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Langerhans Cell Histiocytosis, Skull and Brain
Luke L. Linscott, MD; Anne G. Osborn, MD, FACR; Gary L. Hedlund, DO
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KEY FACTS

  • Terminology

    • Imaging

      • Top Differential Diagnoses

        • Diagnostic Checklist

          TERMINOLOGY

          • Synonyms

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

            • LCH now best understood as neoplastic disease
              • Activating somatic BRAF mutations, LCH cell clonality
              • Inflammatory myeloid neoplasia
            • Divided into 3 groups (based on number of lesions, systems involved)
              • Unifocal localized form (a.k.a. eosinophilic granuloma)
                • 70% of cases
                • Limited to single or a few bones, may involve lung
              • Multifocal unisystem
                • 20% of cases
                • Chronic, recurring
                • Multiple bones, reticuloendothelial system, pituitary/hypothalamus
              • Multifocal, multisystem
                • 10% of cases
                • Fulminant (often fatal)

          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. 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
                                            2. Zheng H et al: Pediatric Langerhans cell histiocytosis of the temporal bone: clinical and imaging studies of 27 cases. World J Surg Oncol. 16(1):72, 2018
                                            3. Chevallier KM et al: Differentiating pediatric rhabdomyosarcoma and Langerhans cell histiocytosis of the temporal bone by imaging appearance. AJNR Am J Neuroradiol. 37(6):1185-9, 2016
                                            4. Berres ML et al: Progress in understanding the pathogenesis of Langerhans cell histiocytosis: back to Histiocytosis X? Br J Haematol. 169(1):3-13, 2015
                                            5. De La Hoz Polo M et al: Neuroimaging of Langerhans cell histiocytosis in the central nervous system of children. Radiologia. 57(2):123-130, 2015
                                            6. Demellawy DE et al: Langerhans cell histiocytosis: a comprehensive review. Pathology. 47(4):294-301, 2015
                                            7. Esmaili N et al: Langerhans cll histiocytosis of the orbit: spectrum of disease and risk of central nervous system sequelae in unifocal cases. Ophthal Plast Reconstr Surg. ePub, 2015
                                            8. Modest MC et al: Langerhans cell histiocytosis of the temporal bone: A review of 29 cases at a single center. Laryngoscope. ePub, 2015
                                            9. Porto L et al: Central nervous system imaging in childhood Langerhans cell histiocytosis - a reference center analysis. Radiol Oncol. 49(3):242-9, 2015
                                            10. Nabavizadeh SA et al: CT and MRI of Pediatric Skull Lesions with Fluid-Fluid Levels. AJNR Am J Neuroradiol. 35(3):604-8, 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. Badalian-Very G et al: Pathogenesis of Langerhans cell histiocytosis. Annu Rev Pathol. 8:1-20, 2013
                                            13. Laurencikas E et al: Incidence and pattern of radiological central nervous system Langerhans cell histiocytosis in children: a population based study. Pediatr Blood Cancer. 56(2):250-7, 2011
                                            14. Grois N et al: Central nervous system disease in Langerhans cell histiocytosis. J Pediatr. 156(6):873-81, 881, 2010
                                            15. 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
                                            16. Demaerel P et al: Paediatric neuroradiological aspects of Langerhans cell histiocytosis. Neuroradiology. 50(1):85-92, 2008
                                            17. Saliba I et al: Langerhans' cell histiocytosis of the temporal bone in children. Int J Pediatr Otorhinolaryngol. 72(6):775-86, 2008
                                            18. van der Knaap MS et al: Cerebellar leukoencephalopathy: most likely histiocytosis-related. Neurology. 71(17):1361-7, 2008
                                            19. Varan A et al: Radiological evaluation of patients with pituitary langerhans cell histiocytosis at diagnosis and at follow-up. Pediatr Hematol Oncol. 25(6):567-74, 2008
                                            20. Kaste SC et al: PET-CT in pediatric Langerhans cell histiocytosis. Pediatr Radiol. 37(7):615-22, 2007
                                            21. Prosch H et al: Long-term MR imaging course of neurodegenerative Langerhans cell histiocytosis. AJNR Am J Neuroradiol. 28(6):1022-8, 2007
                                            22. Horn EM et al: Isolated Langerhans cell histiocytosis of the infundibulum presenting with fulminant diabetes insipidus. Childs Nerv Syst. 22(5):542-4, 2006
                                            23. Martin-Duverneuil N et al: MRI features of neurodegenerative Langerhans cell histiocytosis. Eur Radiol. 16(9):2074-82, 2006
                                            Related Anatomy
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                                            Related Differential Diagnoses
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                                            References
                                            Tables

