Bone Involvement in Erdheim-Chester Disease: Imaging Findings including Periostitis and Partial Epiphyseal Involvement

Dion, Élisabeth; Graef, Claire; Miquel, Anne; Haroche, Julien; Wechsler, B.; Amoura, Zahir; Zeitoun, Delphine; Greniér, Philippe; Piette, J.C.; Larédo, Jean-Denis

doi:10.1148/radiol.2382041525

Cited by 136 publications

(77 citation statements)

References 29 publications

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“…This uptake pattern is similar to that observed with 99m Tc bone scintigraphy. This is an important finding, since this typical pattern is very specific for ECD, and thus, it is highly evocative of this diagnosis (19). When compared with 99m Tc bone scintigraphy, the initial PET scan had moderate sensitivity (58.6%) but excellent specificity (100%).…”

Section: Discussionmentioning

confidence: 84%

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¹⁸F‐fluorodeoxyglucose–positron emission tomography scanning is more useful in followup than in the initial assessment of patients with Erdheim‐Chester disease

Arnaud¹,

Malek²,

Archambaud³

et al. 2009

Arthritis & Rheumatism

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Objective. Erdheim-Chester disease (ECD) is a rare form of non-Langerhans' cell histiocytosis. The aim of this study was to assess the value of whole-body scanning with 18 F-fluorodeoxyglucose-positron emission tomography (FDG-PET) in a large cohort of ECD patients from a single center.Methods. We retrospectively reviewed all PET scans performed on 31 patients with ECD who were referred to our department between 2005 and 2008. PET images were reviewed by 2 independent nuclear medicine specialist physicians and were compared with other imaging modalities performed within 15 days of each PET scan.Results. Thirty-one patients (10 women and 21 men; median age 59.5 years) underwent a total of 65 PET scans. Twenty-three patients (74%) were untreated at the time of the initial PET scan, whereas 30 of the 34 followup PET scans (88%) were performed in patients who were undergoing immunomodulatory therapy. Comparison of the initial and followup PET scans with other imaging modalities revealed that the sensitivity of PET scanning varied greatly among the different organs studied (range 4.3-100%), while the specificity remained high (range 69.2-100%). Followup PET scans were particularly helpful in assessing central nervous system (CNS) involvement, since the PET scan was able to detect an early therapeutic response of CNS lesions, even before magnetic resonance imaging showed a decrease in their size. PET scanning was also very helpful in evaluating the cardiovascular system, which is a major prognostic factor in ECD, by assessing the heart and the entire vascular tree during a single session.Conclusion. The results of our large, singlecenter, retrospective study suggest that the findings of a FDG-PET scan may be interesting in the initial assessment of patients with ECD, but its greater contribution is in followup of these patients.

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Section: Discussionmentioning

confidence: 84%

“…Both radiography and 99m Tc bone scintigraphy may reveal osteosclerosis of the long bones, which is a typical finding in ECD (19). Assessment of osseous involvement of ECD is mostly performed as part of the diagnosis process.…”

Section: Discussionmentioning

confidence: 99%

¹⁸F‐fluorodeoxyglucose–positron emission tomography scanning is more useful in followup than in the initial assessment of patients with Erdheim‐Chester disease

Arnaud¹,

Malek²,

Archambaud³

et al. 2009

Arthritis & Rheumatism

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“…Recently, positron-emission tomography (PET) with 18F-labeled fluorodeoxyglucose (PET-CT) has been used in place of bone CT scans [17,18]. In some cases, MRI of the long bones can be informative, because it may reveal epiphyseal involvement of the long bones and periostitis, which is not detected by X-rays [19]. MRI may also be valuable in the rare cases of ECD for which anomalies are not detected by bone CT scans.…”

Section: Epidemiologymentioning

confidence: 99%

Erdheim–Chester Disease

et al. 2014

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Erdheim-Chester disease (ECD) is a rare (approximately 500 known cases worldwide), non-inherited, nonLangerhans form of histiocytosis of unknown origin, first described in 1930. It is characterized by xanthomatous or xanthogranulomatous infiltration of tissues by foamy histiocytes, "lipid-laden" macrophages, or histiocytes, surrounded by fibrosis. Diagnosis of ECD involves the analysis of histiocytes in tissue biopsies: these are typically foamy and CD68+ CD1a− in ECD, whereas in Langerhans cell histiocytosis (LCH) they are CD68+ CD1a+. 99 Technetium bone scintigraphy revealing nearly constant tracer uptake by the long bones is highly suggestive of ECD, and a "hairy kidney" appearance on abdominal CT scan is observed in approximately half of ECD cases. Central nervous system involvement is a strong prognostic factor and an independent predictor of death in cases of ECD. Optimum initial therapy for ECD seems to be administration of interferon α (or pegylated interferon α), and prolonged treatment significantly improves survival; however, tolerance may be poor. Cases of ECD present with strong systemic immune activation, involving IFNα, IL-1/IL1-RA, IL-6, IL-12, and MCP-1, consistent with the systemic immune Th-1-oriented disturbance associated with the disease. More than half of ECD patients carry the BRAF V600E mutation, an activating mutation of the protooncogene BRAF. A small number of patients harboring this mutation and with severe multisystemic and refractory ECD have been treated with vemurafenib, a BRAF inhibitor, which was proved very beneficial.

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“…5 They also show an increased uptake of tracer on Technetium 99m bone scintigraphy. 4 These bone changes may be misdiagnosed as Paget's disease, lymphoma, sarcoidosis, metastases, or lipid storage diseases.…”

Section: Discussionmentioning

confidence: 96%

Erdheim-Chester Disease With Renal Involvement: A Case Report

Ganiyusufoglu¹,

Gökşenoğlu²,

Tunç³

et al. 2017

Arch Rheumatol

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Bone Involvement in Erdheim-Chester Disease: Imaging Findings including Periostitis and Partial Epiphyseal Involvement

Abstract: This series provides a detailed description of bone involvement in Erdheim-Chester disease. Periostitis and partial epiphyseal involvement of the long bones are also features of this disease. (c) RSNA, 2005.

Cited by 136 publications

References 29 publications

¹⁸F‐fluorodeoxyglucose–positron emission tomography scanning is more useful in followup than in the initial assessment of patients with Erdheim‐Chester disease

¹⁸F‐fluorodeoxyglucose–positron emission tomography scanning is more useful in followup than in the initial assessment of patients with Erdheim‐Chester disease

Erdheim–Chester Disease

Erdheim-Chester Disease With Renal Involvement: A Case Report

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Bone Involvement in Erdheim-Chester Disease: Imaging Findings including Periostitis and Partial Epiphyseal Involvement

Abstract: This series provides a detailed description of bone involvement in Erdheim-Chester disease. Periostitis and partial epiphyseal involvement of the long bones are also features of this disease. (c) RSNA, 2005.

Cited by 136 publications

References 29 publications

18F‐fluorodeoxyglucose–positron emission tomography scanning is more useful in followup than in the initial assessment of patients with Erdheim‐Chester disease

18F‐fluorodeoxyglucose–positron emission tomography scanning is more useful in followup than in the initial assessment of patients with Erdheim‐Chester disease

Erdheim–Chester Disease

Erdheim-Chester Disease With Renal Involvement: A Case Report

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¹⁸F‐fluorodeoxyglucose–positron emission tomography scanning is more useful in followup than in the initial assessment of patients with Erdheim‐Chester disease

¹⁸F‐fluorodeoxyglucose–positron emission tomography scanning is more useful in followup than in the initial assessment of patients with Erdheim‐Chester disease