Erdheim-Chester disease and radiobiphosphonates bone scan: from a simple peripheral form to a rare axial location: A case report

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Published Nov 29, 2016
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Vol. 94 No. 6 (2016): Issue Jun 2016

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Salah Oueriagli Nabih

Abstract

Abstract:
The Erdheim-Chester disease is a rare form of non Langherans histiocytosis. It can be expressed by a multi systemic disease interesting: bone, lung, heart, brain, retro peritoneum, skin and retro-orbital tissue. The diagnosis is established under the radiological aspect, bone scan uptake and immunohistological profile. The bone lesions are almost constant and rather characteristic of bone scintigraphy. We related the case of a young female patient of 26 years old with Erdheim-Chester bone disease. Diagnosis was revealed by diabetes insipidus (nerve damage) and diffuses bones pain. Histological confrontation was obtained on a biopsy, guided by abnormalities described in bone scan. The evolution under interferon was marked by clinical and scintigraphic worsening. Through this clinical observation we will discuss the interest of bone scintigraphy exploration in the monitoring of this disease under a therapy most often not codified.

Keywords:

Erdheim-Chester disease, radiobiphosphonates bone scan, simple peripheral form, rare axial locations.

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References

  1. Sheu SY, Wenzel RR, Kersting C, Merten R, Otterbach F, Schmid KW. Erdheim-Chester disease: Case report with multisystemic manifestations including testes, thyroid, and lymph nodes, and a review of literature. J Clin Pathol 2004; 57: 1225-8.
  2. Veyssier-Belot C, Cacoub P, Caparros-Lefebvre D., et al. Erdheim-Chester Disease: Clinical and Radiologic Characteristics of 59 Cases. Medecine 75: 3; 157- 169. 1996.
  3. Gomez C., Diard F., Chateil JF. et al. Imagerie de la maladie d'Erdheim-Chester. J. Radiol 77 : 1213-1221. 1996.
  4. Cattin F., Runge M., Nagy N. et al. Cas n°5 des jfr 2004. J. Radiol. 86 : 527-530. 2005.
  5. Dion E, Graef C, Miquel A, Haroche J, Wechsler B, Amoura Z, et al. Bone involvement in Erdheim-Chester disease: imaging findings including peri-ostitis and partial epiphyseal involvement. Radiology 2006; 238: 632-9.
  6. Kenn W, Stäbler A, Zachoval R. et al. Erdheim-Chester disease: a case report and literature overview. Eur Radiol 9: 153-158. 1999.
  7. Weidauer S., Von Stuckrad-Barre S., Dettman E et al. Cerebral Erdheim-Chester disease: case report and review of the literature. Neuroradiology 45: 241-245. 2003.
  8. Chung JH, Park MS, Shin DH, Choe KO, Kim SK, Chang J, et al. Pulmonary involvement in Erdheim-Chester disease. Respirology 2005; 10: 389-92.
  9. Haroche J, Amoura Z, Dion E, Wechsler B, Costedoat-Chalumeau N, Cacoub P, et al. Cardiovascular involvement, an overlooked feature of Erdheim-Chester disease: report of six new cases and a literature review. Medicine (Baltimore) 2004; 83: 371- 92.
  10. Dion E, Graef C, Haroche J. et al. Imaging of Thoraco-abdominal Involvement in Erdheim-Chester Disease. AJR 183: 1253-1260. 2004.
  11. Rushing EJ, Kaplan KJ, Mena H, Sandberg GD, Koeller K, Bouffard JP. Erdheim-Chester disease of the brain: cytological features and differential diagnosis of a challenging case. Diagn Cytopathol 2004; 31: 420-2.
  12. Valmaggia C, Neuweiler J, Fretz C, Gottlob I. A case of Erdheim-Chester disease with orbital involvement. Arch Ophthalmol 1997; 115: 1467-8.
  13. Watermann DF, Kiesewetter F, Frosch PJ. Skin manifestations of Erdheim-Chester disease. Case report and review of the literature. Hautarzt 2001; 52:510-7.
  14. Al-Quran S, Reith J, Bradley J, Rimsza L. Erdheim-Chester disease: case report, PCR-based analysis of clonality, and review of literature. Mod Pathol 2002; 15: 666-72.
  15. Rosier R.N., Rosenberg A.E., Case 9-2000 - A 41-Year-Old man with Multiple Bony Lesions and Adjacent Soft-Tissue Masses. N Eng J Med 342(12): 875-883. 2000.
  16. Gotthardt M, Welcke U, Brandt D, Tontsch D, Barth PJ, Schaefer J, et al. The role of bone scintigraphy in patients with Erdheim-Chester disease. Clin Nucl Med 2000; 25: 414-20.
  17. Nunez R, Tronco GG, Rini JN, Hofman J, Amoashiy M, Bhuiya T, et al. Radionuclide bone imaging in Erdheim-Chester disease. Clin Nucl Med 2005; 30: 32-4.
  18. Paycha F, Richard B. Exploration scintigraphique du squelette. Radio-diagnostic squelette normal. Encycl Med Chir, 30-480-A-10. Paris: Editions Scientiï¬ques et Médicales Elsevier SAS; 2001. p. 37.
  19. Wimpissinger TF, Schernthaner G, Feichtinger H, Stackl W. Compression of kidneys in Erdheim-Chester disease of retroperitoneum: Open surgical approach. Urology 2005; 65: 798.
  20. Namwongprom S, Nunez R, Kim EE, Macapinlac HA. Tc-99m MDP bone scintigraphy and positron emission tomography/computed tomography (PET/CT) imaging in Erdheim-Chester disease. Clin Nucl Med 2007; 32:35-8
  21. Caoduro C, Ungureanu CM, Rudenko B, Angoue O, Blagosklonov O, Paycha F, et al. 18F-fluoride PET/CTaspect of an unusual case of ErdheimChester disease with histologic features of Langerhans cell histiocytosis. Clin Nucl Med 2013;3 8: 541-2.
  22. Raptis DA, Raptis CA, Jokerst C, Bhalla S. Erdheim-Chester disease with interatrial septum involvement. J Thorac Imaging 2012; 27: W105-7.
  23. Adam Z, Balsikova K, Krejci M, Pour L, Stepankova S, Svacina P, et al. Central diabetesinsipidusin adult patients-the firstsign of Langerhans cell histiocytosis and Erdheim-Chester disease. Three case studies and literature review. Vnitr Lek 2010;56:138-48.
  24. Arnaud L, Malek Z, Archambaud F, Kas A, Toledano D, Drier A, et al. 18F- fluorodeoxyglucose-positron emission tomography scanning is more useful in followup than in the initial assessment of patients with Erdheim- Chester disease. Arthritis Rheum 2009;60: 3128-38.
  25. L. M. Vija, G. Bera, J. Haroche, F. Cohen, P. Chaumet-Riffaud, A. Prigent, P. Maksud. Rôle de la TEP/TDM au 18FDG dans la caractérisation et le suivi thérapeutique des histiocytoses. Imagerie métabolique et fonctionnelle 2015; 39 357-370.