Incidence and centralization of chordoma in the Netherlands : A nationwide study between 1991 and 2020

Copyright © 2024 The Authors. Published by Elsevier Ltd.. All rights reserved..

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.

Medienart:

E-Artikel

Erscheinungsjahr:

2024

Erschienen:

2024

Enthalten in:

Zur Gesamtaufnahme - volume:89

Enthalten in:

Cancer epidemiology - 89(2024) vom: 20. März, Seite 102527

Sprache:

Englisch

Beteiligte Personen:

Lipplaa, A [VerfasserIn]
van der Wal, R J P [VerfasserIn]
Krol, A D G [VerfasserIn]
Peul, W C [VerfasserIn]
Bovée, J V M G [VerfasserIn]
Gelderblom, H [VerfasserIn]

Links:

Volltext

Themen:

Centralization
Chordoma
Conventional chordoma
Dedifferentiated chordoma
Incidence
Journal Article
Poorly differentiated chordoma

Anmerkungen:

Date Completed 18.03.2024

Date Revised 18.03.2024

published: Print-Electronic

Citation Status MEDLINE

doi:

10.1016/j.canep.2024.102527

funding:

Förderinstitution / Projekttitel:

PPN (Katalog-ID):

NLM367681315