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Langerhans Cell Histiocytosis, Brain
Gary L. Hedlund, DO
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KEY FACTS

  • Terminology

    • Imaging

      • Top Differential Diagnoses

        • Clinical Issues

          • Diagnostic Checklist

            TERMINOLOGY

            • Synonyms

              • Langerhans cell histiocytosis (LCH)
                • a.k.a. histiocytosis X (former)
              • Langerhans cell granulomatosis, eosinophilic granulomatosis
            • Definitions

              • Proliferation of Langerhans cell histiocytes that form granulomas within any organ system

            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. Badalian-Very G et al: Pathogenesis of Langerhans cell histiocytosis. Annu Rev Pathol. 8:1-20, 2013
                                              2. Huang WD et al: Langerhans cell histiocytosis of spine: a comparative study of clinical, imaging features, and diagnosis in children, adolescents, and adults. Spine J. 13(9):1108-17, 2013
                                              3. Badalian-Very G et al: Recent advances in the understanding of Langerhans cell histiocytosis. Br J Haematol. 156(2):163-72, 2012
                                              4. Grois N et al: Central nervous system disease in Langerhans cell histiocytosis. J Pediatr. 156(6):873-81, 881, 2010
                                              5. Giovannetti F et al: Langerhans cell histiocytosis with orbital involvement: our experience. J Oral Maxillofac Surg. 67(1):212-6, 2009
                                              6. Phillips M et al: Comparison of FDG-PET scans to conventional radiography and bone scans in management of Langerhans cell histiocytosis. Pediatr Blood Cancer. 52(1):97-101, 2009
                                              7. 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
                                              8. Demaerel P et al: Paediatric neuroradiological aspects of Langerhans cell histiocytosis. Neuroradiology. 50(1):85-92, 2008
                                              9. Nemet AY et al: A 7th nerve palsy in a child with Langerhans histiocytosis. Orbit. 27(2):123-5, 2008
                                              10. Saliba I et al: Langerhans' cell histiocytosis of the temporal bone in children. Int J Pediatr Otorhinolaryngol. 72(6):775-86, 2008
                                              11. van der Knaap MS et al: Cerebellar leukoencephalopathy: most likely histiocytosis-related. Neurology. 71(17):1361-7, 2008
                                              12. 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
                                              13. Hoover KB et al: Langerhans cell histiocytosis. Skeletal Radiol. 36(2):95-104, 2007
                                              14. Kaste SC et al: PET-CT in pediatric Langerhans cell histiocytosis. Pediatr Radiol. 37(7):615-22, 2007
                                              15. Prosch H et al: Long-term MR imaging course of neurodegenerative Langerhans cell histiocytosis. AJNR Am J Neuroradiol. 28(6):1022-8, 2007
                                              16. Goo HW et al: Whole-body MRI of Langerhans cell histiocytosis: comparison with radiography and bone scintigraphy. Pediatr Radiol. 36(10):1019-31, 2006
                                              17. Horn EM et al: Isolated Langerhans cell histiocytosis of the infundibulum presenting with fulminant diabetes insipidus. Childs Nerv Syst. 22(5):542-4, 2006
                                              18. Martin-Duverneuil N et al: MRI features of neurodegenerative Langerhans cell histiocytosis. Eur Radiol. 16(9):2074-82, 2006
                                              19. Kilborn TN et al: Paediatric manifestations of Langerhans cell histiocytosis: a review of the clinical and radiological findings. Clin Radiol. 58(4):269-78, 2003
                                              20. Oliveira M et al: Spontaneous resolution of calvarial eosinophilic granuloma in children. Pediatr Neurosurg. 38(5):247-52, 2003
                                              21. Chen HC et al: Langerhans cell histiocytosis of the skull complicated with an epidural hematoma. AJNR Am J Neuroradiol. 23(3):493-5, 2002
                                              22. Cho DY et al: Eosinophilic granuloma with acute epidural hematoma: a case report. Pediatr Neurosurg. 35(5):266-9, 2001
                                              23. Gizewski ER et al: Histiocytosis mimicking a pineal gland tumour. Neuroradiology. 43(8):644-6, 2001
                                              24. Gulam I et al: Langerhans' cell granulomatosis in an adult: a 22-year follow up. Eur Arch Otorhinolaryngol. 258(4):203-7, 2001
                                              25. Fernández-Latorre F et al: Langerhans' cell histiocytosis of the temporal bone in pediatric patients: imaging and follow-up. AJR Am J Roentgenol. 174(1):217-21, 2000
                                              26. Shuper A et al: Cerebellar involvement in Langerhans' cell histiocytosis: a progressive neuropsychiatric disease. J Child Neurol. 15(12):824-6, 2000
                                              Related Anatomy
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                                              Related Differential Diagnoses
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                                              References
                                              Tables

