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Langerhans cell histiocytosis of the shoulder girdle, pelvis and extremities: a review of radiographic and MRI features in 85 cases

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Abstract

Objectives

To describe the radiographic and MRI features of histologically proven Langerhans cell histiocytosis (LCH) of the bone.

Materials and methods

A retrospective review of the radiographic and MRI features of 85 histologically proven cases of skeletal LCH over a 12-year period. Clinical data recorded included age, gender and location. Radiographic features evaluated included Lodwick grading, cortical/periosteal response and matrix mineralisation. MRI features assessed included lesion size and T1-weighted signal intensity (T1W SI), nature of margin, hypointense rim, enhancement pattern, bone marrow and soft tissue oedema, soft tissue mass, fluid-fluid levels, the penumbra sign and the budding and bulging signs.

Results

The study included 85 patients, 54 males and 31 females with mean age of 13 years (range 1–76 years). The femur was the commonest bone involved (38.8%), followed by the scapula (9.4%), clavicle (8.2%), ilium (8.2%) and ischium (8.2%). The mean maximal lesion size was 40 mm (range 16–85 mm). The commonest radiographic appearance was of a lytic lesion with no appreciable sclerotic rim, an intact expanded cortex and either absent or laminated periosteal response. MRI demonstrated a hypointense rim (41.5%), the budding (31.7%) and bulging (36.6%) signs, eccentric extra-osseous mass (42.7%), prominent bone marrow (95.3%) and soft tissue oedema (84.1%). Rarer features included haemorrhage (2.4%), the penumbra sign (3.5%) and fluid-fluid levels (2.4%). Thirteen of 25 post-contrast studies showed peripheral/rim enhancement with central necrosis.

Conclusions

LCH classically presents as a moderately aggressive lytic bone lesion on radiography, with prominent reactive bone and soft tissue oedema being a characteristic feature on MRI.

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References

  1. Howarth DM, Gilchrist GS, Mullan BP, Wiseman GA, Edmonson JH, Schomberg PJ. Langerhans cell histiocytosis: diagnosis, natural history, management, and outcome. Cancer. 1999;85:2278–90.

    CAS  PubMed  Google Scholar 

  2. Demellawy DE, Young JL, Nanassy J, Chernetsova E, Nasr A. Langerhans cell histiocytosis: a comprehensive review. Pathology. 2015;47(4):294–301.

    PubMed  Google Scholar 

  3. Badalian-Very G, Vergilio JA, Degar BA, MacConaill LE, Brandner B, Calicchio ML, et al. Recurrent BRAF mutations in Langerhans cell histiocytosis. Blood. 2010;116(11):1919–23.

    CAS  PubMed  PubMed Central  Google Scholar 

  4. Mickelson MR, Bonfiglio M. Eosinophilic granuloma and its variations. OrthopClinNorth Am. 1977;8:933–45.

    CAS  Google Scholar 

  5. Resnick D. Lipidoses, histiocytoses, and hypenlipoproteinemias. In: Resnick D, Niwayama G, editors. Diagnosis ofbone and joint disorders. Vol 4. 2nd ed. Philadelphia: Saunders; 1988. p. 2429–39.

    Google Scholar 

  6. Huvos AG. Bone tumors. 2nd ed. Philadelphia: Saunders; 1991. p. 695–771.

    Google Scholar 

  7. Berry DH, Gresik M, Maybee D, Marcus R. Histiocytosis X in bone only. Med PediatrOncol. 1990;18:292–4.

    CAS  Google Scholar 

  8. Carstensen H, Ornvold K. The epidemiology of Langerhans cell histiocytosis in children in Denmark, 1975–1989. Med PediatrOncol. 1993;21:387–8.

    Google Scholar 

  9. Baumgartner I, von Hochstetter A, Baumert B, Luetolf U, Follath F. Langerhans’ cell histiocytosis in adults. Med PediatrOncol. 1997;28(1):9–14.

    CAS  Google Scholar 

  10. Venkatramani R, Rosenberg S, Indramohan G, Jeng M, Jubran R. An exploratory epidemiological study of Langerhans cell histiocytosis. Pediatr Blood Cancer. 2012;59(7):1324–6.

