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Temporal Bone Langerhans Cell Histiocytosis
Richard H. Wiggins, III, MD, CIIP, FSIIM, FAHSE, FACR
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KEY FACTS

  • Terminology

    • Imaging

      • Top Differential Diagnoses

        • Pathology

          • Clinical Issues

            TERMINOLOGY

            • Abbreviations

              • Langerhans cell histiocytosis (LCH)
            • Synonyms

              • Dendritic cell histiocytosis (DCH), histiocytosis X, eosinophilic granuloma (EG), Hand-Schüller-Christian disease (HSCD), Letterer-Siwe disease, Abt-Letterer-Siwe disease (ALSD), Hashimoto-Pritzker
            • Definitions

              • Proliferation of dendritic cell histiocytes, which form granulomas within any organ system, including T-bone

            IMAGING

            • General Features

              • Radiographic Findings

                • CT Findings

                  • MR 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

                                            • Reporting Tips

                                              Selected References

                                              1. Ong AA et al: Langerhans cell histiocytosis of the skull. Ear Nose Throat J. 100(4):219-21, 2021
                                              2. Mayer S et al: Langerhans cell histiocytosis of the temporal bone. Ochsner J. 20(3):315-8, 2020
                                              3. Flaman AN et al: Ear and temporal bone pathology: neural, sclerosing and myofibroblastic lesions. Head Neck Pathol. 12(3):392-406, 2018
                                              4. Haefliger S et al: PET-positive bone lesion due to Langerhans cell histiocytosis after BEACOPP therapy for Hodgkin lymphoma: how anamnesis, histopathological accuracy, and molecular analysis could resolve a clinical dilemma. Ann Hematol. 97(2):355-7, 2018
                                              5. 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
                                              6. Majumder A et al: Pediatric Langerhans cell histiocytosis of the lateral skull base. Int J Pediatr Otorhinolaryngol. 99:135-40, 2017
                                              7. 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
                                              8. Shew M et al: Langerhan's cell histiocytosis in an immunosuppressed patient isolated to the temporal bone. Otol Neurotol. 37(6):e194-6, 2016
                                              9. Mener DJ et al: Temporal bone involvement in Hand-Schuller-Christian disease. Otol Neurotol. 36(3):e95-6, 2015
                                              10. Modest MC et al: Langerhans cell histiocytosis of the temporal bone: a review of 29 cases at a single center. Laryngoscope. 126(8):1899-904, 2015
                                              11. Abdel-Aziz M et al: Eosinophilic granuloma of the temporal bone in children. J Craniofac Surg. 25(3):1076-8, 2014
                                              12. Nabavizadeh SA et al: CT and MRI of pediatric skull lesions with fluid-fluid levels. AJNR Am J Neuroradiol. 35(3):604-8, 2014
                                              13. Radhakrishnan R et al: Petrous apex lesions in the pediatric population. Pediatr Radiol. 44(3):325-39; quiz 323-4, 2014
                                              14. Alexander RL et al: Langerhans cell histiocytosis: temporal bone invasion in an adult. Ear Nose Throat J. 92(10-11):496, 498, 2013
                                              15. Coleman MA et al: Bilateral temporal bone langerhans cell histiocytosis: radiologic pearls. Open Neuroimag J. 7:53-7, 2013
                                              16. Inci MF et al: Multidetector CT findings of calvarial eosinophilic granuloma. BMJ Case Rep, 2013
                                              17. Neilan RE et al: Langerhans cell histiocytosis of the temporal bone. Otol Neurotol. 33(4):e31-2, 2012
                                              18. 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
                                              19. Grois N et al: Central nervous system disease in Langerhans cell histiocytosis. J Pediatr. 156(6):873-81, 881, 2010
                                              20. Nicollas R et al: Head and neck manifestation and prognosis of Langerhans' cell histiocytosis in children. Int J Pediatr Otorhinolaryngol. 74(6):669-73, 2010
                                              21. Roger G et al: Cholesteatoma secondary to temporal bone involvement by Langerhans cell histiocytosis: a complication amenable to curative surgery. Otol Neurotol. 30(2):190-3, 2009
                                              22. Saliba I et al: Prognostic indicators for sensorineural hearing loss in temporal bone histiocytosis. Int J Pediatr Otorhinolaryngol. 73(12):1616-20, 2009
                                              23. Zlodre JK et al: Langerhans' cell histiocytosis of the temporal bone: successful treatment of sensorineural hearing loss with low-dose radiotherapy. J Laryngol Otol. 123(6):676-9, 2009
                                              24. 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
                                              25. Saliba I et al: Langerhans' cell histiocytosis of the temporal bone in children. Int J Pediatr Otorhinolaryngol. 72(6):775-86, 2008
                                              26. del Río L et al: Petrous bone Langerhans cell histiocytosis treated with radiosurgery. Stereotact Funct Neurosurg. 85(2-3):129-31, 2007
                                              27. Baumann C et al: Destruction of the vestibular organ by Langerhans' cell histiocytosis. Eur Radiol. 16(5):1177-8, 2006
                                              28. de Brito Macedo Ferreira LM et al: Histiocytosis X of the temporal bone. Braz J Otorhinolaryngol. 72(4):575, 2006
                                              29. Mosnier I et al: Langerhans' cell histiocytosis of the labyrinth in adults. Otol Neurotol. 25(1):27-32, 2004
                                              30. Kleinjung T et al: Langerhans' cell histiocytosis with bilateral temporal bone involvement. Am J Otolaryngol. 24(4):265-70, 2003
                                              31. Mistry H et al: Remodeling of the temporal bone after involvement with Langerhans' cell histiocytosis. Acta Radiol. 44(3):355, 2003
                                              32. Boston M et al: Langerhans' cell histiocytosis of the temporal bone and skull base. Am J Otolaryngol. 23(4):246-8, 2002
                                              33. Yetiser S et al: Eosinophilic granuloma of the bilateral temporal bone. Int J Pediatr Otorhinolaryngol. 62(2):169-73, 2002
                                              34. Bayazit Y et al: Eosinophilic granuloma of the temporal bone. Auris Nasus Larynx. 28(1):99-102, 2001
                                              35. Marioni G et al: Langerhans' cell histiocytosis: temporal bone involvement. J Laryngol Otol. 115(10):839-41, 2001
                                              36. 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
                                              37. Koch BL: Langerhans histiocytosis of temporal bone: role of magnetic resonance imaging. Top Magn Reson Imaging. 11(1):66-74, 2000
                                              38. Bonafé A et al: Histiocytosis X of the petrous bone in the adult: MRI. Neuroradiology. 36(4):330-3, 1994
                                              39. Goldsmith AJ et al: Unifocal Langerhans' cell histiocytosis (eosinophilic granuloma) of the petrous apex. Arch Otolaryngol Head Neck Surg. 119(1):113-6, 1993
                                              40. Cunningham MJ et al: Histiocytosis X of the temporal bone: CT findings. J Comput Assist Tomogr. 12(1):70-4, 1988
                                              41. Fradis M et al: Eosinophilic granuloma of the temporal bone. J Laryngol Otol. 99(5):475-9, 1985
                                              42. Kimmelman CP et al: Histiocytosis X of the temporal bone. Otolaryngol Head Neck Surg. 92(5):588-90, 1984
                                              43. McCaffrey TV et al: Histiocytosis X of the ear and temporal bone: review of 22 cases. Laryngoscope. 89(11):1735-42, 1979
                                              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

