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Erdheim-Chester disease: CT findings of thoracic involvement

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Abstract

Objective:

To retrospectively assess the association of mediastinal, cardiovascular and pleuropulmonary findings on chest CT of 40 patients with immunohistochemically and histologically proven Erdheim-Chester disease (ECD).

Methods:

The multidetector chest CT images of 40 ECD patients were reviewed in consensus by chest and cardiovascular radiologists.

Results:

Thirty-four (85%) patients had periaortic infiltration that extended around the aortic branches of 29 (73%). Perivascular infiltration extended into the cardiac sulci in 22 (55%) (p < 0.005). Infiltration involved the right atrium wall in 12 patients, associated with severe narrowing of the atrial lumen in 8. Pericardial effusion and/or thickening were observed in 24 (60%) patients. Lung involvement, seen in 22 (55%) patients, was associated with mediastinal infiltration (20; p < 0.005) and pleural thickening or effusion (16; p = 0.001); it consisted of smooth interlobular septa (21), subpleural thickening (13), poorly defined centrilobular nodular opacities (9), ground-glass opacities (8) and/or lung cysts (5).

Conclusion:

The detailed description of thoracic ECD involvement seen in these patients showed that infiltration into the mediastinal spaces including the pericardium, coronary sulci and right atrium is frequently associated with pleural and interstitial lung diseases.

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Abbreviations

ECD:

Erdheim-Chester disease

LCH:

Langerhans cell histiocytosis

References

  1. Veyssier-Belot C, Cacoub P, Caparros-Lefebvre D, Wechsler J, Brun B, Remy M, Wallaert B, Petit H, Grimaldi A, Wechsler B, Godeau P (1996) Erdheim-Chester disease. Clinical and radiologic characteristics of 59 cases. Medicine (Baltimore) 75:157–169

    Article  CAS  Google Scholar 

  2. Haroche J, Amoura Z, Dion E, Wechsler B, Costedoat-Chalumeau N, Cacoub P, Isnard R, Genereau T, Wechsler J, Weber N, Graef C, Cluzel P, Grenier P, Piette JC (2004) Cardiovascular involvement, an overlooked feature of Erdheim-Chester disease: report of six new cases and a literature review. Medicine (Baltimore) 83:371–392

    Article  Google Scholar 

  3. Allen TC, Chevez-Barrios P, Shetlar DJ, Cagle PT (2004) Pulmonary and ophthalmic involvement with Erdheim-Chester disease: a case report and review of the literature. Arch Pathol Lab Med 128:1428–1431

    PubMed  Google Scholar 

  4. Bassou D, El Kharras A, Amezyane TT, En Nouali H, Elbaaj M, Benameur M, Darbi A, Vaglio A, Corradi D, Maestri R, Callegari S, Buzio C, Salvarani C (2009) Cardiac erdheim-chester. Intern Med 48:83–84

    Article  PubMed  Google Scholar 

  5. Bourke SC, Nicholson AG, Gibson GJ (2003) Erdheim-Chester disease: pulmonary infiltration responding to cyclophosphamide and prednisolone. Thorax 58:1004–1005

    Article  CAS  PubMed  Google Scholar 

  6. Chew HC, Lee CH, Cheah FK, Lim ST, Loo CM (2009) Cardiac tumor and renal involvement in a nonsmoker with centrilobular pulmonary nodules. Chest 135:1102–1106

    Article  PubMed  Google Scholar 

  7. Chung JH, Park MS, Shin DH, Choe KO, Kim SK, Chang J, Kim YS (2005) Pulmonary involvement in Erdheim-Chester disease. Respirology 10:389–392

    Article  PubMed  Google Scholar 

  8. Dion E, Graef C, Haroche J, Renard-Penna R, Cluzel P, Wechsler B, Piette JC, Grenier PA (2004) Imaging of thoracoabdominal involvement in Erdheim-Chester disease. AJR Am J Roentgenol 183:1253–1260

    PubMed  Google Scholar 

  9. Egan AJ, Boardman LA, Tazelaar HD, Swensen SJ, Jett JR, Yousem SA, Myers JL (1999) Erdheim-Chester disease: clinical, radiologic, and histopathologic findings in five patients with interstitial lung disease. Am J Surg Pathol 23:17–26