    PubMed  Google Scholar 

  11. Jezierska M, Stefanowicz J, Romanowicz G, Kosiak W, Lange M. Langerhans cell histiocytosis in children - a disease with many faces. Recent advances in pathogenesis, diagnostic examinations and treatment. Postepy Dermatol Alergol. 2018;35(1):6–17.

    PubMed  PubMed Central  Google Scholar 

  12. Khung S, Budzik JF, Amzallag-Bellenger E, et al. Skeletal involvement in Langerhans cell histiocytosis. Insights Imaging. 2013;4(5):569–79.

    PubMed  PubMed Central  Google Scholar 

  13. George JC, Buckwalter KA, Cohen MD, Edwards MK, Smith RR. Langerhans cell histiocytosis of bone: MR imaging. PediatrRadiol. 1994;24(1):29–32.

    CAS  Google Scholar 

  14. Kilborn TN, The J, Goodman TR. Paediatric manifestations of Langerhans cell histiocytosis: a review of the clinical and radiological findings. Clin Radiol. 2003;58(4):269–78.

    CAS  PubMed  Google Scholar 

  15. David R, Oria RA, Kumar R, Singleton EB, Lindell MM, Shirkhoda A, et al. Radiologic features of eosinophilic granuloma of bone. AJR Am J Roentgenol. 1989;153(5):1021–6.

    CAS  PubMed  Google Scholar 

  16. Samet J, Weinstein J, Fayad LM. MRI and clinical features of Langerhans cell histiocytosis (LCH) in the pelvis and extremities: can LCH really look like anything? Skelet Radiol. 2016;45:607–13.

    Google Scholar 

  17. De Schepper AM, Ramon F, Van Marck E. MR imaging of eosinophilic granuloma: report of 11 cases. Skelet Radiol. 1993;22(3):163–6.

    Google Scholar 

  18. Song YS, Lee IS, Yi JH, Cho K, Kim DK, Song JW. Radiologic findings of adult pelvis and appendicular skeletal Langerhans cell histiocytosis in nine patients. Skelet Radiol. 2011;40:1421–6.

    Google Scholar 

  19. Davies AM, Pikoulas C, Griffith J. MRI of eosinophilic granuloma. Eur J Radiol. 1994;18:205–9.

    CAS  PubMed  Google Scholar 

  20. McCarville MB, Chen JY, Coleman JL, Li Y, Li X, Adderson EE, et al. Distinguishing osteomyelitis from Ewing sarcoma on radiography and MRI. AJR Am J Roentgenol. 2015;205:640–51.

    PubMed  PubMed Central  Google Scholar 

  21. Henninger B, Glodny B, Rudisch A, Trieb T, Loizides A, Putzer D, et al. Ewing sarcoma versus osteomyelitis: differential diagnosis with magnetic resonance imaging. Skelet Radiol. 2013;42(8):1,097–104.

    CAS  Google Scholar 

  22. Kasalak O, Overbosch J, Adams HJA, Dammann A, Dierckx RAJO, Jutte PC, et al. Diagnostic value of MRI signs in differentiating Ewing sarcoma from osteomyelitis. Acta Radiol. 2019;60(2):204–12.

    PubMed  Google Scholar 

  23. Caracciolo JT, Temple HT, Letson GD, Kransdorf MJ. A modified Lodwick-Madewell grading system for the evaluation of lytic bone lesions. AJR Am J Roentgenol. 2016;207(1):150–6.

    PubMed  Google Scholar 

  24. James SL, Hughes RJ, Ali KE, Saifuddin A. MRI of bone marrow oedema associated with focal bone lesions. Clin Radiol. 2006;61(12):1003–9.

    CAS  PubMed  Google Scholar 

  25. Moon TY, Lee J, Lee IS, Choi KU, Chae JM, Kim JI, et al. MRI and histopathologic classification of Langerhans cell histiocytosis. Curr Med Imaging Rev. 2009;5:14–8.

    Google Scholar 

  26. Zaveri J, La Q, Yarmish G, Neuman J. More than just Langerhans cell histiocytosis: a radiologic review of histiocytic disorders. Radiographics. 2014;34:2008–24.