                                            Tables

                                            KEY FACTS

                                            • Terminology

                                              • Imaging

                                                • Top Differential Diagnoses

                                                  • Diagnostic Checklist

                                                    TERMINOLOGY

                                                    • Synonyms

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

                                                      • LCH now best understood as neoplastic disease
                                                        • Activating somatic BRAF mutations, LCH cell clonality
                                                        • Inflammatory myeloid neoplasia
                                                      • Divided into 3 groups (based on number of lesions, systems involved)
                                                        • Unifocal localized form (a.k.a. eosinophilic granuloma)
                                                          • 70% of cases
                                                          • Limited to single or a few bones, may involve lung
                                                        • Multifocal unisystem
                                                          • 20% of cases
                                                          • Chronic, recurring
                                                          • Multiple bones, reticuloendothelial system, pituitary/hypothalamus
                                                        • Multifocal, multisystem
                                                          • 10% of cases
                                                          • Fulminant (often fatal)

                                                    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. 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
                                                                                      2. Zheng H et al: Pediatric Langerhans cell histiocytosis of the temporal bone: clinical and imaging studies of 27 cases. World J Surg Oncol. 16(1):72, 2018
                                                                                      3. Chevallier KM et al: Differentiating pediatric rhabdomyosarcoma and Langerhans cell histiocytosis of the temporal bone by imaging appearance. AJNR Am J Neuroradiol. 37(6):1185-9, 2016
                                                                                      4. Berres ML et al: Progress in understanding the pathogenesis of Langerhans cell histiocytosis: back to Histiocytosis X? Br J Haematol. 169(1):3-13, 2015
                                                                                      5. De La Hoz Polo M et al: Neuroimaging of Langerhans cell histiocytosis in the central nervous system of children. Radiologia. 57(2):123-130, 2015
                                                                                      6. Demellawy DE et al: Langerhans cell histiocytosis: a comprehensive review. Pathology. 47(4):294-301, 2015
                                                                                      7. Esmaili N et al: Langerhans cll histiocytosis of the orbit: spectrum of disease and risk of central nervous system sequelae in unifocal cases. Ophthal Plast Reconstr Surg. ePub, 2015
                                                                                      8. Modest MC et al: Langerhans cell histiocytosis of the temporal bone: A review of 29 cases at a single center. Laryngoscope. ePub, 2015
                                                                                      9. Porto L et al: Central nervous system imaging in childhood Langerhans cell histiocytosis - a reference center analysis. Radiol Oncol. 49(3):242-9, 2015
                                                                                      10. Nabavizadeh SA et al: CT and MRI of Pediatric Skull Lesions with Fluid-Fluid Levels. AJNR Am J Neuroradiol. 35(3):604-8, 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. Badalian-Very G et al: Pathogenesis of Langerhans cell histiocytosis. Annu Rev Pathol. 8:1-20, 2013
                                                                                      13. Laurencikas E et al: Incidence and pattern of radiological central nervous system Langerhans cell histiocytosis in children: a population based study. Pediatr Blood Cancer. 56(2):250-7, 2011
                                                                                      14. Grois N et al: Central nervous system disease in Langerhans cell histiocytosis. J Pediatr. 156(6):873-81, 881, 2010
                                                                                      15. 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
                                                                                      16. Demaerel P et al: Paediatric neuroradiological aspects of Langerhans cell histiocytosis. Neuroradiology. 50(1):85-92, 2008
                                                                                      17. Saliba I et al: Langerhans' cell histiocytosis of the temporal bone in children. Int J Pediatr Otorhinolaryngol. 72(6):775-86, 2008
                                                                                      18. van der Knaap MS et al: Cerebellar leukoencephalopathy: most likely histiocytosis-related. Neurology. 71(17):1361-7, 2008
                                                                                      19. Varan A et al: Radiological evaluation of patients with pituitary langerhans cell histiocytosis at diagnosis and at follow-up. Pediatr Hematol Oncol. 25(6):567-74, 2008
                                                                                      20. Kaste SC et al: PET-CT in pediatric Langerhans cell histiocytosis. Pediatr Radiol. 37(7):615-22, 2007
                                                                                      21. Prosch H et al: Long-term MR imaging course of neurodegenerative Langerhans cell histiocytosis. AJNR Am J Neuroradiol. 28(6):1022-8, 2007
                                                                                      22. Horn EM et al: Isolated Langerhans cell histiocytosis of the infundibulum presenting with fulminant diabetes insipidus. Childs Nerv Syst. 22(5):542-4, 2006
                                                                                      23. Martin-Duverneuil N et al: MRI features of neurodegenerative Langerhans cell histiocytosis. Eur Radiol. 16(9):2074-82, 2006