    Article  CAS  PubMed  Google Scholar 

  10. Haroche J, Cluzel P, Toledano D, Montalescot G, Touitou D, Grenier PA, Piette JC, Amoura Z (2009) Images in cardiovascular medicine. Cardiac involvement in Erdheim-Chester disease: magnetic resonance and computed tomographic scan imaging in a monocentric series of 37 patients. Circulation 119:e597–e598

    Article  PubMed  Google Scholar 

  11. Kong PM, Pinheiro L, Kaw G, Sittampalam K, Teo CH (2007) Erdheim-Chester disease: a rare cause of interstitial lung disease. Singapore Med J 48:e57–e59

    CAS  PubMed  Google Scholar 

  12. Protopapadakis C, Antoniou KM, Nicholson AG, Voloudaki A, Tzanakis N, Karantanas A, Siafakas NM (2009) Erdheim-Chester disease: pulmonary presentation in a case with advanced systemic involvement. Respiration 77:337–340

    Article  PubMed  Google Scholar 

  13. Remy-Jardin M, Remy J, Gosselin B (1993) Pulmonary involvement in Erdheim-Chester disease: high-resolution CT findings. Eur Radiol 3:389–392

    Article  Google Scholar 

  14. Serratrice J, Granel B, De Roux C, Pellissier JF, Swiader L, Bartoli JM, Disdier P, Weiller PJ (2000) Coated aorta: a new sign of Erdheim-Chester disease. J Rheumatol 27:1550–1553

    CAS  PubMed  Google Scholar 

  15. Vaglio A, Corradi D, Maestri R, Callegari S, Buzio C, Salvarani C (2008) Pericarditis heralding Erdheim-Chester disease. Circulation 118:e511–e512

    Article  PubMed  Google Scholar 

  16. Wittenberg KH, Swensen SJ, Myers JL (2000) Pulmonary involvement with Erdheim-Chester disease: radiographic and CT findings. AJR Am J Roentgenol 174:1327–1331

    CAS  PubMed  Google Scholar 

  17. Fink MG, Levinson DJ, Brown NL, Sreekanth S, Sobel GW (1991) Erdheim-Chester disease. Case report with autopsy findings. Arch Pathol Lab Med 115:619–623

    CAS  PubMed  Google Scholar 

  18. Araoz PA, Eklund HE, Welch TJ, Breen JF (1999) CT and MR imaging of primary cardiac malignancies. Radiographics 19:1421–1434

    CAS  PubMed  Google Scholar 

  19. Keenan N, Davies S, Sheppard MN, Maceira A, Serino W, Mohiaddin RH (2006) Angiosarcoma of the right atrium: a diagnostic dilemma. Int J Cardiol 113:425–426

    Article  PubMed  Google Scholar 

  20. Rush WL, Andriko JA, Galateau-Salle F, Brambilla E, Brambilla C, Ziany-bey I, Rosado-de-Christenson ML, Travis WD (2000) Pulmonary pathology of Erdheim-Chester disease. Mod Pathol 13:747–754

    Article  CAS  PubMed  Google Scholar 

  21. Shamburek RD, Brewer HB Jr, Gochuico BR (2001) Erdheim-Chester disease: a rare multisystem histiocytic disorder associated with interstitial lung disease. Am J Med Sci 321:66–75

    Article  CAS  PubMed  Google Scholar 

  22. Brower AC, Worsham GF, Dudley AH (1984) Erdheim-Chester disease: a distinct lipoidosis or part of the spectrum of histiocytosis? Radiology 151:35–38

    CAS  PubMed  Google Scholar 

  23. Kenn W, Eck M, Allolio B, Jakob F, Illg A, Marx A, Mueller-Hermelink HK, Hahn D (2000) Erdheim-Chester disease: evidence for a disease entity different from Langerhans cell histiocytosis? Three cases with detailed radiological and immunohistochemical analysis. Hum Pathol 31:734–739

    Article  CAS  PubMed  Google Scholar 

  24. Devouassoux G, Lantuejoul S, Chatelain P, Brambilla E, Brambilla C (1998) Erdheim-Chester disease: a primary macrophage cell disorder. Am J Respir Crit Care Med 157:650–653

