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Skull Base Langerhans Cell Histiocytosis
C. Douglas Phillips, MD, FACR
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KEY FACTS

  • Terminology

    • Imaging

      • Top Differential Diagnoses

        • Clinical Issues

          TERMINOLOGY

          • Abbreviations

            • Langerhans cell histiocytosis (LCH)
          • Synonyms

            • Eosinophilic granuloma, histiocytosis X, Hand-Schüller-Christian disease, Letterer-Siwe disease
          • Definitions

            • LCH: Spectrum of disorders caused by neoplastic clonal proliferations of CD1a, CD207, S100 (+) dendritic cells
            • Single system (SS) (unifocal or multifocal) vs. multisystem (MS) disease
              • Bone and skin most frequently involved
              • Worse prognosis: Organs at high risk are liver, spleen, marrow
              • Other organs: Lymph nodes, pituitary, thymus, GI tract, CNS
              • H&N: Skull base, temporal bone (T-bone), craniofacial

          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

                                          • Reporting Tips

                                            Selected References

                                            1. 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
                                            2. Egeler RM et al: Langerhans cell histiocytosis is a neoplasm and consequently its recurrence is a relapse: In memory of Bob Arceci. Pediatr Blood Cancer. ePub, 2016
                                            3. 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, 2016
                                            4. Collin M et al: Cell(s) of Origin of Langerhans cell histiocytosis. Hematol Oncol Clin North Am. 29(5):825-38, 2015
                                            5. Demellawy DE et al: Langerhans cell histiocytosis: a comprehensive review. Pathology. 47(4):294-301, 2015
                                            6. Rollins BJ: Genomic Alterations in Langerhans cell histiocytosis. Hematol Oncol Clin North Am. 29(5):839-51, 2015
                                            7. Chung EM et al: From the archives of the AFIP. Pediatric orbit tumors and tumorlike lesions: osseous lesions of the orbit. Radiographics. 28(4):1193-214, 2008
                                            8. Prosch H et al: Long-term MR imaging course of neurodegenerative Langerhans cell histiocytosis. AJNR Am J Neuroradiol. 28(6):1022-8, 2007
                                            9. Krishna H et al: Solitary Langerhans-cell histiocytosis of the clivus and sphenoid sinus with parasellar and petrous extensions: case report and a review of literature. Surg Neurol. 62(5):447-54, 2004
                                            10. Prayer D et al: MR imaging presentation of intracranial disease associated with Langerhans cell histiocytosis. AJNR Am J Neuroradiol. 25(5):880-91, 2004
                                            11. Cochrane LA et al: Langerhans' cell histiocytosis in the paediatric population: presentation and treatment of head and neck manifestations. J Otolaryngol. 32(1):33-7, 2003
                                            12. Hurwitz CA et al: Case records of the Massachusetts General Hospital. Weekly clinicopathological exercises. Case 5-2002. A 15-year-old boy with a retro-orbital mass and impaired vision. N Engl J Med. 346(7):513-20, 2002
                                            13. Yetiser S et al: Eosinophilic granuloma of the bilateral temporal bone. Int J Pediatr Otorhinolaryngol. 62(2):169-73, 2002
                                            14. Koch BL: Langerhans histiocytosis of temporal bone: role of magnetic resonance imaging. Top Magn Reson Imaging. 11(1):66-74, 2000
                                            15. al-Ammar AY et al: Langerhans' cell histiocytosis: paediatric head and neck study. J Otolaryngol. 28(5):266-72, 1999
                                            16. Nanduri VR et al: Labyrinthine involvement in Langerhans' cell histiocytosis. Int J Pediatr Otorhinolaryngol. 46(1-2):109-15, 1998
                                            17. Angeli SI et al: Magnetic resonance imaging in the evaluation of Langerhans' cell histiocytosis of the temporal bone: case report. Otolaryngol Head Neck Surg. 114(1):120-4, 1996
                                            18. Bonafe A et al: Histiocytosis X of the petrous bone in the adult: MRI. Neuroradiology. 36(4):330-3, 1994
                                            19. Hermans R et al: Eosinophilic granuloma of the head and neck: CT and MRI features in three cases. Pediatr Radiol. 24(1):33-6, 1994
                                            20. Cataltepe O et al: Unusual location of histiocytosis X. Presenting as a petrous apex syndrome. J Clin Neuroophthalmol. 11(1):31-4, 1991
                                            21. Hadjigeorgi C et al: Eosinophilic granuloma of the temporal bone: radiological approach in the pediatric patient. Pediatr Radiol. 20(7):546-9, 1990
                                            22. DiNardo LJ et al: Head and neck manifestations of histiocytosis-X in children. Laryngoscope. 99(7 Pt 1):721-4, 1989
                                            23. Cunningham MJ et al: Histiocytosis X of the temporal bone: CT findings. J Comput Assist Tomogr. 12(1):70-4, 1988
                                            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

