Visualization of Orbital Involvement of Erdheim-Chester Disease on PET/CT

Beylergil, Volkan; Carrasquillo, Jorge A.; Hyman, David M.; Diamond, Eli L.

doi:10.1097/rlu.0000000000000452

Cited by 4 publications

(3 citation statements)

References 13 publications

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“…The importance of FDG PET/CT for assessing the extent of skeletal disease, deciding the site for biopsy, and evaluating treatment response is well known. [8][9][10] In this case, we demonstrated the role of FDG PET/CT in the differential diagnosis and the extent of central nervous system involvement in a patient presenting with neurological symptoms.…”

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confidence: 78%

Central Nervous System Complications of Erdheim-Chester Disease

et al. 2021

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Erdheim-Chester disease is an uncommon non-Langerhans cell histiocytosis. The diagnosis depends on imaging and histological criteria. The most common presentation is the multiple bone lesions with involvement of at least 1 more organ. We present the FDG PET/CT findings of a 19-year-old man who presented with central nervous system symptoms such as progressive polyuria, polydipsia, gait disturbances, and eye pain and was later diagnosed with Erdheim-Chester disease.

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confidence: 78%

Central Nervous System Complications of Erdheim-Chester Disease

et al. 2021

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“…Space-occupying orbital ECD, 8 which is often bilateral and manifests as proptosis, is found in 27% of ECD patients 20 . Occasionally it may be resistant to treatment, requiring surgical debulking.…”

Section: Discussionmentioning

confidence: 99%

Choroidal mass as the first presentation of Erdheim-Chester disease

Pichi

2019

American Journal of Ophthalmology Case Reports

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Purpose To describe a choroidal mass that proved to be histiocytic choroidal infiltration in Erdheim-Chester disease. Observations A 54-years-old Caucasian male presented to our Retina Clinic with a suspect of choroidal melanoma in the left eye. Dilated fundus exam of the left eye showed a yellow-grey lesion along the inferior arcade, with sub-retinal fluid clinically visible. Enhanced depth imaging-OCT (EDI-OCT) showed a dome-shaped choroidal lesion with hyperreflective exudation present between the inner retina and the retinal pigment epithelium (RPE). On fundus autofluorescence the lesion appeared to have a diffuse speckled hyper-autofluorescent pattern secondary to the exudative subretinal material. On ultrasound, the lesion appeared hyper-echoic and dome-shaped, with a baseline thickness of 6.13 mm. Indocyanine green angiography (ICGA) was performed and showed hypocyanescence of the lesion from the early phases that persisted through the whole exam. Chest CT with contrast showed an abnormal, non-calcific, eccentric thickening of segments of the aorta (“coated aorta”) and PET an abnormally strong labeling of the distal ends of the long bones. An additional proximal tibial biopsy was performed to confirm the diagnosis on histology of Erdheim-Chester disease and the patient was started on oral prednisone. The choroidal mass progressively shrunk and the subretinal exudative material on top partially reabsorbed. Conclusions and importance Intraocular involvement in Erdheim-Chester disease is extremely rare but as a result of recent better awareness the number of new diagnosis is increasing. Erdheim-Chester disease should be considered in the differential of every choroidal mass.

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“…However, the rarity of the disease hinders the diagnosis and necessitates extensive imaging examinations, in which bone scans, CT and MRI of the central nervous system (CNS) are essential components. Beylergil et al reported that 18 F-FDG PET/CT may provide additional value in the assessment of orbital involvement in ECD [32]. Previous studies have reported that PET scanning had high specificity for brain ECD lesions and should be considered for the initial assessment and follow-up of ECD patients displaying pituitary involvement [23,33].…”

Section: Erdheim-chester Disease (Ecd)mentioning

confidence: 99%

Differential Diagnosis of Rare Diseases Involving Bilateral Lower Extremities with Similar 99mTc-MDP Bone Scan Patterns: Analysis of a Case Series

Sun

Shen

2022

Diagnostics

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Here, we reported a panel of rare diseases involving bilateral lower extremities with similar imaging patterns on 99mTc-MDP bone scans. Glucose-6-phosphate dehydrogenase deficiency (G-6PD deficiency), Gaucher disease (GD), steroid-induced osteonecrosis, progressive diaphyseal dysplasia (PDD), Erdheim–Chester disease (ECD) and Langerhans cell sarcoma (LCS) were included and imaging characteristics were analyzed. The rare properties of these diseases and mimicking features on 99mTc-MDP bone scans rendered differential diagnosis difficult but necessary. We believe that the rarely known imaging features of the reported diseases will undoubtedly help nuclear medicine physicians make differential diagnoses in clinical practice.

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Visualization of Orbital Involvement of Erdheim-Chester Disease on PET/CT

Cited by 4 publications

References 13 publications

Central Nervous System Complications of Erdheim-Chester Disease

Central Nervous System Complications of Erdheim-Chester Disease

Choroidal mass as the first presentation of Erdheim-Chester disease

Differential Diagnosis of Rare Diseases Involving Bilateral Lower Extremities with Similar 99mTc-MDP Bone Scan Patterns: Analysis of a Case Series

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