Abstract
Langerhans’ cell histiocytosis (LCH) is manifested in a variety of ways, the most common being the eosinophilic granuloma, a localized, often solitary bone lesion that occurs predominantly in the pediatric age group. The hallmark of LCH is the proliferation and accumulation of a specific histiocyte: the Langerhans’ cell. In bone this may cause pain and adjacent soft-tissue swelling, but some lesions are asymptomatic. LCH can involve any bone, but most lesions occur in the skull (especially the calvarium and temporal bones), the pelvis, spine, mandible, ribs, and tubular bones. Imaging diagnosis of the disease in bone is first based on the plain radiographic appearance, which is usually a central destructive, aggressive-looking lesion. In the skull, the lesions develop in the diploic space, are lytic, and their edges may be beveled, scalloped or confluent (geographic), or show a “button sequestrum.” Vertebral body involvement usually causes collapse, resulting in vertebra plana. With significant recent improvements in the quality of gamma cameras, imaging techniques, and in studying children, bone scintigraphy at diagnosis and on follow-up usually reveals the sites of active disease, especially when the involvement is polyostotic. CT and MR imaging are very useful in providing detailed cross-sectional anatomic detail of the involved bone, including the bone marrow and the adjacent soft tissues. CT is better suited for demonstrating bone detail and MR imaging for bone marrow and soft-tissue involvement.
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Egeler RM, D’Angio GJ (1995) Langerhans cell histiocytosis. J Pediatr 127:1–11
Broadbent V, Egeler M, Nesbit ME (1994) Langerhans cell histiocytosis: clinical and epidemiological aspects. Br J Cancer 70:S11-S16
Favara BE, Feller AC, Pauli M (1997) Contemporary classification of histiocytic disorders. Med Pediatr Oncol 29:157–166
Berry DH, Gresik MV, Humphrey GB, et al (1986) Natural history of histiocytosis X: a pediatric oncology group study. Med Pediatr Oncol 14:1–5
Meyer JS, Harty MP, Mahboubi S, et al (1995) Langerhans cell histiocytosis: presentation and evolution of radiologic findings with clinical correlation. Radiographics 15:1135–1146
Arico M, Clementi R, Caselli D, et al (2003) Histiocytic disorders. Hematol J 4:171–179
Chu T, Jaffe R (1994) The normal Langerhans cell and the LCH cell. Br J Cancer 23:S4–S10
Stull MA, Kransdorf MJ, Devaney KO (1992) Langerhans cell histiocytosis of bone. Radiographics 12:801–823
Webb DKH (1999) Histiocytic syndromes. In: Lilleyman J, Hann I, Blanchette V (eds) Pediatric hematology, 2nd edn. Churchill Livingstone, London, pp 356–361
Willman CL (1994) Detection of clonal histiocytes in Langerhans cell histiocytosis: biology and clinical significance. Br J Cancer 23:S29–S33
Hutchison RE, Uner AH (1996) Pediatric neoplasia. Morphology and biology. In: Parham DM (ed) Tumors of the lymphoid organs. Lippincott-Raven, Philadelphia, pp 519–521
Fernandez-Latorre F, Menor-Serrano F, Alonso-Charterina S, et al (2000) Langerhans cell histiocytosis of the temporal bone in pediatric patients: imaging and follow-up. AJR 174:217–221
Ugarriza FL, Cabezudo JM, Porras LF, et al (2003) Solitary eosinophilic granuloma of the cervicothoracic junction causing neurological deficit. Br J Neurosurg 17:178–181
Puertas EB, Milani C, Chagas JC, et al (2003) Surgical treatment of eosinophilic granuloma in the thoracic spine in patients with neurological lesions. J Pediatr Orthop B 12:303–306
Kozlowski K, Grigor WG (1980) Probably congenital histiocytosis X with unusual radiographic finding in a 7-week-old infant. Pediatr Radiol 9:45–47
Seibert DJ (1994) Eosinophilic granuloma. Pediatric case of the day. AJR 162:1473–1474
Bollini G, Jouve JL, Gentet JC, et al (1991) Bone lesions in histiocytosis X. J Pediatr Orthop 11:469–477
Gardner DJ, Azouz EM (1988) Solitary lucent epiphyseal lesions in children. Skeletal Radiol 17:497–504
Usui M, Matsuno T, Kobayashi M, et al (1983) Eosinophilic granuloma of the growing epiphysis. Clin Orthop 176:201–205
Leeson MC, Smith A, Carter JR, et al (1985) Eosinophilic granuloma of bone in the growing epiphysis. J Pediatr Orthop 5:147–150
Hung PC, Wang HS, Jaing TH, et al (2003) From normal to abnormal MR findings within three weeks in a solitary pelvic Langerhans histiocytosis. Skeletal Radiol 32:481–484
Sartoris DJ, Parker BR (1984) Histiocytosis X: rate and pattern of resolution of osseous lesions. Radiology 152:679–684
Derbekyan V, Azouz EM (1984) Radiological case of the month: solitary costal eosinophilic granuloma. Am J Dis Child 138:885–886
Jabra AA, Fishman EK (1992) Eosinophilic granuloma simulating an aggressive rib neoplasm: CT evaluation. Pediatr Radiol 22:447–448
Mitnick JS, Pinto RS (1980) Computed tomography in the diagnosis of eosinophilic granuloma. J Comput Assist Tomogr 4:791–793