    PubMed  Google Scholar 

  27. Kaplan GR, Saifuddin A, Pringle JA, Noordeen MH, Mehta MH. Langerhans’ cell histiocytosis of the spine: use of MRI in guiding biopsy. Skelet Radiol. 1998;27:673–6.

    CAS  Google Scholar 

  28. Wang S, Zhang W, Na S, Zhang L, Lang Z. Langerhans cell histiocytosis of the clavicle: a case report and review of the literature. Medicine (Baltimore). 2014;93:e117.

    Google Scholar 

  29. Stull MA, Devaney KO, Kransdorf MJ. From the archives of the AFIP: Langerhans cell histiocytosis of bone. Radiographics. 1992;12:801–23.

    CAS  PubMed  Google Scholar 

  30. Usui M, Matsuno T, Kobayashi M, Yagi T, Sasaki T, Ishii S. Eosinophilic granuloma of the growing epiphysis: a case report and review of the literature. Clin Orthop. 1983;176:201–5.

    Google Scholar 

  31. Stern MB, Cassidy R, Mirra J. Eosinophilic granuloma of the proximal tibial epiphysis. Clin Orthop. 1976;118:153–6.

    Google Scholar 

  32. Hindman BW, Thomas RD, Young LW, Yu L. Langerhans cell histiocytosis: unusual skeletal manifestations observed in thirty-four cases. Skelet Radiol. 1998;27:177–81.

    CAS  Google Scholar 

  33. Murphey MD, Senchak LT, Mambalam PK, Logie CI, Klassen-Fischer MK, Kransdorf MJ. From the radiologic pathology archives: Ewing sarcoma family of tumors: radiologic-pathologic correlation. Radiographics. 2013;33(3):803–31.

    PubMed  Google Scholar 

  34. Lodwick GS. Radiographic diagnosis and grading of bone tumors, with comments on computer evaluation. Proc Natl Cancer Conf. 1964;5:369–80.

    CAS  PubMed  Google Scholar 

  35. Ragsdale BD, Madewell JE, Sweet DE. Radiologic and pathologic analysis of solitary bone lesions Part II: Periosteal reactions. Radiol Clin N Am. 1981;19:749–83.

    CAS  PubMed  Google Scholar 

  36. Rana RS, Wu JS, Eisenberg RL. Periosteal reaction. AJR AM J Roentenol. 2009;193(4):W259–72.

    Google Scholar 

  37. Resnick D, Kyriakos M, Greenway GD. Tumors and tumor like lesions of bone: imaging and pathology of specific lesions. In: Resnick D, editor. Diagnosis of bone and joint disorders. 4th ed. Philadelphia: Saunders; 2002. p. 4060–73.

    Google Scholar 

  38. Mueller DL, Grant RM, Riding MD, Coppes MJ. Cortical saucerization: an unusual imaging finding of Ewing sarcoma. AJR AM J Roentenol. 1994;163:401–3.

    CAS  Google Scholar 

  39. Reinus WR, Gilula LA. Radiology of Ewing’s sarcoma: Ewing’s sarcoma: intergroup Ewing’s sarcoma study (IESS). Radiographics. 1984;4:929–44.

    Google Scholar 

  40. Alyas F, James S, Davies A, Saifuddin A. The role of MR imaging in the diagnostic characterisation of appendicular bone tumours and tumour-like conditions. Eur Radiol. 2007;17(10):2675–86.

    CAS  PubMed  Google Scholar 

  41. Mirra JM. Histiocytoses. In: Mirra JM, Picci P, Gold RH, editors. Bone tumors: clinical, radiologic, and pathologic correlations. 2nd ed. Philadelphia: Lea &Febiger; 1989. p. 1021–60. 16.

    Google Scholar 

  42. Crim JR, Mirra JM, Eckhardt JJ, Seeger LL. Widespread inflammatory response to osteobtastoma: the flare phenomenon. Radiology. 1990;177:835–6.

    CAS  PubMed  Google Scholar 

  43. Beltran J, Aparisi F, Bonmati LM, Rosenberg ZS, Present D, Steiner GC. Eosinophilic granuloma: MRI manifestations. Skelet Radiol. 1993;22(3):157–61.

