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Miscellaneous Histiocytoses
Miral D. Jhaveri, MD, MBA
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KEY FACTS

  • Terminology

    • Imaging

      • Top Differential Diagnoses

        • Pathology

          • Clinical Issues

            TERMINOLOGY

            • Abbreviations

              • Non-Langerhans cell histiocytoses (non-LCH)
              • Juvenile xanthogranuloma (JXG)
              • Hemophagocytic lymphohistiocytosis (HLH)
              • Histiocytic sarcoma (HS)
            • Definitions

              • JXG: Histiocytic disorder involving brain or meninges ± cutaneous lesions
              • HLH: Rare multisystem disorder characterized by proliferation and diffuse infiltration of multiple organs with histiocytes
              • HS: Rare, aggressive, malignant neoplasm with histologic and immunophenotypic characteristics of mature histiocytes

            IMAGING

            • General Features

              • CT Findings

                • MR Findings

                  • Imaging Recommendations

                    DIFFERENTIAL DIAGNOSIS

                      PATHOLOGY

                      • General Features

                        CLINICAL ISSUES

                        • Presentation

                          • Demographics

                            • Natural History & Prognosis

                              Selected References

                              1. Chauhan S et al: Cytological diagnosis of juvenile xanthogranuloma: a rare histiocytic disorder. Diagn Cytopathol. 48(1):66-70, 2020
                              2. Collie JS et al: Juvenile xanthogranuloma (nevoxanthoendothelioma, JXG). StatPearls [Internet]. Treasure Island (FL): StatPearls Publishing, 2020
                              3. Benson LA et al: Pediatric CNS-isolated hemophagocytic lymphohistiocytosis. Neurol Neuroimmunol Neuroinflamm. 6(3):e560, 2019
                              4. Cichewicz A et al: Congenital multiple juvenile xanthogranuloma. Postepy Dermatol Alergol. 36(3):365-8, 2019
                              5. Hota D et al: Extensive juvenile xanthogranuloma. Indian Pediatr. 55(11):1013, 2018
                              6. May JM et al: Primary histiocytic sarcoma of the central nervous system: a case report with platelet derived growth factor receptor mutation and PD-L1/PD-L2 expression and literature review. Radiat Oncol. 13(1):167, 2018
                              7. Meyer M et al: Systemic juvenile xanthogranuloma: a case report and brief review. Clin Exp Dermatol. 43(5):642-4, 2018
                              8. Samuelov L et al: Risk of intraocular and other extracutaneous involvement in patients with cutaneous juvenile xanthogranuloma. Pediatr Dermatol. 35(3):329-35, 2018
                              9. Ginat DT et al: Imaging features of juvenile xanthogranuloma of the pediatric head and neck. AJNR Am J Neuroradiol. 37(5):910-6, 2016
                              10. Gabbay LB et al: Histiocytosis: a review focusing on neuroimaging findings. Arq Neuropsiquiatr. 72(7):548-58, 2014
                              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)
                                          • Juvenile xanthogranuloma (JXG)
                                          • Hemophagocytic lymphohistiocytosis (HLH)
                                          • Histiocytic sarcoma (HS)
                                        • Definitions

                                          • JXG: Histiocytic disorder involving brain or meninges ± cutaneous lesions
                                          • HLH: Rare multisystem disorder characterized by proliferation and diffuse infiltration of multiple organs with histiocytes
                                          • HS: Rare, aggressive, malignant neoplasm with histologic and immunophenotypic characteristics of mature histiocytes

                                        IMAGING

                                        • General Features

                                          • CT Findings

                                            • MR Findings

                                              • Imaging Recommendations

                                                DIFFERENTIAL DIAGNOSIS

                                                  PATHOLOGY

                                                  • General Features

                                                    CLINICAL ISSUES

                                                    • Presentation

                                                      • Demographics

                                                        • Natural History & Prognosis

                                                          Selected References

                                                          1. Chauhan S et al: Cytological diagnosis of juvenile xanthogranuloma: a rare histiocytic disorder. Diagn Cytopathol. 48(1):66-70, 2020
                                                          2. Collie JS et al: Juvenile xanthogranuloma (nevoxanthoendothelioma, JXG). StatPearls [Internet]. Treasure Island (FL): StatPearls Publishing, 2020
                                                          3. Benson LA et al: Pediatric CNS-isolated hemophagocytic lymphohistiocytosis. Neurol Neuroimmunol Neuroinflamm. 6(3):e560, 2019
                                                          4. Cichewicz A et al: Congenital multiple juvenile xanthogranuloma. Postepy Dermatol Alergol. 36(3):365-8, 2019
                                                          5. Hota D et al: Extensive juvenile xanthogranuloma. Indian Pediatr. 55(11):1013, 2018
                                                          6. May JM et al: Primary histiocytic sarcoma of the central nervous system: a case report with platelet derived growth factor receptor mutation and PD-L1/PD-L2 expression and literature review. Radiat Oncol. 13(1):167, 2018
                                                          7. Meyer M et al: Systemic juvenile xanthogranuloma: a case report and brief review. Clin Exp Dermatol. 43(5):642-4, 2018
                                                          8. Samuelov L et al: Risk of intraocular and other extracutaneous involvement in patients with cutaneous juvenile xanthogranuloma. Pediatr Dermatol. 35(3):329-35, 2018
                                                          9. Ginat DT et al: Imaging features of juvenile xanthogranuloma of the pediatric head and neck. AJNR Am J Neuroradiol. 37(5):910-6, 2016
                                                          10. Gabbay LB et al: Histiocytosis: a review focusing on neuroimaging findings. Arq Neuropsiquiatr. 72(7):548-58, 2014