18F-FDG PET/CT in Erdheim–Chester disease

AbstractErdheim–Chester disease is a rare disease with systemic non-Langerhans cell histiocytosis, the diagnosis of which with conventional imaging modalities is challenging. We describe a case of a 73-year-old woman who was referred with a progressive history of bilateral proptosis. The magnetic resonance imaging (MRI) orbit demonstrated bilateral orbital masses with optic nerve encasement. A subsequent 18F-FDG PET/CT scan showed multi-organ disease with involvement of the orbits, pericardium, aorta, pararenal fascia, and appendicular bones. Metabolically active, easily accessible areas were selected for CT-guided biopsy. The biopsy showed sheets of foamy histiocytes with the expression of CD 68 and CD 163 consistent with a diagnosis of Erdheim–Chester disease. The FDG PET/CT played a pivotal role in establishing the diagnosis with the assessment of disease extent and further guided in the targeted biopsy.

Download Full-text

Metabolic Resolution of Lytic Lesions in Erdheim-Chester Disease on 18F-FDG PET/CT

Clinical Nuclear Medicine ◽

10.1097/rlu.0000000000003118 ◽

2020 ◽

Vol 45 (12) ◽

pp. e514-e515

Author(s):

Divya Yadav ◽

Rachna Seth ◽

Shamim Ahmed Shamim ◽

Rakesh Kumar

Keyword(s):

Fdg Pet ◽

Erdheim Chester Disease ◽

Pet Ct ◽

Lytic Lesions ◽

18F Fdg ◽

Fdg Pet Ct ◽

Erdheim Chester ◽

Chester Disease

Download Full-text

Isolated thoracic intramedullary Erdheim-Chester disease presenting with paraplegia: a case report and literature review

BMC Musculoskeletal Disorders ◽

10.1186/s12891-021-04061-7 ◽

2021 ◽

Vol 22 (1) ◽

Author(s):

Ikchan Jeon ◽

Joon Hyuk Choi

Keyword(s):

Surgical Resection ◽

Fdg Pet ◽

Rare Case ◽

Systemic Disease ◽

Mass Lesion ◽

Erdheim Chester Disease ◽

Pet Ct ◽

Fdg Pet Ct ◽

Erdheim Chester ◽

Chester Disease

Abstract Background Erdheim-Chester disease (ECD) is a rare, idiopathic, systemic non-Langerhans cell histiocytosis involving long bone and visceral organs. Central nervous system (CNS) involvement is uncommon and most cases develop as a part of systemic disease. We present a rare case of variant ECD as an isolated intramedullary tumor. Case presentation A 75-year-old female patient with a medical history of diabetes and hypertension presented with sudden-onset flaccid paraparesis for 1 day. Neurological examination revealed grade 2–3 weakness in both legs, decreased deep tendon reflex, loss of anal tone, and numbness below T4. Leg weakness deteriorated to G1 before surgery. Preoperative magnetic resonance imaging (MRI) and 18F-fluorodeoxyglucose positron emission tomography/computed tomography (FDG-PET/CT) showed an intramedullary mass lesion at T2-T4 with no systemic lesion, which was heterogeneous enhancement pattern with cord swelling and edema from C7 to T6. Gross total removal was achieved for the white-gray-colored and soft-natured intramedullary mass lesion with an ill-defined boundary. Histological finding revealed benign histiocytic proliferation with foamy histiocytes and uniform nuclei. We concluded it as an isolated intramedullary ECD. The patient showed self-standing and walkable at 18-month with no evidence of recurrence and new lesion on spine MRI and whole-body FDG-PET/CT until sudden occurrence of unknown originated thoracic cord infarction. Conclusions We experienced an extremely rare case of isolated intramedullary ECD, which was controlled by surgical resection with no adjuvant therapy. Histological examination is the most important for final diagnosis, and careful serial follow-up after surgical resection is required to identify the recurrence and progression to systemic disease.

Download Full-text

18F-FDG PET/CT versus anatomic imaging for evaluating disease extent and clinical trial eligibility in Erdheim-Chester disease: results from 50 patients in a registry study

European Journal of Nuclear Medicine and Molecular Imaging ◽

10.1007/s00259-020-05047-8 ◽

2020 ◽

Author(s):

Julian Kirchner ◽

Vaios Hatzoglou ◽

Justin B. Buthorn ◽

Dana Bossert ◽

Allison M. Sigler ◽

...

Keyword(s):

Clinical Trial ◽

Fdg Pet ◽

Registry Study ◽

Disease Extent ◽

Erdheim Chester Disease ◽

Pet Ct ◽

Measurable Disease ◽

Fdg Pet Ct ◽

Erdheim Chester ◽

Chester Disease

Abstract Objectives The aim of this study was to [1] characterize distribution of Erdheim-Chester Disease (ECD) by 18F-FDG PET/CT and [2] determine the utility of metabolic (18F-FDG PET/CT) imaging versus anatomic imaging (CT or MRI) in evaluating ECD patients for clinical trial eligibility. Methods 18F-FDG PET/CT and corresponding CT or MRI studies for ECD patients enrolled in a prospective registry study were reviewed. Sites of disease were classified as [1] detectable by 18F-FDG PET only, CT/MRI only, or both and as [2] measurable by modified PERCIST (mPERCIST) only, RECIST only, or both. Descriptive analysis was performed and paired t test for between-group comparisons. Results Fifty patients were included (mean age 51.5 years; range 18–70 years). Three hundred thirty-three disease sites were detected among all imaging modalities, 188 (56%) by both 18F-FDG PET and CT/MRI, 67 (20%) by 18F-FDG PET only, 75 (23%) by MRI brain only, and 3 (1%) by CT only. Of 178 disease sites measurable by mPERCIST or RECIST, 40 (22%) were measurable by both criteria, 136 (76%) by mPERCIST only, and 2 (1%) by RECIST only. On the patient level, 17 (34%) had mPERCIST and RECIST measurable disease, 30 (60%) had mPERCIST measurable disease only, and 0 had RECIST measurable disease only (p < 0.0001). Conclusion Compared with anatomic imaging, 18F-FDG PET/CT augments evaluation of disease extent in ECD and increases identification of disease sites measurable by formal response criteria and therefore eligibility for clinical trials. Complementary organ-specific anatomic imaging offers the capacity to characterize sites of disease in greater anatomic detail. Trial registration ClinicalTrials.gov Identifier: NCT03329274

Download Full-text