    CAS  Google Scholar 

  44. Kroon HM, Bloem JL, Holscher HC, Van der Woude HJ, Reijnierse M, Taminiau AH. MR imaging of edema accompanying benign and malignant bone tumors. Skelet Radiol. 1994;23:261–9.

    CAS  Google Scholar 

  45. Grimer RJ. Size matters for sarcomas. Ann R Coll Surg Engl. 2006;88(6):519.

    PubMed  PubMed Central  Google Scholar 

  46. Bloem JL, Reidsma II. Bone and soft tissue tumors of hip and pelvis. Eur J Radiol. 2012;81:3793–801.

    PubMed  Google Scholar 

  47. Albadr FB, Elgamal EA, Alorainy IA, Hassan HH. Fluid-fluid level in Langerhans cell histiocytosis of the skull. Neurosciences (Riyadh). 2006;11:47–9.

    Google Scholar 

  48. Krishna Varanasi VR, Leong MY, Tan AM, Derrick Lian WQ, Teo ELHJ. Langerhans cell histiocytosis: another cause of a fluid-fluid level within an appendicular bony lesion. BJR Case Rep. 2016;2(1):20150408.

    PubMed  PubMed Central  Google Scholar 

  49. Lomoro P, Simonetti I, Vinci G, Fichera V, Tarotto L, Trovato P, et al. Secondary aneurysmal bone cyst in Langerhans cell histiocytosis: case report, literature review. Eur J Radiol Open. 2019;6:97–100.

    CAS  PubMed  PubMed Central  Google Scholar 

  50. Wu C, Li K, Hei Y, Lan P, Mu X. MR imaging features of orbital Langerhans cell Histiocytosis. BMC Ophthalmol. 2019;19:263.

    PubMed  PubMed Central  Google Scholar 

  51. Grey AC, Davies AM, Mangham DC, Grimer RJ, Ritchie DA. The ‘penumbra sign’ on T1-weighted MR imaging in subacute osteomyelitis: frequency, cause and significance. Clin Radiol. 1998;53(8):587–92.

    CAS  PubMed  Google Scholar 

  52. Davies AM, Grimer R. The penumbra sign in subacute osteomyelitis. Eur Radiol. 2005;15:1268–70.

    CAS  PubMed  Google Scholar 

  53. Chang WF, Hsu YC, Wu YD, Kuo CL, Huang GS. Localized Langerhans cell histiocytosis masquerading as Brodie’s abscess in a 2-year-old child: a case report. EXCLI J. 2016;18(15):33–7.

    Google Scholar 

  54. Verstraete KL, Lang P. Bone and soft tissue tumors: the role of contrast agents for MR imaging. Eur J Radiol. 2000;34(3):229–46.

    CAS  PubMed  Google Scholar 

  55. Kransdorf MJ. The use of gadolinium in the MR evaluation of musculoskeletal tumors. Top Magn Reson Imaging. 1996;8(1):15–23.

    CAS  PubMed  Google Scholar 

  56. Kalus S, Saifuddin A. Whole-body MRI vs bone scintigraphy in the staging of Ewing sarcoma of bone: a 12-year single-institution review. Eur Radiol. 2019;29(10):5700–8.

    PubMed  Google Scholar 

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Authors and Affiliations

  1. Department of Medical Imaging, Guru Nanak Dev Superspeciality hospital, Goindwal Sahib Road, Taran Sahib, Punjab, 143401, India

    J Singh

  2. Department of Medical Imaging, Royal National Orthopaedic Hospital, Stanmore, Middlesex, HA7 4LP, UK

    R Rajakulasingam & A Saifuddin

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  1. J Singh
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Correspondence to R Rajakulasingam.

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The study was approved by the local Research and Innovation Centre of the Institute of Orthopaedics as well as the Outer North London Research Ethics Committee, with no requirement for informed patient consent.

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Singh, J., Rajakulasingam, R. & Saifuddin, A. Langerhans cell histiocytosis of the shoulder girdle, pelvis and extremities: a review of radiographic and MRI features in 85 cases. Skeletal Radiol 49, 1925–1937 (2020). https://doi.org/10.1007/s00256-020-03472-2

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