Erdheim-Chester Disease: Utility of 18F-FDG Positron Emission Tomography

Erdheim-Chester Disease (ECD) is a rare non Langerhans cell histiocytosis of unknown origin with multiorgan involvement. We report a case of a man who presented to us with haematuria, asthenia, fever, nausea and malleolar oedema. After computed tomography and magnetic resonance imaging, the patient...

Full description

Bibliographic Details
Main Authors: Angelina Cistaro, Maria Licari, Simone Margotti, Daniele Penna, Vincenzo Arena
Format: Article
Language:English
Published: JCDR Research and Publications Private Limited 2018-02-01
Series:Journal of Clinical and Diagnostic Research
Subjects:
Online Access:https://jcdr.net/articles/PDF/11165/31708_CE(RA1)_F(T)_PF1_(EK_SL)_PFA(MJ_AnG)%20%20(2)_PN(SL).pdf
_version_ 1831648604991782912
author Angelina Cistaro
Maria Licari
Simone Margotti
Daniele Penna
Vincenzo Arena
author_facet Angelina Cistaro
Maria Licari
Simone Margotti
Daniele Penna
Vincenzo Arena
author_sort Angelina Cistaro
collection DOAJ
description Erdheim-Chester Disease (ECD) is a rare non Langerhans cell histiocytosis of unknown origin with multiorgan involvement. We report a case of a man who presented to us with haematuria, asthenia, fever, nausea and malleolar oedema. After computed tomography and magnetic resonance imaging, the patient underwent a Positron Emission Tomography/Computed Tomography (PET/CT) with 18F-fluorodeoxyglucose (18F-FDG) that revealed multiple focal uptake in the skeleton and also visceral and vascular involvement. After a retroperitoneal biopsy, diagnosis of ECD was made. The treatment begun with steroids and after one month, a second 18F-FDG PET/CT was performed, highlighting a partial response. After five months of specific treatment with inhibitors of tyrosine kinases, a third 18F-FDG PET/CT was done showing a complete response. The 18F-FDG PET/CT allows assessing the extent of involvement in ECD, to detect the most easily accessible sites for diagnostic biopsy and to monitor disease activity and response to consolidated and new therapies.
first_indexed 2024-12-19T14:35:06Z
format Article
id doaj.art-0a6b93b781fd494595604d6cee5b973b
institution Directory Open Access Journal
issn 2249-782X
0973-709X
language English
last_indexed 2024-12-19T14:35:06Z
publishDate 2018-02-01
publisher JCDR Research and Publications Private Limited
record_format Article
series Journal of Clinical and Diagnostic Research
spelling doaj.art-0a6b93b781fd494595604d6cee5b973b2022-12-21T20:17:18ZengJCDR Research and Publications Private LimitedJournal of Clinical and Diagnostic Research2249-782X0973-709X2018-02-01122TD01TD0210.7860/JCDR/2018/31708.11165Erdheim-Chester Disease: Utility of 18F-FDG Positron Emission TomographyAngelina Cistaro0Maria Licari1Simone Margotti2Daniele Penna3Vincenzo Arena4Nuclear Physician, Coordinator of PET Paediatric AIMN Study InterGroup, Positron Emission Tomography Centre, IRMET, Affidea, Turin, Italy.Nuclear Physician, Department of Nuclear Medicine, ARNAS Civico Hospital, Palerm, Italy.Nuclear Physician, Positron Emission Tomography Centre, IRMET, Affidea, Turin, Italy.Nuclear Physician, Positron Emission Tomography Centre, IRMET, Affidea, Turin, Italy.Nuclear Physician, Positron Emission Tomography Centre, IRMET, Affidea, Turin, Italy.Erdheim-Chester Disease (ECD) is a rare non Langerhans cell histiocytosis of unknown origin with multiorgan involvement. We report a case of a man who presented to us with haematuria, asthenia, fever, nausea and malleolar oedema. After computed tomography and magnetic resonance imaging, the patient underwent a Positron Emission Tomography/Computed Tomography (PET/CT) with 18F-fluorodeoxyglucose (18F-FDG) that revealed multiple focal uptake in the skeleton and also visceral and vascular involvement. After a retroperitoneal biopsy, diagnosis of ECD was made. The treatment begun with steroids and after one month, a second 18F-FDG PET/CT was performed, highlighting a partial response. After five months of specific treatment with inhibitors of tyrosine kinases, a third 18F-FDG PET/CT was done showing a complete response. The 18F-FDG PET/CT allows assessing the extent of involvement in ECD, to detect the most easily accessible sites for diagnostic biopsy and to monitor disease activity and response to consolidated and new therapies.https://jcdr.net/articles/PDF/11165/31708_CE(RA1)_F(T)_PF1_(EK_SL)_PFA(MJ_AnG)%20%20(2)_PN(SL).pdffluorodeoxyglucosenon langerhans cell histiocytosisvemurafenib
spellingShingle Angelina Cistaro
Maria Licari
Simone Margotti
Daniele Penna
Vincenzo Arena
Erdheim-Chester Disease: Utility of 18F-FDG Positron Emission Tomography
Journal of Clinical and Diagnostic Research
fluorodeoxyglucose
non langerhans cell histiocytosis
vemurafenib
title Erdheim-Chester Disease: Utility of 18F-FDG Positron Emission Tomography
title_full Erdheim-Chester Disease: Utility of 18F-FDG Positron Emission Tomography
title_fullStr Erdheim-Chester Disease: Utility of 18F-FDG Positron Emission Tomography
title_full_unstemmed Erdheim-Chester Disease: Utility of 18F-FDG Positron Emission Tomography
title_short Erdheim-Chester Disease: Utility of 18F-FDG Positron Emission Tomography
title_sort erdheim chester disease utility of 18f fdg positron emission tomography
topic fluorodeoxyglucose
non langerhans cell histiocytosis
vemurafenib
url https://jcdr.net/articles/PDF/11165/31708_CE(RA1)_F(T)_PF1_(EK_SL)_PFA(MJ_AnG)%20%20(2)_PN(SL).pdf
work_keys_str_mv AT angelinacistaro erdheimchesterdiseaseutilityof18ffdgpositronemissiontomography
AT marialicari erdheimchesterdiseaseutilityof18ffdgpositronemissiontomography
AT simonemargotti erdheimchesterdiseaseutilityof18ffdgpositronemissiontomography
AT danielepenna erdheimchesterdiseaseutilityof18ffdgpositronemissiontomography
AT vincenzoarena erdheimchesterdiseaseutilityof18ffdgpositronemissiontomography