Neoadjuvant Ifosfamide and Epirubicin in the Treatment of Malignant Peripheral Nerve Sheath Tumors

Overview

Journal Sarcoma

Publisher Wiley

Date 2017 May 27

PMID 28546782

Citations 11

Authors

Angela C Hirbe

Pippa F Cosper

Sonika Dahiya

Brian A Van Tine

Affiliations

Soon will be listed here.

Abstract

Malignant peripheral nerve sheath tumors (MPNSTs) are aggressive soft tissue sarcomas with poor overall survival. Response to chemotherapy has been debated for these tumors. We performed a retrospective analysis of the patients at our institution with a biopsy-proven diagnosis of MPNST that underwent neoadjuvant chemotherapy prior to surgery. We retrospectively identified five patients who received neoadjuvant chemotherapy with epirubicin and ifosfamide that demonstrated a 30% reduction in tumor growth and a 60% response rate by RECIST criteria. Additionally, a metabolic response was observed in all three patients who received serial PET scans during neoadjuvant treatment. The clinical benefit rate, which includes stable disease, was 100%. Our data suggest that MPNSTs do respond to epirubicin and ifosfamide based chemotherapy and prospective studies are warranted to further define the clinical benefit.

Citing Articles

The Multimodality Management of Malignant Peripheral Nerve Sheath Tumours.

Seres R, Hameed H, McCabe M, Russell D, Lee A Cancers (Basel). 2024; 16(19).

PMID: 39409887 PMC: 11475700. DOI: 10.3390/cancers16193266.

Malignant Peripheral Nerve Sheath Tumor, a Heterogeneous, Aggressive Cancer with Diverse Biomarkers and No Targeted Standard of Care: Review of the Literature and Ongoing Investigational Agents.

Somaiah N, Paudyal B, Winkler R, Van Tine B, Hirbe A Target Oncol. 2024; 19(5):665-678.

PMID: 38954182 PMC: 11392982. DOI: 10.1007/s11523-024-01078-5.

Contemporary Approach to Neurofibromatosis Type 1-Associated Malignant Peripheral Nerve Sheath Tumors.

Hirbe A, Dehner C, Dombi E, Eulo V, Gross A, Sundby T Am Soc Clin Oncol Educ Book. 2024; 44(3):e432242.

PMID: 38710002 PMC: 11656191. DOI: 10.1200/EDBK_432242.

Analysis of treatment sequence and outcomes in patients with relapsed malignant peripheral nerve sheath tumors.

Zhang L, Lemberg K, Calizo A, Varadhan R, Siegel A, Meyer C Neurooncol Adv. 2023; 5(1):vdad156.

PMID: 38130899 PMC: 10733661. DOI: 10.1093/noajnl/vdad156.

Pigmented malignant peripheral nerve sheath tumor of the nail unit arising in a patient with neurofibromatosis type I.

Kromer C, Knackstedt T, Somach S JAAD Case Rep. 2023; 35:12-15.

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