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Miscellaneous Histiocytoses
Anne G. Osborn, MD, FACR; Karen L. Salzman, MD
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KEY FACTS

  • Terminology

    • Imaging

      • Top Differential Diagnoses

        • Pathology

          • Clinical Issues

            TERMINOLOGY

            • Abbreviations

              • Non-Langerhans cell histiocytoses (non-LCH)
            • Definitions

              • Non-Langerhans cell histiocytoses
                • 4 major disorders
                  • Rosai-Dorfman disease (RDD)
                  • Erdheim-Chester disease (ECD)
                  • Hemophagocytic lymphohistiocytosis (HLH)
                  • Juvenile xanthogranuloma (JXG)
              • RDD: Rare, benign, idiopathic histioproliferative disorder generally (but not always) restricted to lymph nodes
              • ECD: Rare, non-Langerhans form of histiocytosis of unknown etiology that affects multiple organs
              • HLH: Syndrome of pathologic activation of macrophages, T cells with "hemophagocytosis" (engulfment of RBCs and leukocytes)
                • May affect multiple organ systems, including central nervous system (CNS)
              • "Malignant histiocytosis" now considered histiocytic sarcoma, not true histiocytic lesion

            IMAGING

            • General Features

              • CT Findings

                • MR Findings

                  • Imaging Recommendations

                    DIFFERENTIAL DIAGNOSIS

                      PATHOLOGY

                      • General Features

                        • Staging, Grading, & Classification

                          CLINICAL ISSUES

                          • Presentation

                            • Demographics

                              • Natural History & Prognosis

                                • Treatment

                                  DIAGNOSTIC CHECKLIST

                                  • Consider

                                    Selected References

                                    1. Cives M et al: Erdheim-Chester disease: A systematic review. Crit Rev Oncol Hematol. ePub, 2015
                                    2. Gabbay LB et al: Histiocytosis: a review focusing on neuroimaging findings. Arq Neuropsiquiatr. 72(7):548-58, 2014
                                    3. Munoz J et al: Erdheim-Chester disease: characteristics and management. Mayo Clin Proc. 89(7):985-96, 2014
                                    4. Zaveri J et al: More than just Langerhans cell histiocytosis: a radiologic review of histiocytic disorders. Radiographics. 34(7):2008-24, 2014
                                    5. Hinduja A et al: Rosai-Dorfman disease manifesting as intracranial and intraorbital lesion. J Neurooncol. 92(1):117-20, 2009
                                    6. D'Ambrosio N et al: Craniofacial and intracranial manifestations of langerhans cell histiocytosis: report of findings in 100 patients. AJR Am J Roentgenol. 191(2):589-97, 2008
                                    7. La Barge DV 3rd et al: Sinus histiocytosis with massive lymphadenopathy (Rosai-Dorfman disease): imaging manifestations in the head and neck. AJR Am J Roentgenol. 191(6):W299-306, 2008
                                    8. Goo HW et al: A spectrum of neuroradiological findings in children with haemophagocytic lymphohistiocytosis. Pediatr Radiol. 37(11):1110-7, 2007
                                    9. 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
                                    10. Purav P et al: Rosai-Dorfman disease of the central nervous system. J Clin Neurosci. 12(6):656-9, 2005
                                    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

                                                • Non-Langerhans cell histiocytoses (non-LCH)
                                              • Definitions

                                                • Non-Langerhans cell histiocytoses
                                                  • 4 major disorders
                                                    • Rosai-Dorfman disease (RDD)
                                                    • Erdheim-Chester disease (ECD)
                                                    • Hemophagocytic lymphohistiocytosis (HLH)
                                                    • Juvenile xanthogranuloma (JXG)
                                                • RDD: Rare, benign, idiopathic histioproliferative disorder generally (but not always) restricted to lymph nodes
                                                • ECD: Rare, non-Langerhans form of histiocytosis of unknown etiology that affects multiple organs
                                                • HLH: Syndrome of pathologic activation of macrophages, T cells with "hemophagocytosis" (engulfment of RBCs and leukocytes)
                                                  • May affect multiple organ systems, including central nervous system (CNS)
                                                • "Malignant histiocytosis" now considered histiocytic sarcoma, not true histiocytic lesion

                                              IMAGING

                                              • General Features

                                                • CT Findings

                                                  • MR Findings

                                                    • Imaging Recommendations

                                                      DIFFERENTIAL DIAGNOSIS

                                                        PATHOLOGY

                                                        • General Features

                                                          • Staging, Grading, & Classification

                                                            CLINICAL ISSUES

                                                            • Presentation

                                                              • Demographics

                                                                • Natural History & Prognosis

                                                                  • Treatment

                                                                    DIAGNOSTIC CHECKLIST

                                                                    • Consider

                                                                      Selected References

                                                                      1. Cives M et al: Erdheim-Chester disease: A systematic review. Crit Rev Oncol Hematol. ePub, 2015
                                                                      2. Gabbay LB et al: Histiocytosis: a review focusing on neuroimaging findings. Arq Neuropsiquiatr. 72(7):548-58, 2014
                                                                      3. Munoz J et al: Erdheim-Chester disease: characteristics and management. Mayo Clin Proc. 89(7):985-96, 2014
                                                                      4. Zaveri J et al: More than just Langerhans cell histiocytosis: a radiologic review of histiocytic disorders. Radiographics. 34(7):2008-24, 2014
                                                                      5. Hinduja A et al: Rosai-Dorfman disease manifesting as intracranial and intraorbital lesion. J Neurooncol. 92(1):117-20, 2009
                                                                      6. D'Ambrosio N et al: Craniofacial and intracranial manifestations of langerhans cell histiocytosis: report of findings in 100 patients. AJR Am J Roentgenol. 191(2):589-97, 2008
                                                                      7. La Barge DV 3rd et al: Sinus histiocytosis with massive lymphadenopathy (Rosai-Dorfman disease): imaging manifestations in the head and neck. AJR Am J Roentgenol. 191(6):W299-306, 2008
                                                                      8. Goo HW et al: A spectrum of neuroradiological findings in children with haemophagocytic lymphohistiocytosis. Pediatr Radiol. 37(11):1110-7, 2007
                                                                      9. 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
                                                                      10. Purav P et al: Rosai-Dorfman disease of the central nervous system. J Clin Neurosci. 12(6):656-9, 2005