Cardiff University | Prifysgol Caerdydd ORCA
Online Research @ Cardiff 
WelshClear Cookie - decide language by browser settings

Clear cell sarcomas of the kidney registered on International Society of Pediatric Oncology (SIOP) 93-01 and SIOP 2001 protocols: A report of the SIOP Renal Tumour Study Group

Furtwangler, R., Gooskens, S. L., van Tinteren, H., De Kraker, J., Schleiermacher, G., Bergeron, C., de Carmargo, B., Acha, T., Godzinski, J., Sandstedt, B., Leushcner, I., Vujanic, Gordan, Pieters, R., Graf, N. and van den Heuvel-Ebrink, M. M. 2013. Clear cell sarcomas of the kidney registered on International Society of Pediatric Oncology (SIOP) 93-01 and SIOP 2001 protocols: A report of the SIOP Renal Tumour Study Group. European Journal of Cancer 49 (16) , pp. 3497-3506. 10.1016/j.ejca.2013.06.036

Full text not available from this repository.


Purpose Clear Cell Sarcoma of the Kidney (CCSK) is a rare childhood renal tumour. Only a few homogeneously treated CCSK cohorts have been reported. This study aims to describe clinical characteristics and survival of CCSK patients treated according to recent International Society of Pediatric Oncology (SIOP) protocols. Patients and methods We analysed the prospectively collected data of patients with a histologically verified CCSK, entered onto SIOP 93-01/2001 trials. Results A total of 191 CCSK patients (64% male) were analysed, with a median age at diagnosis of 2.6 years. Stage distribution for stages I, II, III and IV was 42%, 23%, 28% and 7%, respectively. Pre-operative chemotherapy was administered to 169/191 patients. All patients underwent total nephrectomy and 189/191 patients received post-operative chemotherapy. Radiotherapy was applied in 2/80 stage I, 33/44 stage II, 44/54 stage III and 6/13 stage IV patients. Five year event-free survival (EFS) and overall survival (OS) were 79% (95% confidence interval (CI): 73-85%) and 86% (95% CI: 80-92%) respectively. Stage IV disease and young age were significant adverse prognostic factors for event-free survival. Factors such as gender, tumour volume and type of initial treatment were not found to be prognostic for EFS and OS. Conclusion In this largest SIOP cohort described so far, overall outcome of CCSK is reasonable, although treatment of young and advanced-stage disease patients is challenging. As further intensification of treatment is hampered by direct and late toxicity, future directions should include the development of targeted therapy based on specific molecular aberrations of CCSK.

Item Type: Article
Date Type: Publication
Status: Published
Schools: Medicine
Subjects: R Medicine > R Medicine (General)
R Medicine > RZ Other systems of medicine
Uncontrolled Keywords: Antineoplastic Combined Chemotherapy Protocols; Chemotherapy Adjuvant;Child Preschool;Clinical Trials as Topic;Disease-Free Survival;Female;Humans;Infant;Kaplan-Meier Estimate;Kidney Neoplasms;Male;Neoadjuvant Therapy; Neoplasm Staging;Nephrectomy;Proportional Hazards Models; Radiotherapy Adjuvant;Risk Factors;Sarcoma Clear Cell;Time Factors;Treatment Outcome
Additional Information: Chemicals and CAS Registry Numbers: carboplatin, 41575-94-4; cyclophosphamide, 50-18-0; dactinomycin, 1402-38-6, 1402-58-0, 50-76-0; doxorubicin, 23214-92-8, 25316-40-9; epirubicin, 56390-09-1, 56420-45-2; etoposide, 33419-42-0; vincristine, 57-22-7
Publisher: Elsevier
ISSN: 0014-2964
Last Modified: 04 Jun 2017 08:13

Citation Data

Cited 37 times in Scopus. View in Scopus. Powered By Scopus® Data

Actions (repository staff only)

Edit Item Edit Item