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Update on Tenosynovial Giant Cell Tumor, an Inflammatory Arthritis With Neoplastic Features
Rheumatoid arthritis (RA) is a chronic inflammatory disease that leads to joint destruction and bone erosion. Even if many treatments were developed with success in the last decades, some patients fail to respond, and disease chronicity is still a burden. Mechanisms involved in such resistance may i...
Autores principales: | , , |
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Formato: | Online Artículo Texto |
Lenguaje: | English |
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Frontiers Media S.A.
2022
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Acceso en línea: | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8899011/ https://www.ncbi.nlm.nih.gov/pubmed/35265077 http://dx.doi.org/10.3389/fimmu.2022.820046 |
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author | Robert, Marie Farese, Helena Miossec, Pierre |
author_facet | Robert, Marie Farese, Helena Miossec, Pierre |
author_sort | Robert, Marie |
collection | PubMed |
description | Rheumatoid arthritis (RA) is a chronic inflammatory disease that leads to joint destruction and bone erosion. Even if many treatments were developed with success in the last decades, some patients fail to respond, and disease chronicity is still a burden. Mechanisms involved in such resistance may include molecular changes in stromal cells. Other explanations can come from observations of tenosynovial giant cell tumor (TGCT), first considered as an inflammatory arthritis, but with unusual neoplastic features. TGCT leads to synovium hypertrophy and hyperplasia with hemosiderin deposition. It affects young adults, resulting in secondary osteoarthritis and increased morbidity. TGCT shows clinical, histological and genetic similarities with RA but affecting a single joint. However, the monoclonality of some synoviocytes, the presence of translocations and rare metastases also suggest a neoplastic disease, with some features common with sarcoma. TGCT is more probably in an intermediate situation between an inflammatory and a neoplastic process, with a main involvement of the proinflammatory cytokine CSF-1/CSF1R signaling axis. The key treatment option is surgery. New treatments, derived from the RA and sarcoma fields, are emerging. The tyrosine kinase inhibitor pexidartinib was recently FDA-approved as the first drug for severe TGCT where surgery is not an option. Options directly targeting the excessive proliferation of synoviocytes are at a preclinical stage. |
format | Online Article Text |
id | pubmed-8899011 |
institution | National Center for Biotechnology Information |
language | English |
publishDate | 2022 |
publisher | Frontiers Media S.A. |
record_format | MEDLINE/PubMed |
spelling | pubmed-88990112022-03-08 Update on Tenosynovial Giant Cell Tumor, an Inflammatory Arthritis With Neoplastic Features Robert, Marie Farese, Helena Miossec, Pierre Front Immunol Immunology Rheumatoid arthritis (RA) is a chronic inflammatory disease that leads to joint destruction and bone erosion. Even if many treatments were developed with success in the last decades, some patients fail to respond, and disease chronicity is still a burden. Mechanisms involved in such resistance may include molecular changes in stromal cells. Other explanations can come from observations of tenosynovial giant cell tumor (TGCT), first considered as an inflammatory arthritis, but with unusual neoplastic features. TGCT leads to synovium hypertrophy and hyperplasia with hemosiderin deposition. It affects young adults, resulting in secondary osteoarthritis and increased morbidity. TGCT shows clinical, histological and genetic similarities with RA but affecting a single joint. However, the monoclonality of some synoviocytes, the presence of translocations and rare metastases also suggest a neoplastic disease, with some features common with sarcoma. TGCT is more probably in an intermediate situation between an inflammatory and a neoplastic process, with a main involvement of the proinflammatory cytokine CSF-1/CSF1R signaling axis. The key treatment option is surgery. New treatments, derived from the RA and sarcoma fields, are emerging. The tyrosine kinase inhibitor pexidartinib was recently FDA-approved as the first drug for severe TGCT where surgery is not an option. Options directly targeting the excessive proliferation of synoviocytes are at a preclinical stage. Frontiers Media S.A. 2022-02-21 /pmc/articles/PMC8899011/ /pubmed/35265077 http://dx.doi.org/10.3389/fimmu.2022.820046 Text en Copyright © 2022 Robert, Farese and Miossec https://creativecommons.org/licenses/by/4.0/This is an open-access article distributed under the terms of the Creative Commons Attribution License (CC BY). The use, distribution or reproduction in other forums is permitted, provided the original author(s) and the copyright owner(s) are credited and that the original publication in this journal is cited, in accordance with accepted academic practice. No use, distribution or reproduction is permitted which does not comply with these terms. |
spellingShingle | Immunology Robert, Marie Farese, Helena Miossec, Pierre Update on Tenosynovial Giant Cell Tumor, an Inflammatory Arthritis With Neoplastic Features |
title | Update on Tenosynovial Giant Cell Tumor, an Inflammatory Arthritis With Neoplastic Features |
title_full | Update on Tenosynovial Giant Cell Tumor, an Inflammatory Arthritis With Neoplastic Features |
title_fullStr | Update on Tenosynovial Giant Cell Tumor, an Inflammatory Arthritis With Neoplastic Features |
title_full_unstemmed | Update on Tenosynovial Giant Cell Tumor, an Inflammatory Arthritis With Neoplastic Features |
title_short | Update on Tenosynovial Giant Cell Tumor, an Inflammatory Arthritis With Neoplastic Features |
title_sort | update on tenosynovial giant cell tumor, an inflammatory arthritis with neoplastic features |
topic | Immunology |
url | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8899011/ https://www.ncbi.nlm.nih.gov/pubmed/35265077 http://dx.doi.org/10.3389/fimmu.2022.820046 |
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