                                              Tables

                                              KEY FACTS

                                              • Terminology

                                                • Imaging

                                                  • Top Differential Diagnoses

                                                    • Clinical Issues

                                                      • Diagnostic Checklist

                                                        TERMINOLOGY

                                                        • Synonyms

                                                          • Langerhans cell histiocytosis (LCH)
                                                            • a.k.a. histiocytosis X (former)
                                                          • Langerhans cell granulomatosis, eosinophilic granulomatosis
                                                        • Definitions

                                                          • Proliferation of Langerhans cell histiocytes that form granulomas within any organ system

                                                        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. Badalian-Very G et al: Pathogenesis of Langerhans cell histiocytosis. Annu Rev Pathol. 8:1-20, 2013
                                                                                          2. Huang WD et al: Langerhans cell histiocytosis of spine: a comparative study of clinical, imaging features, and diagnosis in children, adolescents, and adults. Spine J. 13(9):1108-17, 2013
                                                                                          3. Badalian-Very G et al: Recent advances in the understanding of Langerhans cell histiocytosis. Br J Haematol. 156(2):163-72, 2012
                                                                                          4. Grois N et al: Central nervous system disease in Langerhans cell histiocytosis. J Pediatr. 156(6):873-81, 881, 2010
                                                                                          5. Giovannetti F et al: Langerhans cell histiocytosis with orbital involvement: our experience. J Oral Maxillofac Surg. 67(1):212-6, 2009
                                                                                          6. Phillips M et al: Comparison of FDG-PET scans to conventional radiography and bone scans in management of Langerhans cell histiocytosis. Pediatr Blood Cancer. 52(1):97-101, 2009
                                                                                          7. 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
                                                                                          8. Demaerel P et al: Paediatric neuroradiological aspects of Langerhans cell histiocytosis. Neuroradiology. 50(1):85-92, 2008
                                                                                          9. Nemet AY et al: A 7th nerve palsy in a child with Langerhans histiocytosis. Orbit. 27(2):123-5, 2008
                                                                                          10. Saliba I et al: Langerhans' cell histiocytosis of the temporal bone in children. Int J Pediatr Otorhinolaryngol. 72(6):775-86, 2008
                                                                                          11. van der Knaap MS et al: Cerebellar leukoencephalopathy: most likely histiocytosis-related. Neurology. 71(17):1361-7, 2008
                                                                                          12. 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
                                                                                          13. Hoover KB et al: Langerhans cell histiocytosis. Skeletal Radiol. 36(2):95-104, 2007
                                                                                          14. Kaste SC et al: PET-CT in pediatric Langerhans cell histiocytosis. Pediatr Radiol. 37(7):615-22, 2007
                                                                                          15. Prosch H et al: Long-term MR imaging course of neurodegenerative Langerhans cell histiocytosis. AJNR Am J Neuroradiol. 28(6):1022-8, 2007
                                                                                          16. Goo HW et al: Whole-body MRI of Langerhans cell histiocytosis: comparison with radiography and bone scintigraphy. Pediatr Radiol. 36(10):1019-31, 2006
                                                                                          17. Horn EM et al: Isolated Langerhans cell histiocytosis of the infundibulum presenting with fulminant diabetes insipidus. Childs Nerv Syst. 22(5):542-4, 2006
                                                                                          18. Martin-Duverneuil N et al: MRI features of neurodegenerative Langerhans cell histiocytosis. Eur Radiol. 16(9):2074-82, 2006
                                                                                          19. Kilborn TN et al: Paediatric manifestations of Langerhans cell histiocytosis: a review of the clinical and radiological findings. Clin Radiol. 58(4):269-78, 2003
                                                                                          20. Oliveira M et al: Spontaneous resolution of calvarial eosinophilic granuloma in children. Pediatr Neurosurg. 38(5):247-52, 2003
                                                                                          21. Chen HC et al: Langerhans cell histiocytosis of the skull complicated with an epidural hematoma. AJNR Am J Neuroradiol. 23(3):493-5, 2002
                                                                                          22. Cho DY et al: Eosinophilic granuloma with acute epidural hematoma: a case report. Pediatr Neurosurg. 35(5):266-9, 2001
                                                                                          23. Gizewski ER et al: Histiocytosis mimicking a pineal gland tumour. Neuroradiology. 43(8):644-6, 2001
                                                                                          24. Gulam I et al: Langerhans' cell granulomatosis in an adult: a 22-year follow up. Eur Arch Otorhinolaryngol. 258(4):203-7, 2001
                                                                                          25. Fernández-Latorre F et al: Langerhans' cell histiocytosis of the temporal bone in pediatric patients: imaging and follow-up. AJR Am J Roentgenol. 174(1):217-21, 2000
                                                                                          26. Shuper A et al: Cerebellar involvement in Langerhans' cell histiocytosis: a progressive neuropsychiatric disease. J Child Neurol. 15(12):824-6, 2000