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Langerhans Cell Histiocytosis
Kevin R. Moore, MD
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KEY FACTS

  • Terminology

    • Imaging

      • Top Differential Diagnoses

        • Pathology

          • Clinical Issues

            • Diagnostic Checklist

              TERMINOLOGY

              • Synonyms

                • Langerhans cell histiocytosis (LCH), Langerhans cell granulomatosis, eosinophilic granuloma (EG), histiocytosis X
              • Definitions

                • Abnormal histiocyte proliferation producing granulomatous skeletal lesions

              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. Nakamura N et al: Characteristic reconstitution of the spinal langerhans cell histiocytosis in young children. J Pediatr Orthop. 39(4):e308-e11, 2019
                                                2. Nakashima K et al: Radiotherapy for Langerhans cell histiocytosis with paraplegia: A rare oncologic emergency case report in infancy and literature review. Brain Dev. 40(10):952-55, 2018
                                                3. Lee SK et al: Solitary Langerhans cell histocytosis of skull and spine in pediatric and adult patients. Childs Nerv Syst. 30(2):271-5, 2014
                                                4. Lee SW et al: Long-term clinical outcome of spinal Langerhans cell histiocytosis in children. Int J Hematol. 106(3):441-9, 2017
                                                5. Xu X et al: Clinical features and treatment outcomes of Langerhans cell histiocytosis of the spine. Spine J. 18(10):1755-62, 2018
                                                6. Lü GH et al: Surgical treatment based on pedicle screw instrumentation for thoracic or lumbar spinal Langerhans cell histiocytosis complicated with neurologic deficit in children. Spine J. 14(5):768-76, 2014
                                                7. Vielgut I et al: Langerhans-cell histiocytosis of the cervical spine in an adult patient: case report and review of the literature. J Orthop. 14(2):264-7, 2017
                                                8. Sadashiva N et al: Surgical treatment of Langerhans cell histiocytosis of cervical spine: case report and review of literature. Childs Nerv Syst. 32(6):1149-52, 2016
                                                9. Chaudhary V et al: Neuroimaging of Langerhans cell histiocytosis: a radiological review. Jpn J Radiol. 31(12):786-96, 2013
                                                10. 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
                                                11. Khung S et al: Skeletal involvement in Langerhans cell histiocytosis. Insights Imaging. 4(5):569-79, 2013
                                                12. Montemurro N et al: Epidural spinal cord compression in Langerhans cell histiocytosis: a case report. Br J Neurosurg. 27(6):838-9, 2013
                                                13. Hung YC et al: Langerhans' cell histiocytosis in the pediatric spine: therapeutic dynamic change of spinal deformity. Childs Nerv Syst. 28(8):1243-50, 2012
                                                14. Ueda Y et al: Eosinophilic granuloma of the lumbar spine in an adult. Orthopedics. 35(12):e1818-21, 2012
                                                15. Hyun SJ et al: Simultaneous occurrence of Hodgkin's lymphoma and Langerhans cell histiocytosis of the spine: a rare combination. J Korean Neurosurg Soc. 47(4):302-5, 2010
                                                16. Jiang L et al: Langerhans cell histiocytosis of the cervical spine: a single Chinese institution experience with thirty cases. Spine (Phila Pa 1976). 35(1):E8-15, 2010
                                                17. von Stebut E et al: Successful treatment of adult multisystemic Langerhans cell histiocytosis with psoralen-UV-A, prednisolone, mercaptopurine, and vinblastine. Arch Dermatol. 144(5):649-53, 2008
                                                18. Bavbek M et al: Spontaneous resolution of lumbar vertebral eosinophilic granuloma. Acta Neurochir (Wien). 146(2):165-7, 2004
                                                19. Garg S et al: Langerhans cell histiocytosis of the spine in children. Long-term follow-up. J Bone Joint Surg Am. 86-A(8):1740-50, 2004
                                                20. Kuhn J et al: Caffey's Pediatric Diagnostic Imaging. 10th ed. Philadelphia: Mosby. 704, 2004
                                                21. Simanski C et al: The Langerhans' cell histiocytosis (eosinophilic granuloma) of the cervical spine: a rare diagnosis of cervical pain. Magn Reson Imaging. 22(4):589-94, 2004
                                                22. Fernando Ugarriza L et al: Solitary eosinophilic granuloma of the cervicothoracic junction causing neurological deficit. Br J Neurosurg. 17(2):178-81, 2003
                                                23. Puertas EB et al: Surgical treatment of eosinophilic granuloma in the thoracic spine in patients with neurological lesions. J Pediatr Orthop B. 12(5):303-6, 2003
                                                24. Bertram C et al: Eosinophilic granuloma of the cervical spine. Spine. 27(13):1408-13, 2002
                                                25. Graham D et al: Greenfield's Neuropathology. 7th ed. London: Arnold. 1017-18, 2002
                                                26. Plasschaert F et al: Eosinophilic granuloma. A different behaviour in children than in adults. J Bone Joint Surg Br. 84(6):870-2, 2002
                                                27. Kamimura M et al: Eosinophilic granuloma of the spine: early spontaneous disappearance of tumor detected on magnetic resonance imaging. Case report. J Neurosurg. 93(2 Suppl):312-6, 2000
                                                28. Reddy PK et al: Eosinophilic granuloma of spine in adults: a case report and review of literature. Spinal Cord. 38(12):766-8, 2000
                                                29. Yeom JS et al: Langerhans' cell histiocytosis of the spine. Analysis of twenty-three cases. Spine. 24(16):1740-9, 1999
                                                30. Kandoi M et al: Rapidly progressive polyostotic eosinophilic granuloma involving spine: a case report. Indian J Med Sci. 52(1):22-4, 1998
                                                31. Raab P et al: Vertebral remodeling in eosinophilic granuloma of the spine. A long-term follow-up. Spine. 23(12):1351-4, 1998
                                                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

