Key Points
• Langerhans cell histiocytosis replaces the other descriptive terms for this idiopathic disorder that included histiocytosis X and eosinophilic granuloma.
• LCH is a multisystemic disorder that can affect any organ. Prognosis depends on age of onset and extent of disease involvement.
• Skeletal involvement is common.
• Typically skeletal lesions are well defined. The presence of sclerotic margin is variable.
• Flat bones often have a central button sequestrum.
• A radiographic skeletal survey is the current screening examination of choice, but the use of whole body MR imaging is being increasingly used.
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References
Abbott GF, Rosado-de-Christenson ML, Franks TJ, Frazier AA, Galvin JR (2004) From the archives of the AFIP: pulmonary Langerhans cell histiocytosis. Radiographics 24(3):821–841
Avery ME, McAfee JG, Guild HG (1957) The course and prognosis of reticuloendotheliosis (eosinophilic granuloma, Schuller-Christian disease and Letterer-Siwe disease); a study of forty cases. Am J Med 22(4):636–652
Azouz EM, Saigal G, Rodriguez MM, Podda A (2005) Langerhans’ cell histiocytosis: pathology, imaging and treatment of skeletal involvement. Pediatr Radiol 35(2):103–115
Beltran J, Aparisi F, Bonmati LM, Rosenberg ZS, Present D, Steiner GC (1993) Eosinophilic granuloma: MRI manifestations. Skeletal Radiol 22:157–161
Berry DH, Brecon DL (1987) Natural history of hitiocytosis X. Hematol Oncol Clin North Am 1:23–34
Betts DR, Leibundgut KE, Feldges A, Pluss HJ, Niggli FK (1998) Cytogenetic abnormalities in Langerhans cell histiocytosis. Br J Cancer 77(4):552–555
Binkovitz LA, Olshefski RS, Adler BH (2003) Coincidence FDG-PET in the evaluation of Langerhans’ cell histiocytosis: preliminary findings. Pediatr Radiol 33(9):598–602
Blum R, Seymour JF, Hicks RJ (2002) Role of 18FDG-positron emission tomography scanning in the management of histiocytosis. Leuk Lymphoma. 43(11):2155–2157
Brauner MW, Grenier P, Mouelhi MM, Mompoint D, Lenoir S (1989) Pulmonary histiocytosis X: evaluation with high-resolution CT. Radiology 172(1):255–258
Broadbent V, Gadner H, Komp DM, Ladisch S (1989) Histiocytosis syndromes in children: II. Approach to the clinical and laboratory evaluation of children with Langerhans cell histiocytosis. Clinical Writing Group of the Histiocyte Society. Med Pediatr Oncol 17:492–495
Buchmann L, Emami A, Wei JL (2006) Primary head and neck Langerhans cell histiocytosis in children. Otolaryngol Head Neck Surg 135:312–317
Carstensen H, Ornvold K (1993) The epidemiology of Langerhans cell histiocytosis in children in Denmark, 1975–1989. Med Pediatr Oncol 21:387–388
Ceci A et al. (1988) Etoposide in recurrent childhood Langerhans’ cell histiocytosis: an Italian co-sperative Study. Cancer 62:2528–2531
Chan YL, Li CK, Lee CY (1997) Sonographic appearance of hepatic Langerhans cell histiocytosis. Clin Radiol 52(10):761–763
Chu T, D’Angio GJ, Favara BE, Ladisch S, Nesbit M, Pritchard J (1987) Histiocytosis syndromes in children. Lancet 2(8549):41–42
Cochrane LA, Prince M, Clarke K (2003) Langerhans’ cell histiocytosis in the paediatric population: presentation and treatment of head and neck manifestations. J Otolaryngol 1:33–37
Coppes-Zantinga A, Egeler RM (2002) The Langerhans cell histiocytosis X files revealed. Br J Haematol 116(1):3–9
David R, Oria RA, Kumar R, Singleton EB, Lindell MM, Shirkhoda A et al. (1989) Radiologic features of eosinophilic granuloma of bone. AJR Am J Roentgenol 153(5):1021–1026
Davies AM, Pikoulas C, Griffith J (1994) MRI of eosinophilic granuloma. Eur J Radiol 18:205–209