                                                    TERMINOLOGY

                                                    • Abbreviations

                                                      • Langerhans cell histiocytosis (LCH)
                                                    • Synonyms

                                                      • Eosinophilic granuloma, histiocytosis X, Hand-Schüller-Christian disease, Letterer-Siwe disease
                                                    • Definitions

                                                      • LCH: Spectrum of disorders caused by neoplastic clonal proliferations of CD1a, CD207, S100 (+) dendritic cells
                                                      • Single system (SS) (unifocal or multifocal) vs. multisystem (MS) disease
                                                        • Bone and skin most frequently involved
                                                        • Worse prognosis: Organs at high risk are liver, spleen, marrow
                                                        • Other organs: Lymph nodes, pituitary, thymus, GI tract, CNS
                                                        • H&N: Skull base, temporal bone (T-bone), craniofacial

                                                    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

                                                                                    • Reporting Tips

                                                                                      Selected References

                                                                                      1. 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
                                                                                      2. Egeler RM et al: Langerhans cell histiocytosis is a neoplasm and consequently its recurrence is a relapse: In memory of Bob Arceci. Pediatr Blood Cancer. ePub, 2016
                                                                                      3. 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, 2016
                                                                                      4. Collin M et al: Cell(s) of Origin of Langerhans cell histiocytosis. Hematol Oncol Clin North Am. 29(5):825-38, 2015
                                                                                      5. Demellawy DE et al: Langerhans cell histiocytosis: a comprehensive review. Pathology. 47(4):294-301, 2015
                                                                                      6. Rollins BJ: Genomic Alterations in Langerhans cell histiocytosis. Hematol Oncol Clin North Am. 29(5):839-51, 2015
                                                                                      7. Chung EM et al: From the archives of the AFIP. Pediatric orbit tumors and tumorlike lesions: osseous lesions of the orbit. Radiographics. 28(4):1193-214, 2008
                                                                                      8. Prosch H et al: Long-term MR imaging course of neurodegenerative Langerhans cell histiocytosis. AJNR Am J Neuroradiol. 28(6):1022-8, 2007
                                                                                      9. Krishna H et al: Solitary Langerhans-cell histiocytosis of the clivus and sphenoid sinus with parasellar and petrous extensions: case report and a review of literature. Surg Neurol. 62(5):447-54, 2004
                                                                                      10. Prayer D et al: MR imaging presentation of intracranial disease associated with Langerhans cell histiocytosis. AJNR Am J Neuroradiol. 25(5):880-91, 2004
                                                                                      11. Cochrane LA et al: Langerhans' cell histiocytosis in the paediatric population: presentation and treatment of head and neck manifestations. J Otolaryngol. 32(1):33-7, 2003
                                                                                      12. Hurwitz CA et al: Case records of the Massachusetts General Hospital. Weekly clinicopathological exercises. Case 5-2002. A 15-year-old boy with a retro-orbital mass and impaired vision. N Engl J Med. 346(7):513-20, 2002
                                                                                      13. Yetiser S et al: Eosinophilic granuloma of the bilateral temporal bone. Int J Pediatr Otorhinolaryngol. 62(2):169-73, 2002
                                                                                      14. Koch BL: Langerhans histiocytosis of temporal bone: role of magnetic resonance imaging. Top Magn Reson Imaging. 11(1):66-74, 2000
                                                                                      15. al-Ammar AY et al: Langerhans' cell histiocytosis: paediatric head and neck study. J Otolaryngol. 28(5):266-72, 1999
                                                                                      16. Nanduri VR et al: Labyrinthine involvement in Langerhans' cell histiocytosis. Int J Pediatr Otorhinolaryngol. 46(1-2):109-15, 1998
                                                                                      17. Angeli SI et al: Magnetic resonance imaging in the evaluation of Langerhans' cell histiocytosis of the temporal bone: case report. Otolaryngol Head Neck Surg. 114(1):120-4, 1996
                                                                                      18. Bonafe A et al: Histiocytosis X of the petrous bone in the adult: MRI. Neuroradiology. 36(4):330-3, 1994
                                                                                      19. Hermans R et al: Eosinophilic granuloma of the head and neck: CT and MRI features in three cases. Pediatr Radiol. 24(1):33-6, 1994
                                                                                      20. Cataltepe O et al: Unusual location of histiocytosis X. Presenting as a petrous apex syndrome. J Clin Neuroophthalmol. 11(1):31-4, 1991
                                                                                      21. Hadjigeorgi C et al: Eosinophilic granuloma of the temporal bone: radiological approach in the pediatric patient. Pediatr Radiol. 20(7):546-9, 1990
                                                                                      22. DiNardo LJ et al: Head and neck manifestations of histiocytosis-X in children. Laryngoscope. 99(7 Pt 1):721-4, 1989
                                                                                      23. Cunningham MJ et al: Histiocytosis X of the temporal bone: CT findings. J Comput Assist Tomogr. 12(1):70-4, 1988