et al: Eosinophilic granuloma of the pediatric cervical spine. Spine (Phila Pa 1976). 33(24):E936-41, 2008
                                                                                  16. Gasent Blesa JM et al: Langerhans cell histiocytosis. Clin Transl Oncol. 10(11):688-96, 2008
                                                                                  17. Schmidt S et al: Extraosseous langerhans cell histiocytosis in children. Radiographics. 28(3):707-26; quiz 910-1, 2008
                                                                                  18. Hoover KB et al: Langerhans cell histiocytosis. Skeletal Radiol. 36(2):95-104, 2007
                                                                                  19. Azouz EM et al: Langerhans' cell histiocytosis: pathology, imaging and treatment of skeletal involvement. Pediatr Radiol. 35(2):103-15, 2005
                                                                                  20. 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
                                                                                  21. Stull MA et al: Langerhans cell histiocytosis of bone. Radiographics. 12(4):801-23, 1992
                                                                                  22. Sartoris DJ et al: Histiocytosis X: rate and pattern of resolution of osseous lesions. Radiology. 152(3):679-84, 1984