Dreyer ZE, Dowell BL, Chen H, Hawkins E, McClain KL (1991) Infection-associated hemophagocytic syndrome. Evidence for Epstein-Barr virus gene expression. Am J Pediatr Hematol Oncol 13(4):476–481
Favara BE (1991) Langerhans’ cell histiocytosis pathobiology and pathogenesis. Semin Oncol 18(1):3–7
Favara BE, Feller AC, Pauli M et al. (1997) A contemporary classification of histiocytic disorders. The WHO Committee on Histiocytic/Reticulum Cell Proliferations. Reclassification Working Group of the Histiocytic Society. Med Pediatr Oncol 29:157–166
Fernández-Latorre F, Menor-Serrano F, Alonso-Charterina S, Arenas-Jiménez J (2000) Langerhans’ cell histiocytosis of the temporal bone in pediatric patients: imaging and follow-up. AJR Am J Roentgenol 174:217–221
Gadner H, Heitger A, Grois N, Gatterer-Menz I, Ladisch S (1994) Treatment strategy for disseminated Langerhans cell histiocytosis. Med Ped Oncol 23:72–80
Geissmann F, Thomas C, Emile JF, Micheau M, Canioni D, Cerf-Bensussan N et al. (1996) Digestive tract involvement in Langerhans cell histiocytosis. The French Langerhans Cell Histiocytosis Study Group. J Pediatr 129(6):836–845
Ghanem I, Tolo VT, D’Ambra P, Malogalowkin MH (2003) Langerhans cell histiocytosis of bone in children and adolescents. J Pediatr Orthop 23(1):124–130
Glajchen N, Springfield DA, Lipton JM (1997) Langerhans’ cell histiocytosis of the synovium. AJR Am J Roentgenol 169(6):1751–1752
Goo HW, Yang DH, Ra YS, Song JS, Im HJ, Seo JJ, Ghim T, Moon HN (2006) Whole-body MRI of Langerhans cell histiocytosis: comparison with radiography and bone scintigraphy. Pediatr Radiol 36:1019–1031
Grundy P, Ellis R (1986) Histiocytosis X: a review of the etiology, pathology, staging, and therapy. Med Pediatr Oncol 14(1):45–50
Hand A (1893) Polyuria and tuberculosis. Arch Pediatric 10:673–675
Hoover KB, Rosenthal DI, Mankin H (2007) Langerhans cell histiocytosis. Skeletal Radiol 36:95–104
Hurley ME, O’Meara A, Fogarty E, Hayes R (2004) Langerhans’ cell histiocytosis of the clivus: case report and literature review. Pediatr Radiol 34:267–270
Jaffe R (2004) Liver involvement in the histiocytic disorders of childhood. Pediatr Dev Pathol 7(3):214–225
Junewick JJ, Fitzgerald NE (1999) The thymus in Langerhans’ cell histiocytosis. Pediatr Radiol 29:904–907
Kaste SC, Rodriguez-Galindo C, McCarville ME, Shulkin BL (2007) PET-CT in pediatric Langerhans cell histiocytosis. Pediatr Radiol 37:615–622
Kelly KM, Pritchard J (1994) Monoclonal antibody therapy in Langerhans cell histiocytosis – feasible and reasonable? Br J Cancer 70(Suppl.XXIII):54–55
Kilborn TN, Teh J, Goodman TR (2003) Paediatric manifestations of Langerhans cell histiocytosis: a review of the clinical and radiological findings. Clin Radiol 58(4):269–278
Kilpatrick SE, Wenger DE, Gilchrist GS, Shives TC, Wollan PC, Unni KK (1995) Langerhans’ cell histiocytosis (histiocytosis X) of bone. A clinicopathologic analysis of 263 pediatric and adult cases. Cancer 76(12):2471–2484
Kim M, Lyu C, Jin Y, Yoo H (1999) Langerhans’ cell histiocytosis as a cause of periportal abnormal signal intensity on MRI. Abdom Imaging 24(4):373–377
Komp DM (1987) Historical perspectives of Langerhans cell histiocytosis. Hematol Oncol Clin North Am 1:9–21
Kulwiec EL, Lynch DA, Aguayo SM, Schwarz MI, King TE Jr (1992) Imaging of pulmonary histiocytosis X. Radiographics 12(3):515–526
Kumar R, Balachandran S (1980) Relative roles of radionuclide scanning and radiographic imaging in eosinophilic granuloma. Clin Nucl Med.5:538–542
