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Erdheim-Chester Disease
Kirkland W. Davis, MD, FACR; B. J. Manaster, MD, PhD, FACR
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KEY FACTS

  • Terminology

    • Imaging

      • Top Differential Diagnoses

        • Pathology

          • Clinical Issues

            TERMINOLOGY

            • Abbreviations

              • Erdheim-Chester disease (ECD)
            • Synonyms

              • Lipogranulomatosis, lipoid granulomatosis, lipid (cholesterol) granulomatosis, polyostotic sclerosing histiocytosis
            • Definitions

              • Rare histiocytosis characterized by infiltration of skeleton and viscera by lipid-laden histiocytes
                • Leads to fibrosis and bone sclerosis
              • Because Langerhans cell histiocytosis is more common than all other histiocytoses combined, ECD is grouped with non-Langerhans histiocytoses

            IMAGING

            • General Features

              • Radiographic Findings

                • CT Findings

                  • MR Findings

                    • Nuclear Medicine Findings

                      DIFFERENTIAL DIAGNOSIS

                        PATHOLOGY

                        • Gross Pathologic & Surgical Features

                          • Microscopic Features

                            • Genetics

                              CLINICAL ISSUES

                              • Presentation

                                • Demographics

                                  • Natural History & Prognosis

                                    • Treatment

                                      DIAGNOSTIC CHECKLIST

                                      • Consider

                                        Selected References

                                        1. Choraria A et al: Musculoskeletal imaging features of non-Langerhans cell histiocytoses. Skeletal Radiol. 50(10):1921-40, 2021
                                        2. Chung AD et al: Primary and secondary diseases of the perinephric space: an approach to imaging diagnosis with emphasis on MRI. Clin Radiol. 76(1):75.e13-26, 2021
                                        3. Garg N et al: Clinical and neuroimaging manifestations of Erdheim-Chester disease: a review. J Neuroimaging. 31(1):35-44, 2021
                                        4. Salama HA et al: Highlights of the management of adult histiocytic disorders: Langerhans cell histiocytosis, Erdheim-Chester disease, Rosai-Dorfman disease, and hemophagocytic lymphohistiocytosis. Clin Lymphoma Myeloma Leuk. 21(1):e66-75, 2020
                                        5. Kumar P et al: Imaging findings in Erdheim-Chester disease: what every radiologist needs to know. Pol J Radiol. 83:e54-62, 2018
                                        6. Triffo WJ et al: The "hairy kidney" sign. Abdom Radiol (NY). 42(3):979-80, 2017
                                        7. White TV et al: Non-sclerotic bone involvement in Erdheim-Chester: PET/CT and MRI findings in a 15-year-old boy. Pediatr Radiol. 46(9):1345-9, 2016
                                        8. Campochiaro C et al: Erdheim-Chester disease. Eur J Intern Med. 26(4):223-9, 2015
                                        9. Bindra J et al: Erdheim-Chester disease: an unusual presentation of an uncommon disease. Skeletal Radiol. 43(6):835-40, 2014
                                        10. Zaveri J et al: More than just Langerhans cell histiocytosis: a radiologic review of histiocytic disorders. Radiographics. 34(7):2008-24, 2014
                                        11. Drier A et al: Cerebral, facial, and orbital involvement in Erdheim-Chester disease: CT and MR imaging findings. Radiology. 255(2):586-94, 2010
                                        12. Arnaud L et al: 18F-fluorodeoxyglucose-positron emission tomography scanning is more useful in followup than in the initial assessment of patients with Erdheim-Chester disease. Arthritis Rheum. 60(10):3128-38, 2009
                                        13. De Filippo M et al: Erdheim-Chester disease: clinical and radiological findings. Radiol Med. 114(8):1319-29, 2009
                                        14. Tran TA et al: Erdheim-Chester disease in childhood: a challenging diagnosis and treatment. J Pediatr Hematol Oncol. 31(10):782-6, 2009
                                        15. Zanglis A et al: Erdheim-Chester disease: symmetric uptake in the (99m)Tc-MDP bone scan. Hell J Nucl Med. 11(3):164-7, 2008
                                        16. Srikulmontree T et al: Treatment of skeletal Erdheim-Chester disease with zoledronic acid: case report and proposed mechanisms of action. Rheumatol Int. 27(3):303-7, 2007
                                        17. Dion E et al: Bone involvement in Erdheim-Chester disease: imaging findings including periostitis and partial epiphyseal involvement. Radiology. 238(2):632-9, 2006
                                        18. Braiteh F et al: Successful treatment of Erdheim-Chester disease, a non-Langerhans-cell histiocytosis, with interferon-alpha. Blood. 106(9):2992-4, 2005
                                        19. De Abreu MR et al: Erdheim-Chester disease: MR imaging, anatomic, and histopathologic correlation of orbital involvement. AJNR Am J Neuroradiol. 25(4):627-30, 2004
                                        20. Ivan D et al: Erdheim-Chester disease: a unique presentation with liver involvement and vertebral osteolytic lesions. Arch Pathol Lab Med. 127(8):e337-9, 2003
                                        21. Klieger MR et al: Erdheim-Chester disease: a unique presentation with multiple osteolytic lesions of the spine and pelvis that spared the appendicular skeleton. AJR Am J Roentgenol. 178(2):429-32, 2002
                                        22. Vinh TN et al: Erdheim-Chester disease. Fletcher CDM et al: World Health Organization Classification of Tumors: Tumours of Soft tissue and Bone. Lyon: IARC Press. 347, 2002
                                        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

