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KEY FACTS
Terminology
Imaging
Top Differential Diagnoses
Pathology
Clinical Issues
Diagnostic Checklist
TERMINOLOGY
Abbreviations
- Erdheim-Chester disease (ECD)
Definitions
- ECD is rare, non-Langerhans cell histiocytosis (non-LCH) that affects multiple organs characterized by xanthomatous infiltrates of foamy histiocytes
IMAGING
General Features
CT Findings
MR Findings
Imaging Recommendations
DIFFERENTIAL DIAGNOSIS
PATHOLOGY
General Features
Gross Pathologic & Surgical Features
Microscopic Features
CLINICAL ISSUES
Presentation
Demographics
Natural History & Prognosis
Treatment
DIAGNOSTIC CHECKLIST
Consider
Selected References
- Fernández-Eulate G et al: Vemurafenib as first-line therapy in BRAF-V600E-mutant Erdheim-Chester disease with CNS involvement. BMJ Case Rep. 12(11), 2019
- Wang F et al: Multisystemic imaging findings in Chinese patients with Erdheim-Chester disease. AJR Am J Roentgenol. 213(6):1179-86, 2019
- Wang Y et al: Neuroimaging features of CNS histiocytosis syndromes. Clin Imaging. 60(1):131-40, 2019
- Parks NE et al: Neuroradiologic manifestations of Erdheim-Chester disease. Neurol Clin Pract. 8(1):15-20, 2018
- Wagner KM et al: Intracranial Erdheim-Chester disease mimicking parafalcine meningioma: report of two cases and review of the literature. World Neurosurg. 110:365-70, 2018
- Young JR et al: 18F-FDG PET/CT in Erdheim-Chester disease: imaging findings and potential BRAF mutation biomarker. J Nucl Med. 59(5):774-79, 2018
- Pan Z et al: CNS Erdheim-Chester disease: a challenge to diagnose. J Neuropathol Exp Neurol. 76(12):986-96, 2017
- Cives M et al: Erdheim-Chester disease: a systematic review. Crit Rev Oncol Hematol. 95(1):1-11, 2015
- Gabbay LB et al: Histiocytosis: a review focusing on neuroimaging findings. Arq Neuropsiquiatr. 72(7):548-58, 2014
- Munoz J et al: Erdheim-Chester disease: characteristics and management. Mayo Clin Proc. 89(7):985-96, 2014
- Zaveri J et al: More than just Langerhans cell histiocytosis: a radiologic review of histiocytic disorders. Radiographics. 34(7):2008-24, 2014
- Lachenal F et al: Neurological manifestations and neuroradiological presentation of Erdheim-Chester disease: report of 6 cases and systematic review of the literature. J Neurol. 253(10):1267-77, 2006
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Tables
KEY FACTS
Terminology
Imaging
Top Differential Diagnoses
Pathology
Clinical Issues
Diagnostic Checklist
TERMINOLOGY
Abbreviations
- Erdheim-Chester disease (ECD)
Definitions
- ECD is rare, non-Langerhans cell histiocytosis (non-LCH) that affects multiple organs characterized by xanthomatous infiltrates of foamy histiocytes
IMAGING
General Features
CT Findings
MR Findings
Imaging Recommendations
DIFFERENTIAL DIAGNOSIS
PATHOLOGY
General Features
Gross Pathologic & Surgical Features
Microscopic Features
CLINICAL ISSUES
Presentation
Demographics
Natural History & Prognosis
Treatment
DIAGNOSTIC CHECKLIST
Consider
Selected References
- Fernández-Eulate G et al: Vemurafenib as first-line therapy in BRAF-V600E-mutant Erdheim-Chester disease with CNS involvement. BMJ Case Rep. 12(11), 2019
- Wang F et al: Multisystemic imaging findings in Chinese patients with Erdheim-Chester disease. AJR Am J Roentgenol. 213(6):1179-86, 2019
- Wang Y et al: Neuroimaging features of CNS histiocytosis syndromes. Clin Imaging. 60(1):131-40, 2019
- Parks NE et al: Neuroradiologic manifestations of Erdheim-Chester disease. Neurol Clin Pract. 8(1):15-20, 2018
- Wagner KM et al: Intracranial Erdheim-Chester disease mimicking parafalcine meningioma: report of two cases and review of the literature. World Neurosurg. 110:365-70, 2018
- Young JR et al: 18F-FDG PET/CT in Erdheim-Chester disease: imaging findings and potential BRAF mutation biomarker. J Nucl Med. 59(5):774-79, 2018
- Pan Z et al: CNS Erdheim-Chester disease: a challenge to diagnose. J Neuropathol Exp Neurol. 76(12):986-96, 2017
- Cives M et al: Erdheim-Chester disease: a systematic review. Crit Rev Oncol Hematol. 95(1):1-11, 2015
- Gabbay LB et al: Histiocytosis: a review focusing on neuroimaging findings. Arq Neuropsiquiatr. 72(7):548-58, 2014
- Munoz J et al: Erdheim-Chester disease: characteristics and management. Mayo Clin Proc. 89(7):985-96, 2014
- Zaveri J et al: More than just Langerhans cell histiocytosis: a radiologic review of histiocytic disorders. Radiographics. 34(7):2008-24, 2014
- Lachenal F et al: Neurological manifestations and neuroradiological presentation of Erdheim-Chester disease: report of 6 cases and systematic review of the literature. J Neurol. 253(10):1267-77, 2006
