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Langerhans Cell Histiocytosis, Skull and Brain
Anne G. Osborn, MD, FACR
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KEY FACTS

  • Terminology

    • Imaging

      • Top Differential Diagnoses

        • Clinical Issues

          • Diagnostic Checklist

            TERMINOLOGY

            • Synonyms

              • Langerhans cell histiocytosis (LCH)
                • Several entities (eosinophilic granuloma, Hand-Schuller-Christian disease, Letterer-Siwe disease, and "histiocytosis X" now under single designation of LCH
            • Definitions

              • LCH = dendritic cell disorder
              • LCH now best understood as neoplastic disease
                • Activating somatic BRAF V600E mutations
                  • Associated with more severe disease
              • Divided into 2 groups (based on number of lesions, systems involved)
                • Single-system LCH
                  • 70-80% of cases (usually skin, bones, or lymph nodes)
                    • Single site (e.g., 1 bone lesion)
                    • Multisite (e.g., multiple bone lesions)
                • Multisystem
                  • 20% of cases
                  • Chronic, recurring
                  • Multiple bones, reticuloendothelial system, pituitary/hypothalamus

            IMAGING

            • General Features

              • Radiographic Findings

                • 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

                                          • Consider

                                            • Image Interpretation Pearls

                                              Selected References

                                              1. Durham BH: Molecular characterization of the histiocytoses: neoplasia of dendritic cells and macrophages. Semin Cell Dev Biol. 86:62-76, 2019
                                              2. Héritier S et al: Progress towards molecular-based management of childhood Langerhans cell histiocytosis. Arch Pediatr. 26(5):301-7, 2019
                                              3. Leung AKC et al: Childhood Langerhans cell histiocytosis: a disease with many faces. World J Pediatr. 15(6):536-45, 2019
                                              4. Pan Z et al: Histiocytic and dendritic cell neoplasms. Surg Pathol Clin. 12(3):805-29, 2019
                                              5. Papo M et al: Systemic histiocytosis (langerhans cell histiocytosis, Erdheim-Chester disease, Destombes-Rosai-Dorfman disease): from oncogenic mutations to inflammatory disorders. Curr Oncol Rep. 21(7):62, 2019
                                              6. Park H et al: Imaging of histiocytosis in the era of genomic medicine. Radiographics. 39(1):95-114, 2019
                                              7. Thacker NH et al: Pediatric Langerhans cell histiocytosis: state of the science and future directions. Clin Adv Hematol Oncol. 17(2):122-31, 2019
                                              8. Wang Y et al: Neuroimaging features of CNS histiocytosis syndromes. Clin Imaging. 60(1):131-40, 2019
                                              9. Kim JH et al: Magnetic resonance imaging features in solitary cerebral Langerhans cell histiocytosis: case report and review of literature. World Neurosurg. 116:333-6, 2018
                                              10. Kobayashi M et al: Langerhans cell histiocytosis in adults: advances in pathophysiology and treatment. Cancer Sci. 109(12):3707-13, 2018
                                              11. Krooks J et al: Langerhans cell histiocytosis in children: diagnosis, differential diagnosis, treatment, sequelae, and standardized follow-up. J Am Acad Dermatol. 78(6):1047-56, 2018
                                              12. Tran G et al: Langerhans cell histiocytosis: a neoplastic disorder driven by Ras-ERK pathway mutations. J Am Acad Dermatol. 78(3):579-90.e4, 2018
                                              13. Zaveri J et al: More than just Langerhans cell histiocytosis: a radiologic review of histiocytic disorders. Radiographics. 34(7):2008-24, 2014
                                              14. Badalian-Very G et al: Pathogenesis of Langerhans cell histiocytosis. Annu Rev Pathol. 8:1-20, 2013
                                              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

                                                      • Diagnostic Checklist

                                                        TERMINOLOGY

                                                        • Synonyms

                                                          • Langerhans cell histiocytosis (LCH)
                                                            • Several entities (eosinophilic granuloma, Hand-Schuller-Christian disease, Letterer-Siwe disease, and "histiocytosis X" now under single designation of LCH
                                                        • Definitions

                                                          • LCH = dendritic cell disorder
                                                          • LCH now best understood as neoplastic disease
                                                            • Activating somatic BRAF V600E mutations
                                                              • Associated with more severe disease
                                                          • Divided into 2 groups (based on number of lesions, systems involved)
                                                            • Single-system LCH
                                                              • 70-80% of cases (usually skin, bones, or lymph nodes)
                                                                • Single site (e.g., 1 bone lesion)
                                                                • Multisite (e.g., multiple bone lesions)
                                                            • Multisystem
                                                              • 20% of cases
                                                              • Chronic, recurring
                                                              • Multiple bones, reticuloendothelial system, pituitary/hypothalamus

                                                        IMAGING

                                                        • General Features

                                                          • Radiographic Findings

                                                            • 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

                                                                                      • Consider

                                                                                        • Image Interpretation Pearls

                                                                                          Selected References

                                                                                          1. Durham BH: Molecular characterization of the histiocytoses: neoplasia of dendritic cells and macrophages. Semin Cell Dev Biol. 86:62-76, 2019
                                                                                          2. Héritier S et al: Progress towards molecular-based management of childhood Langerhans cell histiocytosis. Arch Pediatr. 26(5):301-7, 2019
                                                                                          3. Leung AKC et al: Childhood Langerhans cell histiocytosis: a disease with many faces. World J Pediatr. 15(6):536-45, 2019
                                                                                          4. Pan Z et al: Histiocytic and dendritic cell neoplasms. Surg Pathol Clin. 12(3):805-29, 2019
                                                                                          5. Papo M et al: Systemic histiocytosis (langerhans cell histiocytosis, Erdheim-Chester disease, Destombes-Rosai-Dorfman disease): from oncogenic mutations to inflammatory disorders. Curr Oncol Rep. 21(7):62, 2019
                                                                                          6. Park H et al: Imaging of histiocytosis in the era of genomic medicine. Radiographics. 39(1):95-114, 2019
                                                                                          7. Thacker NH et al: Pediatric Langerhans cell histiocytosis: state of the science and future directions. Clin Adv Hematol Oncol. 17(2):122-31, 2019
                                                                                          8. Wang Y et al: Neuroimaging features of CNS histiocytosis syndromes. Clin Imaging. 60(1):131-40, 2019
                                                                                          9. Kim JH et al: Magnetic resonance imaging features in solitary cerebral Langerhans cell histiocytosis: case report and review of literature. World Neurosurg. 116:333-6, 2018
                                                                                          10. Kobayashi M et al: Langerhans cell histiocytosis in adults: advances in pathophysiology and treatment. Cancer Sci. 109(12):3707-13, 2018
                                                                                          11. Krooks J et al: Langerhans cell histiocytosis in children: diagnosis, differential diagnosis, treatment, sequelae, and standardized follow-up. J Am Acad Dermatol. 78(6):1047-56, 2018
                                                                                          12. Tran G et al: Langerhans cell histiocytosis: a neoplastic disorder driven by Ras-ERK pathway mutations. J Am Acad Dermatol. 78(3):579-90.e4, 2018
                                                                                          13. Zaveri J et al: More than just Langerhans cell histiocytosis: a radiologic review of histiocytic disorders. Radiographics. 34(7):2008-24, 2014
                                                                                          14. Badalian-Very G et al: Pathogenesis of Langerhans cell histiocytosis. Annu Rev Pathol. 8:1-20, 2013