                                                  TERMINOLOGY

                                                  • Abbreviations

                                                    • Erdheim-Chester disease (ECD)
                                                  • Synonyms

                                                    • Lipogranulomatosis, lipoid granulomatosis, lipid (cholesterol) granulomatosis, polyostotic sclerosing histiocytosis
                                                  • Definitions

                                                    • Rare histiocytosis characterized by infiltration of skeleton and viscera by lipid-laden histiocytes
                                                      • Leads to fibrosis and bone sclerosis
                                                    • Because Langerhans cell histiocytosis is more common than all other histiocytoses combined, ECD is grouped with non-Langerhans histiocytoses

                                                  IMAGING

                                                  • General Features

                                                    • Radiographic Findings

                                                      • CT Findings

                                                        • MR Findings

                                                          • Nuclear Medicine Findings

                                                            DIFFERENTIAL DIAGNOSIS

                                                              PATHOLOGY

                                                              • Gross Pathologic & Surgical Features

                                                                • Microscopic Features

                                                                  • Genetics

                                                                    CLINICAL ISSUES

                                                                    • Presentation

                                                                      • Demographics

                                                                        • Natural History & Prognosis

                                                                          • Treatment

                                                                            DIAGNOSTIC CHECKLIST

                                                                            • Consider

                                                                              Selected References

                                                                              1. Choraria A et al: Musculoskeletal imaging features of non-Langerhans cell histiocytoses. Skeletal Radiol. 50(10):1921-40, 2021
                                                                              2. Chung AD et al: Primary and secondary diseases of the perinephric space: an approach to imaging diagnosis with emphasis on MRI. Clin Radiol. 76(1):75.e13-26, 2021
                                                                              3. Garg N et al: Clinical and neuroimaging manifestations of Erdheim-Chester disease: a review. J Neuroimaging. 31(1):35-44, 2021
                                                                              4. Salama HA et al: Highlights of the management of adult histiocytic disorders: Langerhans cell histiocytosis, Erdheim-Chester disease, Rosai-Dorfman disease, and hemophagocytic lymphohistiocytosis. Clin Lymphoma Myeloma Leuk. 21(1):e66-75, 2020
                                                                              5. Kumar P et al: Imaging findings in Erdheim-Chester disease: what every radiologist needs to know. Pol J Radiol. 83:e54-62, 2018
                                                                              6. Triffo WJ et al: The "hairy kidney" sign. Abdom Radiol (NY). 42(3):979-80, 2017
                                                                              7. White TV et al: Non-sclerotic bone involvement in Erdheim-Chester: PET/CT and MRI findings in a 15-year-old boy. Pediatr Radiol. 46(9):1345-9, 2016
                                                                              8. Campochiaro C et al: Erdheim-Chester disease. Eur J Intern Med. 26(4):223-9, 2015
                                                                              9. Bindra J et al: Erdheim-Chester disease: an unusual presentation of an uncommon disease. Skeletal Radiol. 43(6):835-40, 2014
                                                                              10. Zaveri J et al: More than just Langerhans cell histiocytosis: a radiologic review of histiocytic disorders. Radiographics. 34(7):2008-24, 2014
                                                                              11. Drier A et al: Cerebral, facial, and orbital involvement in Erdheim-Chester disease: CT and MR imaging findings. Radiology. 255(2):586-94, 2010
                                                                              12. Arnaud L et al: 18F-fluorodeoxyglucose-positron emission tomography scanning is more useful in followup than in the initial assessment of patients with Erdheim-Chester disease. Arthritis Rheum. 60(10):3128-38, 2009
                                                                              13. De Filippo M et al: Erdheim-Chester disease: clinical and radiological findings. Radiol Med. 114(8):1319-29, 2009
                                                                              14. Tran TA et al: Erdheim-Chester disease in childhood: a challenging diagnosis and treatment. J Pediatr Hematol Oncol. 31(10):782-6, 2009
                                                                              15. Zanglis A et al: Erdheim-Chester disease: symmetric uptake in the (99m)Tc-MDP bone scan. Hell J Nucl Med. 11(3):164-7, 2008
                                                                              16. Srikulmontree T et al: Treatment of skeletal Erdheim-Chester disease with zoledronic acid: case report and proposed mechanisms of action. Rheumatol Int. 27(3):303-7, 2007
                                                                              17. Dion E et al: Bone involvement in Erdheim-Chester disease: imaging findings including periostitis and partial epiphyseal involvement. Radiology. 238(2):632-9, 2006
                                                                              18. Braiteh F et al: Successful treatment of Erdheim-Chester disease, a non-Langerhans-cell histiocytosis, with interferon-alpha. Blood. 106(9):2992-4, 2005
                                                                              19. De Abreu MR et al: Erdheim-Chester disease: MR imaging, anatomic, and histopathologic correlation of orbital involvement. AJNR Am J Neuroradiol. 25(4):627-30, 2004
                                                                              20. Ivan D et al: Erdheim-Chester disease: a unique presentation with liver involvement and vertebral osteolytic lesions. Arch Pathol Lab Med. 127(8):e337-9, 2003
                                                                              21. Klieger MR et al: Erdheim-Chester disease: a unique presentation with multiple osteolytic lesions of the spine and pelvis that spared the appendicular skeleton. AJR Am J Roentgenol. 178(2):429-32, 2002
                                                                              22. Vinh TN et al: Erdheim-Chester disease. Fletcher CDM et al: World Health Organization Classification of Tumors: Tumours of Soft tissue and Bone. Lyon: IARC Press. 347, 2002