    CAS  PubMed  Google Scholar 

  25. Andrade VP, Nemer CC, Prezotti AN, Goulart WS (2004) Erdheim-Chester disease of the breast associated with Langerhans-cell histiocytosis of the hard palate. Virchows Arch 445:405–409

    Article  CAS  PubMed  Google Scholar 

  26. Kambouchner M, Colby TV, Domenge C, Battesti JP, Soler P, Tazi A (1997) Erdheim-Chester disease with prominent pulmonary involvement associated with eosinophilic granuloma of mandibular bone. Histopathology 30:353–358

    Article  CAS  PubMed  Google Scholar 

  27. Kerzl R, Eyerich K, Eberlein B, Hein R, Weichenmeier I, Behrendt H, Clemm C, Fend F, Mempel S, Waldt S, Ring J, Mempel M (2009) Parallel occurrence of Erdheim-Chester disease and eosinophilic granuloma in the same patient. J Eur Acad Dermatol Venereol 23:224–226

    Article  CAS  PubMed  Google Scholar 

  28. Furmanczyk PS, Bruckner JD, Gillespy T 3rd, Rubin BP (2007) An unusual case of Erdheim-Chester disease with features of Langerhans cell histiocytosis. Skeletal Radiol 36:885–889

    Article  PubMed  Google Scholar 

  29. Boralevi F, Leaute-Labreze C, Tison F, Bioulac-Sage P, Vital C, Delbrel X, Cony M, Geniaux M (1998) Langerhans-cell histiocytosis and Erdheim-Chester disease: probably not a fortuitous association. Ann Dermatol Vénéréol 125:335–338

    CAS  PubMed  Google Scholar 

  30. Favara BE, Jaffe R (1987) Pathology of Langerhans cell histiocytosis. Hematol Oncol Clin N Am 1:75–97

    CAS  Google Scholar 

  31. Dion E, Graef C, Miquel A, Haroche J, Wechsler B, Amoura Z, Zeitoun D, Grenier PA, Piette JC, Laredo JD (2006) Bone involvement in Erdheim-Chester disease: imaging findings including periostitis and partial epiphyseal involvement. Radiology 238:632–639

    Article  PubMed  Google Scholar 

  32. Dalinka MK, Turner ML, Thompson JJ, Lee RE (1982) Lipid granulomatosis of the ribs: focal Erdheim-Chester disease. Radiology 142:297–299

    CAS  PubMed  Google Scholar 

  33. Klieger MR, Schultz E, Elkowitz DE, Arlen M, Hajdu SI, Dalinka MK, Turner ML, Thompson JJ, Lee RE (2002) Erdheim-Chester disease: a unique presentation with multiple osteolytic lesions of the spine and pelvis that spared the appendicular skeleton. AJR Am J Roentgenol 178:429–432

    PubMed  Google Scholar 

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Authors and Affiliations

  1. Radiologie, Hôpital Pitié-Salpêtrière, Assistance Publique-Hôpitaux de Paris, Université Pierre et Marie Curie, Paris VI, Paris, France

    Anne-Laure Brun, Diane Touitou-Gottenberg, Dan Toledano, Philippe Cluzel, Catherine Beigelman-Aubry & Philippe A. Grenier

  2. Médecine Interne, Hôpital Pitié-Salpêtrière, Assistance Publique-Hôpitaux de Paris, Université Pierre et Marie Curie, Paris VI, Paris, France

    Julien Haroche, Jean-Charles Piette & Zahir Amoura

  3. Radiologie Polyvalente Diagnostique et Interventionnelle, 47/83, boulevard de l’Hôpital, 75651, Paris Cedex 13, France

    Philippe A. Grenier

Authors
  1. Anne-Laure Brun
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  2. Diane Touitou-Gottenberg
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  3. Julien Haroche
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  4. Dan Toledano
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  5. Philippe Cluzel
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  6. Catherine Beigelman-Aubry
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  7. Jean-Charles Piette
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  8. Zahir Amoura
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  9. Philippe A. Grenier
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Corresponding author

Correspondence to Philippe A. Grenier.

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Brun, AL., Touitou-Gottenberg, D., Haroche, J. et al. Erdheim-Chester disease: CT findings of thoracic involvement. Eur Radiol 20, 2579–2587 (2010). https://doi.org/10.1007/s00330-010-1830-7

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  • DOI: https://doi.org/10.1007/s00330-010-1830-7

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