Tordeur M, Wybier M, Laporte JL, et al (2000) Button sequestrum in a case of localized Langerhans cell histiocytosis of the ilium: case report. Can Assoc Radiol J 51:90–92
Van Nieuwenhuyse JP, Clapuyt P, Malghem J, et al (1996) Radiographic skeletal survey and radionuclide bone scan in Langerhans cell histiocytosis of bone. Pediatr Radiol 26:734–738
Schaub T, Eissner D, Hahn K, et al (1983) Bone scanning in the detection and follow-up of skeletal lesions in histiocytosis X. Ann Radiol 26:407–410
Crone-Münzebrock W, Brassow F (1983) A comparison of radiographic and bone scan findings in histiocytosis X. Skeletal Radiol 9:170–173
Conway JJ (1996) Commentary. Pediatr Radiol 26:742–743
Shenouda NF, Azouz EM (1991) Case report 702: Langerhans cell histiocytosis of the skin, skull, and mandible with calcification in associated soft tissue masses. Skeletal Radiol 20:620–623
Geusens E, Brys P, Ghekiere J, et al (1998) Langerhans cell histiocytosis of the cervical spine: case report of an unusual location. Eur Radiol 8:1142–1144
Woertler K (2003) Benign bone tumors and tumor-like lesions: value of cross-sectional imaging. Eur Radiol 13:1820–1835
George JC, Buckwalter KA, Cohen MD, et al (1994) Langerhans cell histiocytosis of bone: MR imaging. Pediatr Radiol 24:29–32
Binkovitz LA, Olshefski RS, Adler BH (2003) Coincidence FDG-PET in the evaluation of Langerhans cell histiocytosis: preliminary findings. Pediatr Radiol 33:598–602
Schmidt S, Eich G, Hanquinet S, et al (2004) Extra-osseous involvement of Langerhans cell histiocytosis in children. Pediatr Radiol 34:313–321
Hindman BW, Thomas RD, Young LW, et al (1998) Langerhans cell histiocytosis: unusual skeletal manifestations observed in thirty-four cases. Skeletal Radiol 27:177–181
Taranath A, Onikul E (2004) Fluid-fluid levels in transcalvarial Langerhans cell histiocytosis. Pediatr Radiol 34:363
Oliveira M, Steinbok P, Wu J, et al (2003) Spontaneous resolution of calvarial eosinophilic granuloma in children. Pediatr Neurosurg 38:247–252
Muscolo DL, Slullitel G, Ranalletta M, et al (2003) Spontaneous remission of massive solitary eosinophilic granuloma of the femur. J Pediatr Orthop 23:763–765
Key SJ, O’Brien CJ, Silvester KC, et al (2004) Eosinophilic granuloma: resolution of maxillofacial bony lesions following minimal intervention. Report of three cases and a review of the literature. J Craniomaxillofac Surg 32:170–175
Cohen M, Zornoza J, Cangir A, et al (1980) Direct injection of methylprednisolone sodium succinate in the treatment of solitary eosinophilic granuloma of bone: a report of 9 cases. Radiology 136:289–293
Harris GJ, Woo KI (2003) Eosinophilic granuloma of the orbit: a paradox of aggressive destruction responsive to minimal intervention. Trans Am Ophthalmol Soc 101:93–103
Libicher M, Roeren TH, Tröger J (1995) Localized Langerhans cell histiocytosis of bone: treatment and follow-up in children. Pediatr Radiol 25:S134–S137
Gadner H, Heitger A, Grois N, et al (1994) DAL HX 83 study group. Treatment strategy for disseminated Langerhans cell histiocytosis. Med Ped Oncol 23:72–80
Gadner H, Grois N, Arico M, et al (2001) Histiocyte society: a randomized trial of treatment for multisystem Langerhans cell histiocytosis. J Pediatr 138:728–734. Erratum in: J Pediatr 139:170
Mahmoud HH, Wang WC, Murphy SB (1991) Cyclosporine therapy for advanced Langerhans cell histiocytosis. Blood 77:721–725
Kelly KM, Pritchard J (1994) Monoclonal antibody therapy in Langerhans cell histiocytosis—feasible and reasonable? Br J Cancer 23:S54–S55
Jakobson AM, Kreuger A, Hagberg H, et al (1987) Treatment of Langerhans cell histiocytosis with alpha-interferon. Lancet 2:1520–1521
Bellmunt J, Albanell J, Salud A, et al (1992) Interferon and disseminated Langerhans cell histiocytosis. Med Pediatr Oncol 20:336–337
Saven A, Burian C (1999) Cladribine activity in adult Langerhans-cell histiocytosis. Blood 93:4125–4130
Stine KC, Saylors RL, Williams LL, et al (1997) 2-Chlorodeoxyadenosine (2-CDA) for the treatment of refractory or recurrent Langerhans cell histiocytosis (LCH) in pediatric patients. Med Pediatr Oncol 29:288–292
Thomas L, Ducros B, Secchi T, et al (1993) Successful treatment of adult’s Langerhans cell histiocytosis with thalidomide: report of two cases and literature review. Arch Dermatol 129:1261–1264
Henter JI, Karlen J, Calming U, et al (2001) Successful treatment of Langerhans cell histiocytosis with etanercept. N Engl J Med 345:1577–1578
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We would like to thank Ms. Nora Del Pozo for secretarial assistance and Mr. Robert Burke for the illustrations.
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Azouz, E.M., Saigal, G., Rodriguez, M.M. et al. Langerhans’ cell histiocytosis: pathology, imaging and treatment of skeletal involvement. Pediatr Radiol 35, 103–115 (2005). https://doi.org/10.1007/s00247-004-1262-0
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DOI: https://doi.org/10.1007/s00247-004-1262-0