                                                          • Diagnostic Checklist

                                                            TERMINOLOGY

                                                            • Synonyms

                                                              • Langerhans cell histiocytosis (LCH), Langerhans cell granulomatosis, eosinophilic granuloma (EG), histiocytosis X
                                                            • Definitions

                                                              • Abnormal histiocyte proliferation producing granulomatous skeletal lesions

                                                            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. Nakamura N et al: Characteristic reconstitution of the spinal langerhans cell histiocytosis in young children. J Pediatr Orthop. 39(4):e308-e11, 2019
                                                                                              2. Nakashima K et al: Radiotherapy for Langerhans cell histiocytosis with paraplegia: A rare oncologic emergency case report in infancy and literature review. Brain Dev. 40(10):952-55, 2018
                                                                                              3. Lee SK et al: Solitary Langerhans cell histocytosis of skull and spine in pediatric and adult patients. Childs Nerv Syst. 30(2):271-5, 2014
                                                                                              4. Lee SW et al: Long-term clinical outcome of spinal Langerhans cell histiocytosis in children. Int J Hematol. 106(3):441-9, 2017
                                                                                              5. Xu X et al: Clinical features and treatment outcomes of Langerhans cell histiocytosis of the spine. Spine J. 18(10):1755-62, 2018
                                                                                              6. Lü GH et al: Surgical treatment based on pedicle screw instrumentation for thoracic or lumbar spinal Langerhans cell histiocytosis complicated with neurologic deficit in children. Spine J. 14(5):768-76, 2014
                                                                                              7. Vielgut I et al: Langerhans-cell histiocytosis of the cervical spine in an adult patient: case report and review of the literature. J Orthop. 14(2):264-7, 2017
                                                                                              8. Sadashiva N et al: Surgical treatment of Langerhans cell histiocytosis of cervical spine: case report and review of literature. Childs Nerv Syst. 32(6):1149-52, 2016
                                                                                              9. Chaudhary V et al: Neuroimaging of Langerhans cell histiocytosis: a radiological review. Jpn J Radiol. 31(12):786-96, 2013
                                                                                              10. 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
                                                                                              11. Khung S et al: Skeletal involvement in Langerhans cell histiocytosis. Insights Imaging. 4(5):569-79, 2013
                                                                                              12. Montemurro N et al: Epidural spinal cord compression in Langerhans cell histiocytosis: a case report. Br J Neurosurg. 27(6):838-9, 2013
                                                                                              13. Hung YC et al: Langerhans' cell histiocytosis in the pediatric spine: therapeutic dynamic change of spinal deformity. Childs Nerv Syst. 28(8):1243-50, 2012
                                                                                              14. Ueda Y et al: Eosinophilic granuloma of the lumbar spine in an adult. Orthopedics. 35(12):e1818-21, 2012