                                                          • Langerhans cell histiocytosis (LCH)
                                                        • Synonyms

                                                          • Dendritic cell histiocytosis (DCH), histiocytosis X, eosinophilic granuloma (EG), Hand-Schüller-Christian disease (HSCD), Letterer-Siwe disease, Abt-Letterer-Siwe disease (ALSD), Hashimoto-Pritzker
                                                        • Definitions

                                                          • Proliferation of dendritic cell histiocytes, which form granulomas within any organ system, including T-bone

                                                        IMAGING

                                                        • General Features

                                                          • Radiographic Findings

                                                            • CT Findings

                                                              • MR 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

                                                                                        • Reporting Tips

                                                                                          Selected References

                                                                                          1. Ong AA et al: Langerhans cell histiocytosis of the skull. Ear Nose Throat J. 100(4):219-21, 2021
                                                                                          2. Mayer S et al: Langerhans cell histiocytosis of the temporal bone. Ochsner J. 20(3):315-8, 2020
                                                                                          3. Flaman AN et al: Ear and temporal bone pathology: neural, sclerosing and myofibroblastic lesions. Head Neck Pathol. 12(3):392-406, 2018
                                                                                          4. Haefliger S et al: PET-positive bone lesion due to Langerhans cell histiocytosis after BEACOPP therapy for Hodgkin lymphoma: how anamnesis, histopathological accuracy, and molecular analysis could resolve a clinical dilemma. Ann Hematol. 97(2):355-7, 2018
                                                                                          5. 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
                                                                                          6. Majumder A et al: Pediatric Langerhans cell histiocytosis of the lateral skull base. Int J Pediatr Otorhinolaryngol. 99:135-40, 2017
                                                                                          7. 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
                                                                                          8. Shew M et al: Langerhan's cell histiocytosis in an immunosuppressed patient isolated to the temporal bone. Otol Neurotol. 37(6):e194-6, 2016
                                                                                          9. Mener DJ et al: Temporal bone involvement in Hand-Schuller-Christian disease. Otol Neurotol. 36(3):e95-6, 2015
                                                                                          10. Modest MC et al: Langerhans cell histiocytosis of the temporal bone: a review of 29 cases at a single center. Laryngoscope. 126(8):1899-904, 2015
                                                                                          11. Abdel-Aziz M et al: Eosinophilic granuloma of the temporal bone in children. J Craniofac Surg. 25(3):1076-8, 2014
                                                                                          12. Nabavizadeh SA et al: CT and MRI of pediatric skull lesions with fluid-fluid levels. AJNR Am J Neuroradiol. 35(3):604-8, 2014
                                                                                          13. Radhakrishnan R et al: Petrous apex lesions in the pediatric population. Pediatr Radiol. 44(3):325-39; quiz 323-4, 2014
                                                                                          14. Alexander RL et al: Langerhans cell histiocytosis: temporal bone invasion in an adult. Ear Nose Throat J. 92(10-11):496, 498, 2013
                                                                                          15. Coleman MA et al: Bilateral temporal bone langerhans cell histiocytosis: radiologic pearls. Open Neuroimag J. 7:53-7, 2013
                                                                                          16. Inci MF et al: Multidetector CT findings of calvarial eosinophilic granuloma. BMJ Case Rep, 2013
                                                                                          17. Neilan RE et al: Langerhans cell histiocytosis of the temporal bone. Otol Neurotol. 33(4):e31-2, 2012
                                                                                          18. 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
                                                                                          19. Grois N et al: Central nervous system disease in Langerhans cell histiocytosis. J Pediatr. 156(6):873-81, 881, 2010
