Chester-Erdheim disease: A neoplastic disorder

Chetritt, Jérôme; Paradis, Valérie; Dargère, Delphine; Adle‐Biassette, Homa; Maurage, Claude Alain; Jm, Mussini; Vital, Anne; Wechsler, J; Bédossa, Pierre

doi:10.1016/s0046-8177(99)90228-9

Cited by 107 publications

(77 citation statements)

References 20 publications

Supporting

Mentioning

Contrasting

Unclassified

Order By: Relevance

“…In these diseases, FDG uptake is believed to be related to the high density of activated macrophages present in inflammatory lesions. While there is ongoing debate regarding the exact origin of the histiocytes in ECD (25,26), these cells are of the monocyte/ macrophage/dendritic cell lineages (27,28). This may account for the observed uptake in other regions.…”

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

View full text Add to dashboard Cite

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.

show abstract

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

View full text Add to dashboard Cite

show abstract

“…This may support the reactive nature of the lesions. Chetritt et al (17) studied the clonality of the non-Langerhans' cell histiocytes in ECD using the HUMARA assay. Their results showed that the histiocytes in three patients were monoclonal, one was polyclonal, and one was homozygous for the gene and therefore was not informative.…”

Section: Discussionmentioning

confidence: 99%

“…All forms of Langerhans' cell histiocytosis were recently proven to be monoclonal using different X-linked polymorphic DNA probes, including the HUMARA assay, in lesional tissues (15,16). Few attempts had been made to determine the clonality of the non-Langerhans' cell histiocytes in ECD (17). The aim of the authors was to determine the clonality of these cells from both the bone marrow and pericardial biopsies in a 35-year-old woman with ECD, using the HUMARA assay.…”

mentioning

confidence: 99%

Erdheim-Chester Disease: Case Report, PCR-Based Analysis of Clonality, and Review of Literature

et al. 2002

View full text Add to dashboard Cite

Erdheim-Chester disease (ECD) is a rare, distinct clinicopathologic entity with nearly pathognomonic radiographic features. The lesions consist of lipidstoring CD68 (؉), CD1a (؊) non-Langerhans' cell histiocytes, either localized to the bone or involving multiple organ systems in the body. Whether these histiocytic proliferations represent monoclonal neoplastic populations or are part of a polyclonal reactive process is unclear. We present a case report of ECD in a 35-year-old African-American woman with a progressive course over 6 years. We investigated the clonality of the histiocytes using the HU-MARA assay on paraffin-embedded tissue sections but did not find any evidence that these cells represent a monoclonal population. In this report, the characteristics of ECD are reviewed, the genetic basis of the HUMARA assay is discussed, and our results in the context of other clonality investigations reported in the literature to date are summarized.

show abstract

“…13,27 The extent and distribution of the disease determine the clinical course. 18 The presence of widespread systemic disease appears to be an ominous prognostic feature.…”

Section: Prognosis and Surveillancementioning

confidence: 99%

Erdheim-Chester disease mimicking an intracranial trigeminal schwannoma: case report

Alimohamadi

Hartmann

Paternó

et al. 2015

PED

View full text Add to dashboard Cite

Erdheim-Chester disease (ECD) is non-Langerhans histiocytosis that can affect multiple organ systems. It usually affects middle-aged patients, and only a few reports of ECD in children appear in the literature. Central nervous system involvement is a common feature that usually occurs as infiltration of the hypothalamus-pituitary axis, cerebellum, and/or brainstem. Meningeal involvement occurs less commonly. In this article, the authors discuss a rare pediatric case of ECD presenting as an infiltrative mass of the trigeminal nerve and resembling the clinical and imaging features of a trigeminal schwannoma.

show abstract

Chester-Erdheim disease: A neoplastic disorder

Cited by 107 publications

References 20 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: Case Report, PCR-Based Analysis of Clonality, and Review of Literature

Erdheim-Chester disease mimicking an intracranial trigeminal schwannoma: case report

Contact Info

Product

Resources

About

Chester-Erdheim disease: A neoplastic disorder

Cited by 107 publications

References 20 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: Case Report, PCR-Based Analysis of Clonality, and Review of Literature

Erdheim-Chester disease mimicking an intracranial trigeminal schwannoma: case report

Contact Info

Product

Resources

About

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