Skip to main content
Key Specialties
Cardiology Diabetology Endocrinology Gastroenterology Geriatrics and Gerontology Gynecology and Obstetrics Hematology Infectious Disease Internal Medicine Nephrology Neurology Oncology Pediatrics Respiratory Medicine Rheumatology Surgery
Congress Coverage
2024 ACC Congress 2023 ESMO Congress 2023 EASD Congress 2023 ERS Congress 2023 ESC Congress
Clinical Collections
Advances in Alzheimer’s

Keynote webinar | Spotlight on medication adherence

LIVE: Thursday 27th June 2024, 18:00-19:30 (CEST)
Join our expert panel to discover why you need to understand the drivers of non-adherence in your patients, and how you can optimize medication adherence in your clinics to drastically improve patient outcomes.
ADVANCED SEARCH
Log in
Top
European Journal of Nuclear Medicine and Molecular Imaging
Published in: European Journal of Nuclear Medicine and Molecular Imaging 4/2021

Open Access 01-04-2021 | Original Article

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

Authors: Julian Kirchner, Vaios Hatzoglou, Justin B. Buthorn, Dana Bossert, Allison M. Sigler, Anne S. Reiner, Gary A. Ulaner, Eli L. Diamond

Published in: European Journal of Nuclear Medicine and Molecular Imaging | Issue 4/2021