                                                                                          20. Nicollas R et al: Head and neck manifestation and prognosis of Langerhans' cell histiocytosis in children. Int J Pediatr Otorhinolaryngol. 74(6):669-73, 2010
                                                                                          21. Roger G et al: Cholesteatoma secondary to temporal bone involvement by Langerhans cell histiocytosis: a complication amenable to curative surgery. Otol Neurotol. 30(2):190-3, 2009
                                                                                          22. Saliba I et al: Prognostic indicators for sensorineural hearing loss in temporal bone histiocytosis. Int J Pediatr Otorhinolaryngol. 73(12):1616-20, 2009
                                                                                          23. Zlodre JK et al: Langerhans' cell histiocytosis of the temporal bone: successful treatment of sensorineural hearing loss with low-dose radiotherapy. J Laryngol Otol. 123(6):676-9, 2009
                                                                                          24. 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
                                                                                          25. Saliba I et al: Langerhans' cell histiocytosis of the temporal bone in children. Int J Pediatr Otorhinolaryngol. 72(6):775-86, 2008
                                                                                          26. del Río L et al: Petrous bone Langerhans cell histiocytosis treated with radiosurgery. Stereotact Funct Neurosurg. 85(2-3):129-31, 2007
                                                                                          27. Baumann C et al: Destruction of the vestibular organ by Langerhans' cell histiocytosis. Eur Radiol. 16(5):1177-8, 2006
                                                                                          28. de Brito Macedo Ferreira LM et al: Histiocytosis X of the temporal bone. Braz J Otorhinolaryngol. 72(4):575, 2006
                                                                                          29. Mosnier I et al: Langerhans' cell histiocytosis of the labyrinth in adults. Otol Neurotol. 25(1):27-32, 2004
                                                                                          30. Kleinjung T et al: Langerhans' cell histiocytosis with bilateral temporal bone involvement. Am J Otolaryngol. 24(4):265-70, 2003
                                                                                          31. Mistry H et al: Remodeling of the temporal bone after involvement with Langerhans' cell histiocytosis. Acta Radiol. 44(3):355, 2003
                                                                                          32. Boston M et al: Langerhans' cell histiocytosis of the temporal bone and skull base. Am J Otolaryngol. 23(4):246-8, 2002
                                                                                          33. Yetiser S et al: Eosinophilic granuloma of the bilateral temporal bone. Int J Pediatr Otorhinolaryngol. 62(2):169-73, 2002
                                                                                          34. Bayazit Y et al: Eosinophilic granuloma of the temporal bone. Auris Nasus Larynx. 28(1):99-102, 2001
                                                                                          35. Marioni G et al: Langerhans' cell histiocytosis: temporal bone involvement. J Laryngol Otol. 115(10):839-41, 2001
                                                                                          36. 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
                                                                                          37. Koch BL: Langerhans histiocytosis of temporal bone: role of magnetic resonance imaging. Top Magn Reson Imaging. 11(1):66-74, 2000
                                                                                          38. Bonafé A et al: Histiocytosis X of the petrous bone in the adult: MRI. Neuroradiology. 36(4):330-3, 1994
                                                                                          39. Goldsmith AJ et al: Unifocal Langerhans' cell histiocytosis (eosinophilic granuloma) of the petrous apex. Arch Otolaryngol Head Neck Surg. 119(1):113-6, 1993
                                                                                          40. Cunningham MJ et al: Histiocytosis X of the temporal bone: CT findings. J Comput Assist Tomogr. 12(1):70-4, 1988
                                                                                          41. Fradis M et al: Eosinophilic granuloma of the temporal bone. J Laryngol Otol. 99(5):475-9, 1985
                                                                                          42. Kimmelman CP et al: Histiocytosis X of the temporal bone. Otolaryngol Head Neck Surg. 92(5):588-90, 1984
                                                                                          43. McCaffrey TV et al: Histiocytosis X of the ear and temporal bone: review of 22 cases. Laryngoscope. 89(11):1735-42, 1979