ArticleJournal of neuro-oncology2019
Treatment of dedifferentiated chordoma: a retrospective study from a large volume cancer center.
Article in Journal of neuro-oncology, 2019. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 12 papers.
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Who cites it
12 citing papers in PubMed, 25 citations in OpenAlex.
- Tazemetostat, an EZH2 inhibitor, in solid tumors harboring SWI/SNF alterations: a phase II basket study.Nature communications · 2026Trial
- TP53 mutations as drivers of chordoma progression and hallmarks of aggressive chordoma.Acta neuropathologica communications · 2025Article
- A clinical analysis of 19 cases of chordomas with poor differentiation treated using diverse therapeutic modalities.Discover oncology · 2025Article
- Mutational analysis of primary and advanced chordoma tissue using next-generation sequencing.Cancer · 2025Article
- The Association of Gender in the Management and Prognosis of Vertebral and Sacral Chordoma: A SEER Analysis.Journal of clinical medicine · 2025Article
- Intensity Modulated Carbon Ion Radiation Therapy Using Pencil Beam Scanning Technology for Patients With Unresectable Sacrococcygeal Chordoma.Advances in radiation oncology · 2024Article
- Chordoma: Genetics and Contemporary Management.International journal of molecular sciences · 2024Review
- Article
- The NIH pediatric/young adult chordoma clinic and natural history study: Making advances in a very rare tumor.Pediatric blood & cancer · 2023Article
- Intensity-modulated radiotherapy for the management of primary and recurrent chordomas: a retrospective long-term follow-up study.Reports of practical oncology and radiotherapy : journal of Greatpoland Cancer Center in Poznan and Polish Society of Radiation Oncology · 2023Article
- A chronicle review of new techniques that facilitate the understanding and development of optimal individualized therapeutic strategies for chordoma.Frontiers in oncology · 2022Review
- Clinicopathological and Prognostic Characteristics in Dedifferentiated/Poorly Differentiated Chordomas: A Pooled Analysis of Individual Patient Data From 58 Studies and Comparison With Conventional Chordomas.Frontiers in oncology · 2021Article
Corrections and comments
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Authors and funding
10 authors at 1 institution in 1 country.
Funding
Abstract
objectiveDedifferentiated chordomas (DC) are genetically and clinically distinct from conventional chordomas (CC), exhibiting frequent SMARCB1 alterations and a more aggressive clinical course. We compared treatment and outcomes of DC and CC patients in a retrospective cohort study from a single, large-volume cancer center.
methodsOverall, 11 DC patients were identified from 1994 to 2017 along with a cohort of 68 historical control patients with CC treated during the same time frame. Clinical variables and outcomes were collected from the medical record and Wilcoxon rank sum or Fisher exact tests were used to make comparisons between the two groups. Kaplan-Meier survival analysis and log-rank tests were used to compare DC and CC overall survival.
resultsDC demonstrated a bimodal age distribution at presentation (36% age 0-24; 64% age > 50). DC patients more commonly presented with metastatic disease than CC patients (36% vs. 3% p = 0.000). DC patients had significantly shorter time to local treatment failure after radiation therapy (11.1 months vs. 34.1 months, p = 0.000). The rate of distant metastasis following treatment was significantly higher in DC compared to CC (57% vs. 5%, p = 0.000). The median overall survival after diagnosis for DC was 20 months (95% CI 0-48 months) compared to 155 months (95% CI 94-216 months) for CC (p = 0.007).
conclusionDC patients exhibit significantly higher rates of both synchronous and metachronous metastases, as well as shorter overall survival rates compared to conventional chordoma. The relatively poor survival outcomes with conventional therapies indicate the need to study targeted therapies for the treatment of DC.
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