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Erdheim-Chester Disease
Miral D. Jhaveri, MD, MBA
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KEY FACTS

  • Terminology

    • Imaging

      • Top Differential Diagnoses

        • Pathology

          • Clinical Issues

            • Diagnostic Checklist

              TERMINOLOGY

              • Abbreviations

                • Erdheim-Chester disease (ECD)
              • Definitions

                • ECD is rare, non-Langerhans cell histiocytosis (non-LCH) that affects multiple organs characterized by xanthomatous infiltrates of foamy histiocytes

              IMAGING

              • General Features

                • CT Findings

                  • MR 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. Fernández-Eulate G et al: Vemurafenib as first-line therapy in BRAF-V600E-mutant Erdheim-Chester disease with CNS involvement. BMJ Case Rep. 12(11), 2019
                                        2. Wang F et al: Multisystemic imaging findings in Chinese patients with Erdheim-Chester disease. AJR Am J Roentgenol. 213(6):1179-86, 2019
                                        3. Wang Y et al: Neuroimaging features of CNS histiocytosis syndromes. Clin Imaging. 60(1):131-40, 2019
                                        4. Parks NE et al: Neuroradiologic manifestations of Erdheim-Chester disease. Neurol Clin Pract. 8(1):15-20, 2018
                                        5. Wagner KM et al: Intracranial Erdheim-Chester disease mimicking parafalcine meningioma: report of two cases and review of the literature. World Neurosurg. 110:365-70, 2018
                                        6. Young JR et al: 18F-FDG PET/CT in Erdheim-Chester disease: imaging findings and potential BRAF mutation biomarker. J Nucl Med. 59(5):774-79, 2018
                                        7. Pan Z et al: CNS Erdheim-Chester disease: a challenge to diagnose. J Neuropathol Exp Neurol. 76(12):986-96, 2017
                                        8. Cives M et al: Erdheim-Chester disease: a systematic review. Crit Rev Oncol Hematol. 95(1):1-11, 2015
                                        9. Gabbay LB et al: Histiocytosis: a review focusing on neuroimaging findings. Arq Neuropsiquiatr. 72(7):548-58, 2014
                                        10. Munoz J et al: Erdheim-Chester disease: characteristics and management. Mayo Clin Proc. 89(7):985-96, 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. Lachenal F et al: Neurological manifestations and neuroradiological presentation of Erdheim-Chester disease: report of 6 cases and systematic review of the literature. J Neurol. 253(10):1267-77, 2006
                                        Related Anatomy
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                                        Related Differential Diagnoses
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                                        References
                                        Tables

                                        Tables

                                        KEY FACTS

                                        • Terminology

                                          • Imaging

                                            • Top Differential Diagnoses

                                              • Pathology

                                                • Clinical Issues

                                                  • Diagnostic Checklist

                                                    TERMINOLOGY

                                                    • Abbreviations

                                                      • Erdheim-Chester disease (ECD)
                                                    • Definitions

                                                      • ECD is rare, non-Langerhans cell histiocytosis (non-LCH) that affects multiple organs characterized by xanthomatous infiltrates of foamy histiocytes

                                                    IMAGING

                                                    • General Features

                                                      • CT Findings

                                                        • MR 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. Fernández-Eulate G et al: Vemurafenib as first-line therapy in BRAF-V600E-mutant Erdheim-Chester disease with CNS involvement. BMJ Case Rep. 12(11), 2019
                                                                              2. Wang F et al: Multisystemic imaging findings in Chinese patients with Erdheim-Chester disease. AJR Am J Roentgenol. 213(6):1179-86, 2019
                                                                              3. Wang Y et al: Neuroimaging features of CNS histiocytosis syndromes. Clin Imaging. 60(1):131-40, 2019
                                                                              4. Parks NE et al: Neuroradiologic manifestations of Erdheim-Chester disease. Neurol Clin Pract. 8(1):15-20, 2018
                                                                              5. Wagner KM et al: Intracranial Erdheim-Chester disease mimicking parafalcine meningioma: report of two cases and review of the literature. World Neurosurg. 110:365-70, 2018
                                                                              6. Young JR et al: 18F-FDG PET/CT in Erdheim-Chester disease: imaging findings and potential BRAF mutation biomarker. J Nucl Med. 59(5):774-79, 2018
                                                                              7. Pan Z et al: CNS Erdheim-Chester disease: a challenge to diagnose. J Neuropathol Exp Neurol. 76(12):986-96, 2017
                                                                              8. Cives M et al: Erdheim-Chester disease: a systematic review. Crit Rev Oncol Hematol. 95(1):1-11, 2015
                                                                              9. Gabbay LB et al: Histiocytosis: a review focusing on neuroimaging findings. Arq Neuropsiquiatr. 72(7):548-58, 2014
                                                                              10. Munoz J et al: Erdheim-Chester disease: characteristics and management. Mayo Clin Proc. 89(7):985-96, 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. Lachenal F et al: Neurological manifestations and neuroradiological presentation of Erdheim-Chester disease: report of 6 cases and systematic review of the literature. J Neurol. 253(10):1267-77, 2006