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Incidence and centralization of chordoma in the Netherlands: A nationwide study between 1991 and 2020
A Lipplaa1, R J P van der Wal2, A D G Krol3
1Department of Medical Oncology, Leiden University Medical Center, Leiden, the Netherlands.
Introduction:
Chordomas are rare malignant bone tumors arising in the axial skeleton, with an incidence of 0.3-0.88 per million inhabitants. We studied the annual incidence rate and centralization of treatment for chordoma in the Netherlands.
Methods:
We retrieved pathology excerpts from the PALGA nationwide Dutch Pathology Registry between 1991 and 2019 for patients with a chordoma to calculate incidence rates. From pathology reports we extracted patient age at diagnosis, sex, year of diagnosis, localization of primary tumor, histologic chordoma subtype (conventional including chondroid, poorly differentiated or dedifferentiated), center of diagnosis (bone tumor referral center (BTC) or other hospital), and partial identification of the BTCs.
Results:
A total of 420 individual chordoma patients were identified in the given time period. The incidence of chordoma increased from 0.593 per million inhabitants between 1991-1995 to 1.111 from 2015-2019 (P = 0.001). Median age at diagnosis was 63 years (range 1-95), 252 patients (60%) were male. The proportion of samples analyzed in a BTC either primarily or secondary, as a consultation, revision or referral, increased significantly from 29.3% to 84.4% (P < 0.001). Most primary and secondary samples were analyzed at the Leiden University Medical Center (LUMC, 54.4% and 57% respectively).
Conclusions:
This study shows an increase in the standardized incidence of pathology proven chordoma in the Netherlands. We observed an increase in samples being analysed in the specialized BTCs as well, which is in line with current guidelines and will hopefully lead to more accurate diagnoses and optimal treatment plans for chordoma patients in specialized treatment centers.
Insights
The incidence of chordoma, a rare bone cancer, has increased in the Netherlands. More patients are now treated at specialized bone tumor centers (BTCs) for better diagnosis and care.
Area of Science:
- Oncology
- Epidemiology
- Pathology
Background:
- Chordomas are rare malignant bone tumors originating in the axial skeleton.
- The incidence rate is approximately 0.3-0.88 per million inhabitants.
- This study investigates chordoma incidence and treatment centralization in the Netherlands.
Purpose of the Study:
- To determine the annual incidence rate of chordoma in the Netherlands.
- To assess the trend in treatment centralization for chordoma patients.
- To analyze the impact of specialized bone tumor centers (BTCs) on chordoma diagnosis and treatment.
Main Methods:
- Utilized pathology excerpts from the PALGA nationwide Dutch Pathology Registry (1991-2019).
- Extracted data on patient demographics, tumor characteristics, and diagnostic center.
- Calculated incidence rates and analyzed the proportion of cases managed at BTCs.
Main Results:
- Identified 420 chordoma patients between 1991 and 2019.
- Chordoma incidence increased from 0.593 to 1.111 per million inhabitants (P=0.001).
- The proportion of cases analyzed at BTCs rose significantly from 29.3% to 84.4% (P<0.001).
Conclusions:
- A significant increase in the standardized incidence of pathology-proven chordoma in the Netherlands was observed.
- There is a notable rise in the analysis of chordoma samples at specialized BTCs.
- This centralization is expected to improve diagnostic accuracy and optimize treatment for chordoma patients.
