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CC BY-NC-ND 4.0 · World J Nucl Med 2019; 18(02): 201-203
DOI: 10.4103/wjnm.wjnm_57_18
Case Report

A new role for fluorine-18-fluorodeoxyglucose positron-emission tomography/computed tomography in Erdheim-Chester disease

Ciska-Anne Van Keerberghen
1   Division of Nuclear Medicine and Molecular Imaging, University Hospitals Leuven
2   Department of Internal Medicine, St Elisabeth Hospital, Brussels
,
Antoine Harrouk
2   Department of Internal Medicine, St Elisabeth Hospital, Brussels
,
Luca Leone
2   Department of Internal Medicine, St Elisabeth Hospital, Brussels
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Abstract

Erdheim-Chester disease (ECD) is a rare form of non-Langerhans cell histiocytosis, with multisystem manifestation such as bone pain, being the most common presenting symptom, cardiovascular or central nervous system involvement, interstitial lung disease, skin and orbital lesions, adrenal enlargement, retroperitoneal fibrosis and renal impairment as well fever, and weight loss. The disease is challenging to diagnose due to its rarity and mimicry of other infiltrative processes. Technetium-99 m bone scintigraphy showing pathological bone activity in the long bones is highly suggestive of ECD. However, not all patients have bone complaints. Till now, fluorine-18-fluorodeoxyglucose positron-emission tomography/computed tomography (18F-FDG PET/CT) was especially used after histological diagnosis to determine disease activity and extent, as well as the evaluation of treatment response. With this case, we suggest an additional role for 18F-FDG PET/CT earlier on in the diagnosis workup as follows: detecting a possible biopsy site to establish the diagnosis of ECD especially in a clinical context without bone pain.

Keywords

Erdheim-Chester disease - non-Langerhans cell histiocytosis - normal bone scintigraphy - subcutaneous nodule

Financial support and sponsorship

Nil.




Publication History

Received: 00 00 2019

Accepted: 00 00 2019

Article published online:
22 April 2022

© 2019. Sociedade Brasileira de Neurocirurgia. This is an open access article published by Thieme under the terms of the Creative Commons Attribution-NonDerivative-NonCommercial License, permitting copying and reproduction so long as the original work is given appropriate credit. Contents may not be used for commecial purposes, or adapted, remixed, transformed or built upon. (https://creativecommons.org/licenses/by-nc-nd/4.0/)

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  • References

  • 1 Abdelfattah AM, Arnaout K, Tabbara IA. Erdheim-Chester disease: A comprehensive review. Anticancer Res 2014;34:3257-61.
  • 2 Cavalli G, Guglielmi B, Berti A, Campochiaro C, Sabbadini MG, Dagna L, et al. The multifaceted clinical presentations and manifestations of Erdheim-Chester disease: Comprehensive review of the literature and of 10 new cases. Ann Rheum Dis 2013;72:1691-5.
  • 3 Haroche J, Arnaud L, Cohen-Aubart F, Hervier B, Charlotte F, Emile JF, et al. Erdheim-Chester disease. Rheum Dis Clin North Am 2013;39:299-311.
  • 4 Dagna L, Girlanda S, Langheim S, Rizzo N, Bozzolo EP, Sabbadini MG, et al. Erdheim-Chester disease: Report on a case and new insights on its immunopathogenesis. Rheumatology (Oxford) 2010;49:1203-6.
  • 5 Haroche J, Charlotte F, Arnaud L, von Deimling A, Hélias-Rodzewicz Z, Hervier B, et al. High prevalence of BRAF V600E mutations in Erdheim-Chester disease but not in other non-langerhans cell histiocytoses. Blood 2012;120:2700-3.
  • 6 Haroche J, Cohen-Aubart F, Emile JF, Arnaud L, Maksud P, Charlotte F, et al. Dramatic efficacy of vemurafenib in both multisystemic and refractory Erdheim-Chester disease and langerhans cell histiocytosis harboring the BRAF V600E mutation. Blood 2013;121:1495-500.
  • 7 Chasset F, Barete S, Charlotte F, Cohen-Aubart F, Arnaud L, Le Pelletier F, et al. Cutaneous manifestations of Erdheim-Chester disease (ECD): Clinical, pathological, and molecular features in a monocentric series of 40 patients. J Am Acad Dermatol 2016;74:513-20.
  • 8 García-Gómez FJ, Acevedo-Báñez I, Martínez-Castillo R, Tirado-Hospital JL, Cuenca-Cuenca JI, Pachón-Garrudo VM, et al. The role of 18FDG, 18FDOPA PET/CT and 99mTc bone scintigraphy imaging in Erdheim-Chester disease. Eur J Radiol 2015;84:1586-92.
  • 9 Antunes C, Graça B, Donato P. Thoracic, abdominal and musculoskeletal involvement in Erdheim-Chester disease: CT, MR and PET imaging findings. Insights Imaging 2014;5:473-82.
  • 10 Adens A, Landy P, Terriou L, Baillet C, Beron A, Lambert M, et al. Usefulness of nuclear medicine in Erdheim-Chester disease: A Lille experience. Rev Med Interne 2017;38:235-42.