Immunologic and Targeted Molecular Therapies for Chordomas: A Narrative Review

Overview

Journal J Clin Med

Specialty General Medicine

Date 2024 Oct 16

PMID 39407739

Authors

Regina Golding

Rami Abuqubo

Christopher J Pansa

Manish Bhatta

Vishal Shankar

Kyle Mani

Emily Kleinbart

Yaroslav Gelfand

Saikiran Murthy

Rafael De La Garza Ramos

Jonathan Krystal

Ananth Eleswarapu

Reza Yassari

Evan Mostafa

Mitchell S Fourman

Anne Schlumprecht

Affiliations

Soon will be listed here.

Abstract

Chordomas are rare sarcomas arising from notochordal tissue and occur most commonly in the spine. The standard of care for chordomas without evidence of metastatic disease generally consists of en bloc resection followed by adjuvant radiotherapy. However, long-term (20-year) survival rates are approximately 30%. Chordomas are generally considered as chemo resistant. Therefore, systemic therapies have rarely been employed. Novel immunotherapies, including antibody therapy and tumor vaccines, have shown promise in early trials, leading to extended progression-free survival and symptom relief. However, the outcomes of larger trials using these vectors are heterogeneous. The aim of this review is to summarize novel chordoma treatments in immune-targeted therapies. The current merits, trial outcomes, and toxicities of these novel immune and targeted therapies, including those targeting vascular endothelial growth factor receptor (VEGFR) targets and the epidermal growth factor receptor (EGFR), will be discussed.

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