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Treatment of dedifferentiated chordoma: a retrospective study from a large volume cancer center
- Source :
- J Neurooncol
- Publication Year :
- 2019
- Publisher :
- Springer Science and Business Media LLC, 2019.
-
Abstract
- OBJECTIVE: Dedifferentiated 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. METHODS: Overall, 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. RESULTS: DC 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). CONCLUSION: DC 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.
- Subjects :
- Male
Oncology
Cancer Research
medicine.medical_treatment
0302 clinical medicine
Neoplasm Metastasis
Child
Fisher's exact test
Aged, 80 and over
Medical record
Cell Differentiation
Middle Aged
Survival Rate
Treatment Outcome
Neurology
Child, Preschool
030220 oncology & carcinogenesis
Cohort
symbols
Female
Adult
medicine.medical_specialty
Adolescent
Article
Young Adult
03 medical and health sciences
symbols.namesake
Internal medicine
Chordoma
medicine
Humans
Survival analysis
Aged
Retrospective Studies
Spinal Neoplasms
Radiotherapy
business.industry
Infant, Newborn
Infant
Cancer
Retrospective cohort study
medicine.disease
Radiation therapy
Neurology (clinical)
Neoplasm Recurrence, Local
business
Hospitals, High-Volume
030217 neurology & neurosurgery
Follow-Up Studies
Subjects
Details
- ISSN :
- 15737373 and 0167594X
- Volume :
- 144
- Database :
- OpenAIRE
- Journal :
- Journal of Neuro-Oncology
- Accession number :
- edsair.doi.dedup.....c97f09dec645ee6e769eb5450159bff8