link
Bookmarks
Langerhans Cell Histiocytosis, Skull and Brain
Luke L. Linscott, MDAnne G. Osborn, MD, FACRGary L. Hedlund, DO
To access 4,300 diagnoses written by the world's leading experts in radiology.Try it free - 15 days
0
10
24
4

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
                                            Loading...
                                            Related Differential Diagnoses
                                            Loading...
                                            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