Lichtenstein L (1953) Histiocytosis X; integration of eosinophilic granuloma of bone, Letterer-Siwe disease, and Schuller-Christian disease as related manifestations of a single nosologic entity. AMA Arch Pathol 56(1):84–102
Maghnie M, Arico M, Villa A, Genovese E, Beluffi G, Severi F (1992) MR of the hypothalamic-pituitary axis in Langerhans cell histiocytosis. AJNR Am J Neuroradiol 13(5):1365–1371
Mahmoud HH, Wang WC, Murphy SB (1991) Cyclosporine therapy for advanced Langerhans cell histiocytosis. Blood 77:721–725
Minkov M, Grois N, Heitger A (2001) Response to initial treatment: an important prognostic predictor in multisystem Langerhans cell histiocytosis. J Pediatr 138:728–734
Moore AD, Godwin JD, Muller NL, Naidich DP, Hammar SP, Buschman DL et al. (1989) Pulmonary histiocytosis X: comparison of radiographic and CT findings. Radiology 172(1):249–254
Nesbit ME Jr, O’Leary M, Dehner LP, Ramsay NK (1981) The immune system and the histiocytosis syndromes. Am J Pediatr Hematol Oncol 3:141–149
Oberman HA (1961) A clinicopathologic study of 40 cases and review of the literature on eosinophilic granuloma of bone. Hand-Schuller-Christian disease and Letterer-Siwe disease. Pediatrics 28:307–327
Osband ME, Pochedly C (1987) Histiocytosis-X: an overview. Hematol Oncol Clin North Am 1(1):1–7
Ochsner SF (1966) Eosinophilic granuloma of bone: experience with 20 cases. AJR Am J Roentgenol 97:719–726
Parker JW, Lichtenstein L (1963) Severe hepatic involvement in chronic disseminated histiocytosis X. Report of a case with necropsy. Am J Clin Pathol 40:624–632
Prayer D, Grois N, Prosch H, Gadner H, Barkovich AJ (2004) MR imaging presentation of intracranial disease associated with Langerhans cell histiocytosis. AJNR Am J Neuroradiol 25(5):880–891
Refabert L, Rambaud C, Mamou-Mani T, Scheinmann P, de Blic J (1996) Cd1a-positive cells in bronchoalveolar lavage samples from children with Langerhans cell histiocytosis. J Pediatr 129(6):913–915
Schlesinger AE, Glass RB, Young S, Fernbach SK (1986) Case report 342: eosinophilic granuloma of the right iliac wing. Skeletal Radiol 15:57–59
Schmidt S, Eich G, Hanquinet S, Tschappeler H, Waibel P, Gudinchet F (2004) Extra-osseous involvement of Langerhans’ cell histiocytosis in children. Pediatr Radiol 34(4):313–321
Smets A, Mortele K, de Praeter G, Francois O, Benoit Y, Kunnen M (1997) Pulmonary and mediastinal lesions in children with Langerhans cell histiocytosis. Pediatr Radiol 27(11):873–876
Steinborn M, Wörtler K, Nathrath M, Schöniger M, Hahn H, Rummeny EJ (2008) Whole-body MRI in children with langerhans cell histiocytosis for the evaluation of the skeletal system. Rofo 180(7):646-53
Stull MA, Kransdorf MJ, Devaney KO (1992) Langerhans cell histiocytosis of bone. Radiographics 12(4):801–823
Sumner TE, Auringer ST, Preston AA (1993) Thymic calcifications in histiocytosis X. Pediatr Radiol 23:204–205
Weitzman S, Egeler RM (2008) Langerhans cell histiocytosis: update for the pediatrician. Curr Opin Pediatr 20(1):23–29
Willman C, Busque L, Griffith B et al. (1994) Langerhans cell histiocytosis (Histiocytosis X) – a clonal proliferative disease. N Engl J Med 331:154–160
Writing Group of the Histiocyte Society (1987) Histiocytosis syndromes in children. Lancet 1:208–209
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Johnson, K., Hobin, D. (2009). Langerhans Cell Histiocytosis. In: Davies, A., Sundaram, M., James, S. (eds) Imaging of Bone Tumors and Tumor-Like Lesions. Medical Radiology. Springer, Berlin, Heidelberg. https://doi.org/10.1007/978-3-540-77984-1_25
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