Login to get access

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
Literature
1.
Stempel JM, Bustamante Alvarez JG, Carpio AM, Mittal V, Dourado C. Erdheim-Chester disease, moving away from the orphan diseases: a case report. Respir Med Case Rep. 2017;20:55–8.PubMed
2.
Diamond EL, Dagna L, Hyman DM, et al. Consensus guidelines for the diagnosis and clinical management of Erdheim-Chester disease. Blood. 2014;124(4):483–92.CrossRef
3.
Haroche J, Arnaud L, Cohen-Aubart F, et al. Erdheim-Chester disease. Rheum Dis Clin N Am. 2013;39(2):299–311.CrossRef
4.
Veyssier-Belot C, Cacoub P, Caparros-Lefebvre D, et al. Erdheim-Chester disease. Clinical and radiologic characteristics of 59 cases. Medicine. 1996;75(3):157–69.CrossRef
5.
Haroche J, Charlotte F, Arnaud L, et al. High prevalence of BRAF V600E mutations in Erdheim-Chester disease but not in other non-Langerhans cell histiocytoses. 2012; 120(13): 2700–3.
6.
Diamond EL, Durham BH, Haroche J, et al. Diverse and targetable kinase alterations drive histiocytic neoplasms. Cancer Discov. 2016;6(2):154–65.CrossRef
7.
Emile JF, Diamond EL, Helias-Rodzewicz Z, et al. Recurrent RAS and PIK3CA mutations in Erdheim-Chester disease. Blood. 2014;124(19):3016–9.CrossRef
8.
Durham BH, Lopez Rodrigo E, Picarsic J, et al. Activating mutations in CSF1R and additional receptor tyrosine kinases in histiocytic neoplasms. Nat Med. 2019;25(12):1839–42.CrossRef
9.
Diamond EL, Subbiah V, Lockhart AC, et al. Vemurafenib for BRAF V600-mutant Erdheim-Chester disease and Langerhans cell histiocytosis: analysis of data from the histology-independent, phase 2, open-label VE-BASKET study. JAMA Oncol. 2018;4(3):384–8.CrossRef
10.
Diamond EL, Durham BH, Ulaner GA, et al. Efficacy of MEK inhibition in patients with histiocytic neoplasms. Nature 2019.
11.
Haroche J, Cohen-Aubart F, Emile JF, et al. Reproducible and sustained efficacy of targeted therapy with vemurafenib in patients with BRAF(V600E)-mutated Erdheim-Chester disease. J Clin Oncol : official journal of the American Society of Clinical Oncology. 2015;33(5):411–8.CrossRef
12.
Cohen Aubart F, Emile JF, Carrat F, et al. Targeted therapies in 54 patients with Erdheim-Chester disease, including follow-up after interruption (the LOVE study). Blood. 2017;130(11):1377–80.CrossRef
13.
Bhatia A, Ulaner G, Rampal R, et al. Single-agent dabrafenib for BRAF(V600E)-mutated histiocytosis. Haematologica. 2018;103(4):e177–e80.CrossRef
14.
Cohen-Aubart F, Emile JF, Carrat F, et al. Phenotypes and survival in Erdheim-Chester disease: results from a 165-patient cohort. Am J Hematol. 2018;93(5):E114–E7.CrossRef
15.
Diamond EL, Durham BH. Erdheim-Chester Disease. In: Abla O, Janka G, editors. Histiocytic disorders. Cham: Springer International Publishing; 2018. p. 313–38.CrossRef
16.
Diamond EL, Dagna L, Hyman DM, et al. Consensus guidelines for the diagnosis and clinical management of Erdheim-Chester disease. Blood 2014.
17.
Diamond EL, Reiner AS, Buthorn JJ, et al. A scale for patient-reported symptom assessment for patients with Erdheim-Chester disease. Blood Adv. 2019;3(7):934–8.CrossRef
18.
Roeser A, Cohen-Aubart F, Breillat P, et al. Autoimmunity associated with Erdheim-Chester disease improves with BRAF/MEK inhibitors. Haematologica. 2019;104(11):e502–e5.CrossRef
19.
Papo M, Diamond EL, Cohen-Aubart F, et al. High prevalence of myeloid neoplasms in adults with non-Langerhans cell histiocytosis. Blood. 2017;130(8):1007–13.CrossRef
20.
Garcia-Gomez FJ, Acevedo-Banez I, Martinez-Castillo R, et al. The role of 18FDG, 18FDOPA PET/CT and 99mTc bone scintigraphy imaging in Erdheim-Chester disease. Eur J Radiol. 2015;84(8):1586–92.CrossRef
21.
Sioka C, Estrada-Veras J, Maric I, Gahl WA, Chen CC. FDG PET images in a patient with Erdheim-Chester disease. Clin Nucl Med. 2014;39(2):170–7.CrossRef
22.
Young JR, Johnson GB, Murphy RC, Go RS, Broski SM. (18)F-FDG PET/CT in Erdheim-Chester disease: imaging findings and potential BRAF mutation biomarker. J Nucl Med : official publication, Society of Nuclear Medicine. 2018;59(5):774–9.CrossRef
23.
Arnaud L, Malek Z, Archambaud F, et al. 18F-fluorodeoxyglucose-positron emission tomography scanning is more useful in followup than in the initial assessment of patients with Erdheim-Chester disease. Arthritis Rheum. 2009;60(10):3128–38.CrossRef
24.
Diamond EL, Durham BH, Ulaner GA, et al. Efficacy of MEK inhibition in patients with histiocytic neoplasms. Nature. 2019;567(7749):521–4.CrossRef
25.
Eisenhauer EA, Therasse P, Bogaerts J, et al. New response evaluation criteria in solid tumours: revised RECIST guideline (version 1.1). Eur J Cancer. 2009;45(2):228–47.CrossRef
26.
Haroche J, Cohen Aubart F, Amoura Z. Erdheim-Chester disease. Blood 2020.
27.
Estrada-Veras JI, O'Brien KJ, Boyd LC, et al. The clinical spectrum of Erdheim-Chester disease: an observational cohort study. Blood Adv. 2017;1(6):357–66.CrossRef
28.
Cives M, Simone V, Rizzo FM, et al. Erdheim-Chester disease: a systematic review. Crit Rev Oncol Hematol. 2015;95(1):1–11.CrossRef
29.
Ulaner GA, Saura C, Piha-Paul SA, et al. Impact of FDG PET imaging for expanding patient eligibility and measuring treatment response in a genome-driven basket trial of the pan-HER kinase inhibitor, neratinib. Clin Cancer Res : an official journal of the American Association for Cancer Research. 2019;25(24):7381–7.CrossRef
30.
Goyal G, Heaney ML, Collin M, et al. Erdheim-Chester disease: consensus recommendations for the evaluation, diagnosis, and treatment in the molecular era. Blood. 2020.
31.
Abla O, Jacobsen E, Picarsic J, et al. Consensus recommendations for the diagnosis and clinical management of Rosai-Dorfman-Destombes disease. Blood. 2018;131(26):2877–90.CrossRef
Metadata
Title
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
Authors
Julian Kirchner
Vaios Hatzoglou
Justin B. Buthorn
Dana Bossert
Allison M. Sigler
Anne S. Reiner
Gary A. Ulaner
Eli L. Diamond
Publication date
01-04-2021
Publisher
Springer Berlin Heidelberg
Published in
European Journal of Nuclear Medicine and Molecular Imaging / Issue 4/2021
Print ISSN: 1619-7070
Electronic ISSN: 1619-7089
DOI
https://doi.org/10.1007/s00259-020-05047-8

Other articles of this Issue 4/2021

European Journal of Nuclear Medicine and Molecular Imaging 4/2021 Go to the issue

Guidelines

Procedural recommendations of cardiac PET/CT imaging: standardization in inflammatory-, infective-, infiltrative-, and innervation (4Is)-related cardiovascular diseases: a joint collaboration of the EACVI and the EANM

Short Communication

Safety and tolerability of 68Ga-NT-20.3, a radiopharmaceutical for targeting neurotensin receptors, in patients with pancreatic ductal adenocarcinoma: the first in-human use

Image of the Month

Diffuse multiple organs uptake of 99mTc-MDP on SPECT/CT

Editorial

[68Ga]Ga-tilmanocept PET/CT lymphoscintigraphy: a novel technique for sentinel lymph node imaging

Guidelines

Expert consensus on the safety prevention and control of nuclear medicine diagnosis and treatment during the outbreak of COVID-19 (1st edition): (translated from Chinese version)

Original Article

Optimal 68Ga-PSMA and 18F-PSMA PET window levelling for gross tumour volume delineation in primary prostate cancer