Evidence map›Paper›PMID 42274206›Full record

ArticleCancer reports (Hoboken, N.J.)2026

Systemic Treatment of Advanced Chordoma With Molecular Targeted Therapies: A Large Multicentre European Retrospective Case Series.

Astrid Lipplaa, Nadia Hindi, Pawel Teterycz, Robin J Young, Ioannis Boukonavis, Josefina Cruz, Javier Martinez-Trufero, Frank Speetjens, Wilco Peul, Robert J P van der Wal and 1 more

Abstract readMulticenter Study
In one paragraph

Article in Cancer reports (Hoboken, N.J.), 2026. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Not yet cited in PubMed.

0numbers the graph read from it
0cells of the map it votes in
0citing papers in PubMed
–field-weighted citation impact
1 · What the graph read from it

What it found

Each row is one number read from the abstract, on the scale the paper reported it, with its interval. Left of the dashed line favours the treatment, right favours the comparator. Under each row is the sentence it came from. New to these charts? A ten-minute tutorial.

The abstract states no effect estimate the extractor could read, or names no intervention and outcome on the map, so this paper lights no cell and moves no belief. It is still indexed, cited and linked below.

2 · The registry

The trial behind it

Trials whose registry record cites this paper, or whose number appears in the abstract. A trial that started after this paper was published is citing it as background, not reporting it.

Neither the registry nor the abstract names a trial number. If this is a trial report, that itself is worth knowing.

3 · Its place in the literature

Who cites it

0 citing papers in PubMed.

No citing paper in PubMed yet.

4 · The record

Corrections and comments

PubMed lists nothing against this paper. Absence here is not a guarantee, only a check that was made.

5 · Who and what money

Authors and funding

11 authors.

Astrid LipplaaDepartment of Medical Oncology, Leiden University Medical Center, Leiden, the Netherlands.ORCID 0000-0001-5400-1138
Nadia HindiDepartment of Oncology, Hospital Universitario Virgen del Rocío, Seville, Spain.
Pawel TeteryczDepartment of Soft Tissue/Bone Sarcoma and Melanoma, Maria Sklodowska-Curie Institute - Oncology Center, Warsaw, Poland.
Robin J YoungDepartment of Oncology, Weston Park Hospital, Sheffield, UK.
Ioannis BoukonavisDepartment of Oncology, Aristotle University of Thessaloniki, Thessaloniki, Greece.
Josefina CruzDepartment of Oncology, Hospital Universitario de Canarias, San Cristóbal de La Laguna, Spain.
Javier Martinez-TruferoDepartment of Oncology, Hospital Universitario Miguel Servet, Zaragoza, Spain.
Frank SpeetjensDepartment of Medical Oncology, Leiden University Medical Center, Leiden, the Netherlands.
Wilco PeulDepartment of Neurosurgery, Leiden University Medical Center, Leiden, the Netherlands.
Robert J P van der WalDepartment of Orthopaedic Surgery, Leiden University Medical Center, Leiden, the Netherlands.
Hans GelderblomDepartment of Medical Oncology, Leiden University Medical Center, Leiden, the Netherlands.ORCID 0000-0001-9270-8636

Funding

No grant is acknowledged in the PubMed record.

6 · The paper itself

Abstract

backgroundThis retrospective series reports outcomes and prognostic factors for advanced chordoma (AC) treatment with molecular targeted therapies (MTTs) in different treatment lines.

methodsThis is a retrospective series of 57 patients with AC treated between 2004 and 2023 at one of seven participating sarcoma centres. Demographics, previous treatment, treatment details and outcomes were recorded.

results57 patients were treated with 7 different MTTs. Treatment was received in first (n = 57), second (n = 16), third (n = 5) or fourth (n = 1) line. The most frequently administered agent in first line was imatinib (84.2%) and in second line imatinib + sirolimus (35.7%). Overall median progression free survival (PFS) and overall survival (OS) in first-line treatment was 6.5 (95% CI 4.0-9.0) and 29.5 months (95% CI 24.0-40.4) and in second-line 10.0 (95% CI 4.0-22.0) and 37.2 months (95% CI 9.4-45.9). Partial response according to RECIST 1.1 was seen in 5/79 treatments (6.3%). Dose reductions and interruptions were reported in 19.0% and 27.8% of treatments.

conclusionsPFS and response rates with these MTTs were in line with previous phase II trials and retrospective series. Although the efficacy does not meet the European Society of Medical Oncology (ESMO) Magnitude of Clinical Benefit Scale (MCBS) criteria for single arm studies in orphan diseases, MTTs are frequently used off-label due to the high unmet need and lack of other systemic treatment options. The toxicity profile and limited efficacy rate should be taken into account when counselling patients. Further research is needed to explore other systemic treatment options including (combinations with) immunotherapy.

Indexed as

Antineoplastic Combined Chemotherapy ProtocolsChordomaMolecular Targeted TherapyAdultAgedEuropeFemaleHumansImatinib MesylateMaleMiddle AgedPrognosisProgression-Free SurvivalProtein Kinase InhibitorsRetrospective StudiesSirolimusImatinib MesylateProtein Kinase InhibitorsSirolimuschordomamolecular targeted therapysystemic therapytyrosine kinase inhibitors

Identifiers

PMID42274206
PMCPMC13255151

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Read under generation 80e0d062 · epoch 390. Bibliography from PubMed, PubMed Central and OpenAlex; grants from NIH RePORTER; trial links from ClinicalTrials.gov; estimates, votes and beliefs from the OpenQuestion graph.