                                                                                              15. Hyun SJ et al: Simultaneous occurrence of Hodgkin's lymphoma and Langerhans cell histiocytosis of the spine: a rare combination. J Korean Neurosurg Soc. 47(4):302-5, 2010
                                                                                              16. Jiang L et al: Langerhans cell histiocytosis of the cervical spine: a single Chinese institution experience with thirty cases. Spine (Phila Pa 1976). 35(1):E8-15, 2010
                                                                                              17. von Stebut E et al: Successful treatment of adult multisystemic Langerhans cell histiocytosis with psoralen-UV-A, prednisolone, mercaptopurine, and vinblastine. Arch Dermatol. 144(5):649-53, 2008
                                                                                              18. Bavbek M et al: Spontaneous resolution of lumbar vertebral eosinophilic granuloma. Acta Neurochir (Wien). 146(2):165-7, 2004
                                                                                              19. Garg S et al: Langerhans cell histiocytosis of the spine in children. Long-term follow-up. J Bone Joint Surg Am. 86-A(8):1740-50, 2004
                                                                                              20. Kuhn J et al: Caffey's Pediatric Diagnostic Imaging. 10th ed. Philadelphia: Mosby. 704, 2004
                                                                                              21. Simanski C et al: The Langerhans' cell histiocytosis (eosinophilic granuloma) of the cervical spine: a rare diagnosis of cervical pain. Magn Reson Imaging. 22(4):589-94, 2004
                                                                                              22. Fernando Ugarriza L et al: Solitary eosinophilic granuloma of the cervicothoracic junction causing neurological deficit. Br J Neurosurg. 17(2):178-81, 2003
                                                                                              23. Puertas EB et al: Surgical treatment of eosinophilic granuloma in the thoracic spine in patients with neurological lesions. J Pediatr Orthop B. 12(5):303-6, 2003
                                                                                              24. Bertram C et al: Eosinophilic granuloma of the cervical spine. Spine. 27(13):1408-13, 2002
                                                                                              25. Graham D et al: Greenfield's Neuropathology. 7th ed. London: Arnold. 1017-18, 2002
                                                                                              26. Plasschaert F et al: Eosinophilic granuloma. A different behaviour in children than in adults. J Bone Joint Surg Br. 84(6):870-2, 2002
                                                                                              27. Kamimura M et al: Eosinophilic granuloma of the spine: early spontaneous disappearance of tumor detected on magnetic resonance imaging. Case report. J Neurosurg. 93(2 Suppl):312-6, 2000
                                                                                              28. Reddy PK et al: Eosinophilic granuloma of spine in adults: a case report and review of literature. Spinal Cord. 38(12):766-8, 2000
                                                                                              29. Yeom JS et al: Langerhans' cell histiocytosis of the spine. Analysis of twenty-three cases. Spine. 24(16):1740-9, 1999
                                                                                              30. Kandoi M et al: Rapidly progressive polyostotic eosinophilic granuloma involving spine: a case report. Indian J Med Sci. 52(1):22-4, 1998
                                                                                              31. Raab P et al: Vertebral remodeling in eosinophilic granuloma of the spine. A long-term follow-up. Spine. 23(12):1351-4, 1998