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

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...

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Autores principales: Jeon, Ikchan, Choi, Joon Hyuk
Formato: Online Artículo Texto
Lenguaje:English
Publicado: BioMed Central 2021
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7955615/
https://www.ncbi.nlm.nih.gov/pubmed/33711983
http://dx.doi.org/10.1186/s12891-021-04061-7
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author Jeon, Ikchan
Choi, Joon Hyuk
author_facet Jeon, Ikchan
Choi, Joon Hyuk
author_sort Jeon, Ikchan
collection PubMed
description 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 (18)F-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.
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spelling pubmed-79556152021-03-15 Isolated thoracic intramedullary Erdheim-Chester disease presenting with paraplegia: a case report and literature review Jeon, Ikchan Choi, Joon Hyuk BMC Musculoskelet Disord Case Report 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 (18)F-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. BioMed Central 2021-03-12 /pmc/articles/PMC7955615/ /pubmed/33711983 http://dx.doi.org/10.1186/s12891-021-04061-7 Text en © The Author(s) 2021 Open AccessThis article is licensed under a Creative Commons Attribution 4.0 International License, which permits use, sharing, adaptation, distribution and reproduction in any medium or format, as long as you give appropriate credit to the original author(s) and the source, provide a link to the Creative Commons licence, and indicate if changes were made. The images or other third party material in this article are included in the article's Creative Commons licence, unless indicated otherwise in a credit line to the material. If material is not included in the article's Creative Commons licence and your intended use is not permitted by statutory regulation or exceeds the permitted use, you will need to obtain permission directly from the copyright holder. To view a copy of this licence, visit http://creativecommons.org/licenses/by/4.0/. The Creative Commons Public Domain Dedication waiver (http://creativecommons.org/publicdomain/zero/1.0/) applies to the data made available in this article, unless otherwise stated in a credit line to the data.
spellingShingle Case Report
Jeon, Ikchan
Choi, Joon Hyuk
Isolated thoracic intramedullary Erdheim-Chester disease presenting with paraplegia: a case report and literature review
title Isolated thoracic intramedullary Erdheim-Chester disease presenting with paraplegia: a case report and literature review
title_full Isolated thoracic intramedullary Erdheim-Chester disease presenting with paraplegia: a case report and literature review
title_fullStr Isolated thoracic intramedullary Erdheim-Chester disease presenting with paraplegia: a case report and literature review
title_full_unstemmed Isolated thoracic intramedullary Erdheim-Chester disease presenting with paraplegia: a case report and literature review
title_short Isolated thoracic intramedullary Erdheim-Chester disease presenting with paraplegia: a case report and literature review
title_sort isolated thoracic intramedullary erdheim-chester disease presenting with paraplegia: a case report and literature review
topic Case Report
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7955615/
https://www.ncbi.nlm.nih.gov/pubmed/33711983
http://dx.doi.org/10.1186/s12891-021-04061-7
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