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Response to imatinib in villonodular pigmented synovitis (PVNS) resistant to nilotinib
BACKGROUND: Pigmented villonodular synovitis (PVNS) is a rare locally aggressive tumor. PVNS is characterized in most cases by a specific t(1;2) translocation, which fuses the colony stimulating factor-1 (CSF1) gene to the collagen type VIa3 (COL6A3) promoter thus leading through a paracrine effect...
Autores principales: | , , , , , , |
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Formato: | Online Artículo Texto |
Lenguaje: | English |
Publicado: |
BioMed Central
2013
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Materias: | |
Acceso en línea: | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3661378/ https://www.ncbi.nlm.nih.gov/pubmed/23668619 http://dx.doi.org/10.1186/2045-3329-3-8 |
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author | Stacchiotti, Silvia Crippa, Flavio Messina, Antonella Pilotti, Silvana Gronchi, Alessandro Blay, Jean Y Casali, Paolo G |
author_facet | Stacchiotti, Silvia Crippa, Flavio Messina, Antonella Pilotti, Silvana Gronchi, Alessandro Blay, Jean Y Casali, Paolo G |
author_sort | Stacchiotti, Silvia |
collection | PubMed |
description | BACKGROUND: Pigmented villonodular synovitis (PVNS) is a rare locally aggressive tumor. PVNS is characterized in most cases by a specific t(1;2) translocation, which fuses the colony stimulating factor-1 (CSF1) gene to the collagen type VIa3 (COL6A3) promoter thus leading through a paracrine effect to the attraction of non-neoplastic inflammatory cells expressing CSF1-receptor. Imatinib is a tirosin-kinase inhibitors (TKI) active against CSF1-receptor whose activity in naïve PVNS was already described. We report on two PVNS patients who responded to imatinib after failure to nilotinib, another CSF1-receptor inhibitor. METHODS: Since August 2012, 2 patients with progressive, locally advanced PVNS resistant to nilotinib (Patient 1: man, 34 years; Patient 2: woman, 24 years) have been treated with second-line imatinib 400 mg/day. Both patients are evaluable for response. RESULTS: Both patients are still on treatment (7 and 4 months). Patient 1 had a dimensional response by MRI after 2 months from starting imatinib, together with symptomatic improvement. In Patient 2 a metabolic response was detected by [18F]fluorodeoxyglucose–positron emission tomography (PET) at 6 weeks coupled with tumor shrinkage by MRI, and symptomatic improvement. CONCLUSIONS: Imatinib showed antitumor activity in 2 patients with nilotinib-resistant PVNS. This observation strengthen the idea that targeted agent with similar profile can give a different clinical result, as already described for gastrointestinal stromal tumor (GIST) patients treated with the same agents. Molecular studies are needed to clarify the biologic mechanism(s) underlying the response. |
format | Online Article Text |
id | pubmed-3661378 |
institution | National Center for Biotechnology Information |
language | English |
publishDate | 2013 |
publisher | BioMed Central |
record_format | MEDLINE/PubMed |
spelling | pubmed-36613782013-05-23 Response to imatinib in villonodular pigmented synovitis (PVNS) resistant to nilotinib Stacchiotti, Silvia Crippa, Flavio Messina, Antonella Pilotti, Silvana Gronchi, Alessandro Blay, Jean Y Casali, Paolo G Clin Sarcoma Res Case Report BACKGROUND: Pigmented villonodular synovitis (PVNS) is a rare locally aggressive tumor. PVNS is characterized in most cases by a specific t(1;2) translocation, which fuses the colony stimulating factor-1 (CSF1) gene to the collagen type VIa3 (COL6A3) promoter thus leading through a paracrine effect to the attraction of non-neoplastic inflammatory cells expressing CSF1-receptor. Imatinib is a tirosin-kinase inhibitors (TKI) active against CSF1-receptor whose activity in naïve PVNS was already described. We report on two PVNS patients who responded to imatinib after failure to nilotinib, another CSF1-receptor inhibitor. METHODS: Since August 2012, 2 patients with progressive, locally advanced PVNS resistant to nilotinib (Patient 1: man, 34 years; Patient 2: woman, 24 years) have been treated with second-line imatinib 400 mg/day. Both patients are evaluable for response. RESULTS: Both patients are still on treatment (7 and 4 months). Patient 1 had a dimensional response by MRI after 2 months from starting imatinib, together with symptomatic improvement. In Patient 2 a metabolic response was detected by [18F]fluorodeoxyglucose–positron emission tomography (PET) at 6 weeks coupled with tumor shrinkage by MRI, and symptomatic improvement. CONCLUSIONS: Imatinib showed antitumor activity in 2 patients with nilotinib-resistant PVNS. This observation strengthen the idea that targeted agent with similar profile can give a different clinical result, as already described for gastrointestinal stromal tumor (GIST) patients treated with the same agents. Molecular studies are needed to clarify the biologic mechanism(s) underlying the response. BioMed Central 2013-05-13 /pmc/articles/PMC3661378/ /pubmed/23668619 http://dx.doi.org/10.1186/2045-3329-3-8 Text en Copyright © 2013 Stacchiotti et al.; licensee BioMed Central Ltd. http://creativecommons.org/licenses/by/2.0 This is an Open Access article distributed under the terms of the Creative Commons Attribution License (http://creativecommons.org/licenses/by/2.0), which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited. |
spellingShingle | Case Report Stacchiotti, Silvia Crippa, Flavio Messina, Antonella Pilotti, Silvana Gronchi, Alessandro Blay, Jean Y Casali, Paolo G Response to imatinib in villonodular pigmented synovitis (PVNS) resistant to nilotinib |
title | Response to imatinib in villonodular pigmented synovitis (PVNS) resistant to nilotinib |
title_full | Response to imatinib in villonodular pigmented synovitis (PVNS) resistant to nilotinib |
title_fullStr | Response to imatinib in villonodular pigmented synovitis (PVNS) resistant to nilotinib |
title_full_unstemmed | Response to imatinib in villonodular pigmented synovitis (PVNS) resistant to nilotinib |
title_short | Response to imatinib in villonodular pigmented synovitis (PVNS) resistant to nilotinib |
title_sort | response to imatinib in villonodular pigmented synovitis (pvns) resistant to nilotinib |
topic | Case Report |
url | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3661378/ https://www.ncbi.nlm.nih.gov/pubmed/23668619 http://dx.doi.org/10.1186/2045-3329-3-8 |
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