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Systemic Treatment for Advanced and Metastatic Malignant Peripheral Nerve Sheath Tumors—A Sarcoma Reference Center Experience

Malignant peripheral nerve sheath tumor (MPNST) is a rare type of soft tissue sarcomas. The localized disease is usually treated with surgery along with perioperative chemo- or radiotherapy. However, up to 70% of patients can develop distant metastases. The study aimed to evaluate the modes and outc...

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Autores principales: Sobczuk, Paweł, Teterycz, Paweł, Czarnecka, Anna M., Świtaj, Tomasz, Koseła-Paterczyk, Hanna, Kozak, Katarzyna, Falkowski, Sławomir, Rutkowski, Piotr
Formato: Online Artículo Texto
Lenguaje:English
Publicado: MDPI 2020
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7601777/
https://www.ncbi.nlm.nih.gov/pubmed/33003503
http://dx.doi.org/10.3390/jcm9103157
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author Sobczuk, Paweł
Teterycz, Paweł
Czarnecka, Anna M.
Świtaj, Tomasz
Koseła-Paterczyk, Hanna
Kozak, Katarzyna
Falkowski, Sławomir
Rutkowski, Piotr
author_facet Sobczuk, Paweł
Teterycz, Paweł
Czarnecka, Anna M.
Świtaj, Tomasz
Koseła-Paterczyk, Hanna
Kozak, Katarzyna
Falkowski, Sławomir
Rutkowski, Piotr
author_sort Sobczuk, Paweł
collection PubMed
description Malignant peripheral nerve sheath tumor (MPNST) is a rare type of soft tissue sarcomas. The localized disease is usually treated with surgery along with perioperative chemo- or radiotherapy. However, up to 70% of patients can develop distant metastases. The study aimed to evaluate the modes and outcomes of systemic treatment of patients with diagnosed MPNST treated in a reference center. In total, 115 patients (56 female and 59 male) diagnosed with MPNST and treated due to unresectable or metastatic disease during 2000–2019 were included in the retrospective analysis. Schemes of systemic therapy and the outcomes—progression-free survival (PFS) and overall survival (OS)—were evaluated. The median PFS in the first line was 3.9 months (95% CI 2.5–5.4). Doxorubicin-based regimens were the most commonly used in the first line (50.4% of patients). There were no significant differences in PFS between chemotherapy regimens most commonly used in the first line (p = 0.111). The median OS was 15.0 months (95% CI 11.0–19.0) and the one-year OS rate was 63%. MPNST are resistant to the majority of systemic therapies, resulting in poor survival in advanced settings. Chemotherapy with doxorubicin and ifosfamide is associated with the best response and longest PFS. Future studies and the development of novel treatment options are necessary for the improvement of treatment outcomes.
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spelling pubmed-76017772020-11-01 Systemic Treatment for Advanced and Metastatic Malignant Peripheral Nerve Sheath Tumors—A Sarcoma Reference Center Experience Sobczuk, Paweł Teterycz, Paweł Czarnecka, Anna M. Świtaj, Tomasz Koseła-Paterczyk, Hanna Kozak, Katarzyna Falkowski, Sławomir Rutkowski, Piotr J Clin Med Article Malignant peripheral nerve sheath tumor (MPNST) is a rare type of soft tissue sarcomas. The localized disease is usually treated with surgery along with perioperative chemo- or radiotherapy. However, up to 70% of patients can develop distant metastases. The study aimed to evaluate the modes and outcomes of systemic treatment of patients with diagnosed MPNST treated in a reference center. In total, 115 patients (56 female and 59 male) diagnosed with MPNST and treated due to unresectable or metastatic disease during 2000–2019 were included in the retrospective analysis. Schemes of systemic therapy and the outcomes—progression-free survival (PFS) and overall survival (OS)—were evaluated. The median PFS in the first line was 3.9 months (95% CI 2.5–5.4). Doxorubicin-based regimens were the most commonly used in the first line (50.4% of patients). There were no significant differences in PFS between chemotherapy regimens most commonly used in the first line (p = 0.111). The median OS was 15.0 months (95% CI 11.0–19.0) and the one-year OS rate was 63%. MPNST are resistant to the majority of systemic therapies, resulting in poor survival in advanced settings. Chemotherapy with doxorubicin and ifosfamide is associated with the best response and longest PFS. Future studies and the development of novel treatment options are necessary for the improvement of treatment outcomes. MDPI 2020-09-29 /pmc/articles/PMC7601777/ /pubmed/33003503 http://dx.doi.org/10.3390/jcm9103157 Text en © 2020 by the authors. Licensee MDPI, Basel, Switzerland. This article is an open access article distributed under the terms and conditions of the Creative Commons Attribution (CC BY) license (http://creativecommons.org/licenses/by/4.0/).
spellingShingle Article
Sobczuk, Paweł
Teterycz, Paweł
Czarnecka, Anna M.
Świtaj, Tomasz
Koseła-Paterczyk, Hanna
Kozak, Katarzyna
Falkowski, Sławomir
Rutkowski, Piotr
Systemic Treatment for Advanced and Metastatic Malignant Peripheral Nerve Sheath Tumors—A Sarcoma Reference Center Experience
title Systemic Treatment for Advanced and Metastatic Malignant Peripheral Nerve Sheath Tumors—A Sarcoma Reference Center Experience
title_full Systemic Treatment for Advanced and Metastatic Malignant Peripheral Nerve Sheath Tumors—A Sarcoma Reference Center Experience
title_fullStr Systemic Treatment for Advanced and Metastatic Malignant Peripheral Nerve Sheath Tumors—A Sarcoma Reference Center Experience
title_full_unstemmed Systemic Treatment for Advanced and Metastatic Malignant Peripheral Nerve Sheath Tumors—A Sarcoma Reference Center Experience
title_short Systemic Treatment for Advanced and Metastatic Malignant Peripheral Nerve Sheath Tumors—A Sarcoma Reference Center Experience
title_sort systemic treatment for advanced and metastatic malignant peripheral nerve sheath tumors—a sarcoma reference center experience
topic Article
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7601777/
https://www.ncbi.nlm.nih.gov/pubmed/33003503
http://dx.doi.org/10.3390/jcm9103157
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