A nationwide cohort study on treatment and survival in patients with malignant peripheral nerve sheath tumours.

Autor: Martin E; Department of Plastic and Reconstructive Surgery, University Medical Center Utrecht, the Netherlands. Electronic address: enrico.martin@hotmail.com., Coert JH; Department of Plastic and Reconstructive Surgery, University Medical Center Utrecht, the Netherlands., Flucke UE; Department of Pathology, University Medical Center Utrecht, the Netherlands; Department of Pathology, Radboud University Medical Center, Nijmegen, the Netherlands; Diagnostic Laboratory and Pathology, Princess Máxima Center for pediatric oncology, Utrecht, the Netherlands., Slooff WM; Department of Neurosurgery, University Medical Center Utrecht, the Netherlands., Ho VKY; Departments of Registry and Research, Netherlands Comprehensive Cancer Organization (IKNL), Utrecht, the Netherlands., van der Graaf WT; Department of Medical Oncology, The Netherlands Cancer Institute, Amsterdam, the Netherlands; Department of Medical Oncology, Radboud University Medical Center, Nijmegen, the Netherlands., van Dalen T; Department of Surgical Oncology, University Medical Center Utrecht, the Netherlands; Department of Surgical Oncology, Diakonessenhuis Utrecht, the Netherlands., van de Sande MAJ; Department of Orthopedic Surgery, Leiden University Medical Center, the Netherlands; Department of Solid Tumors, Princess Máxima Center for pediatric oncology, Utrecht, the Netherlands., van Houdt WJ; Department of Surgical Oncology, The Netherlands Cancer Institute, Amsterdam, the Netherlands., Grünhagen DJ; Department of Surgical Oncology, Erasmus Medical Center Cancer Institute, Rotterdam, the Netherlands., Verhoef C; Department of Surgical Oncology, Erasmus Medical Center Cancer Institute, Rotterdam, the Netherlands.
Jazyk: angličtina
Zdroj: European journal of cancer (Oxford, England : 1990) [Eur J Cancer] 2020 Jan; Vol. 124, pp. 77-87. Date of Electronic Publication: 2019 Nov 21.
DOI: 10.1016/j.ejca.2019.10.014
Abstrakt: Background: Despite curative intents of treatment in localized malignant peripheral nerve sheath tumours (MPNSTs), prognosis remains poor. This study investigated survival and prognostic factors for overall survival in non-retroperitoneal and retroperitoneal MPNSTs in the Netherlands.
Methods: Data were obtained from the Netherlands Cancer Registry and the Dutch Pathology Database. All primary MPNSTs were collected. Paediatric cases (age ≤18 years) and synchronous metastases were excluded from analyses. Separate Cox proportional hazard models were made for retroperitoneal and non-retroperitoneal MPNSTs.
Results: A total of 629 localized adult MPNSTs (35 retroperitoneal cases, 5.5%) were included for analysis. In surgically resected patients (88.1%), radiotherapy and chemotherapy were administered in 44.2% and 6.7%, respectively. In retroperitoneal cases, significantly less radiotherapy and more chemotherapy were applied. In non-retroperitoneal MPNSTs, older age (60+), presence of NF1, size >5 cm, and deep-seated tumours were independently associated with worse survival. In retroperitoneal MPNSTs, male sex and age of 60+ years were independently associated with worse survival. Survival of R1 and that of R0 resections were similar for any location, whereas R2 resections were associated with worse outcomes. Radiotherapy and chemotherapy administrations were not associated with survival.
Conclusion: In localized MPNSTs, risk stratification for survival can be done using several patient- and tumour-specific characteristics. Resectability is the most important predictor for survival in MPNSTs. No difference is present between R1 and R0 resections in both retroperitoneal and non-retroperitoneal MPNSTs. The added value of radiotherapy and chemotherapy is unclear.
(Copyright © 2019 The Author(s). Published by Elsevier Ltd.. All rights reserved.)
Databáze: MEDLINE
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