Skip to main page content
U.S. flag

An official website of the United States government

Dot gov

The .gov means it’s official.
Federal government websites often end in .gov or .mil. Before sharing sensitive information, make sure you’re on a federal government site.

Https

The site is secure.
The https:// ensures that you are connecting to the official website and that any information you provide is encrypted and transmitted securely.

Access keys NCBI Homepage MyNCBI Homepage Main Content Main Navigation
. 2020 Sep 29;9(10):3157.
doi: 10.3390/jcm9103157.

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

Paweł Sobczuk  1   2 , Paweł Teterycz  1 , Anna M Czarnecka  1   3 , Tomasz Świtaj  1 , Hanna Koseła-Paterczyk  1 , Katarzyna Kozak  1 , Sławomir Falkowski  1 , Piotr Rutkowski  1
Affiliations

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

Paweł Sobczuk et al. J Clin Med. .

Abstract

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.

Keywords: MPNST; chemotherapy; doxorubicin; ifosfamide; pazopanib; sarcoma.

PubMed Disclaimer

Conflict of interest statement

P.S. has received travel grants from MSD, Roche, and Pierre Fabre. P.T. has received honoraria and travel grants from Roche, Eli Lilly, Bayer, and Novartis. A.M.C. has received travel grants, payment for lectures, and consulting fees from BMS, MSD, Roche, and Novartis. P.R. has received honoraria for lectures from Novartis, Roche, Pfizer, BMS, Eli Lilly, and MSD and served as a member of the advisory board for Novartis, Merck, Amgen, Blueprint Medicine, Roche, BMS, and MSD. T.Ś. has received travel grants, payment for lectures, and consulting fees from BMS, MSD, Roche, and Novartis. H.K.P. has received honoraria and travel grants from MSD, Novartis, BMS, and Roche. K.K. has received honoraria and travel grants from BMS, MSD, Roche, and Novartis. S.F. declares no conflict of interest.

Figures

Figure A1
Figure A1
Progression-free survival stratified by chemotherapy regimens used: in the second line of treatment (a); and third line of treatment (b).
Figure 1
Figure 1
Progression-free survival stratified by chemotherapy regimens most commonly used in the first line. ADIC, doxorubicin + dacarbazine; AI, doxorubicin + ifosfamide; AP3, doxorubicin + cisplatin; EI, etoposide + ifosfamide; HD-IFO, high-dose ifosfamide.
Figure 2
Figure 2
Comparison of progression-free survival: for all systemic therapy groups irrespective of the line of treatment (a); and for various doxorubicin-based regimens (b). ADIC, doxorubicin + dacarbazine; AI, doxorubicin + ifosfamide; AP3, doxorubicin + cisplatin.
Figure 2
Figure 2
Comparison of progression-free survival: for all systemic therapy groups irrespective of the line of treatment (a); and for various doxorubicin-based regimens (b). ADIC, doxorubicin + dacarbazine; AI, doxorubicin + ifosfamide; AP3, doxorubicin + cisplatin.
See this image and copyright information in PMC

Similar articles

See all similar articles

Cited by

See all "Cited by" articles

References

    1. Stiller C.A., Trama A., Serraino D., Rossi S., Navarro C., Chirlaque M.D., Casali P.G., Group R.W. Descriptive epidemiology of sarcomas in Europe: Report from the RARECARE project. Eur. J. Cancer. 2013;49:684–695. doi: 10.1016/j.ejca.2012.09.011. - DOI - PubMed
    1. Fletcher C.D.M. Soft Tissue and Bone Tumours WHO Classification of Tumours. 5th ed. IARC; Lyon, France: 2013.
    1. Hirbe A.C., Gutmann D.H. The management of neurofibromatosis type 1-associated malignant peripheral nerve sheath tumors: Challenges, progress, and future prospects. Expert Opin. Orphan Drugs. 2017;5:623–631. doi: 10.1080/21678707.2017.1348294. - DOI
    1. Czarnecka A.M., Sobczuk P., Zdzienicki M., Spałek M., Rutkowski P. Malignant peripheral nerve sheath tumour (MPNST) Oncol. Clin. Pract. 2019;14:364–376. doi: 10.5603/OCP.2018.0050. - DOI
    1. Evans D.G.R. Malignant peripheral nerve sheath tumours in neurofibromatosis 1. J. Med. Genet. 2002;39:311–314. doi: 10.1136/jmg.39.5.311. - DOI - PMC - PubMed

LinkOut - more resources