Malignant rhabdoid tumours of the kidney (MRTKs), registered on recent SIOP protocols from 1993 to 2005: A report of the SIOP renal tumour study group

• Published in: Pediatric blood & cancer 2011-05, Vol.56 (5), p.733-737
• Main Authors: van den Heuvel-Eibrink, Marry M., van Tinteren, Harm, Rehorst, Harriet, Coulombe, Aurore, Patte, Catharine, de Camargo, Beatriz, de Kraker, Jan, Leuschner, Ivo, Lugtenberg, Rieneke, Pritchard-Jones, Kathy, Sandstedt, Bengt, Spreafico, Filippo, Graf, Norbert, Vujanic, Gordan M.
• Format: Article
• Language: English
• Subjects: Antineoplastic Combined Chemotherapy Protocols - therapeutic use; Child, Preschool; children; Combined Modality Therapy; Dactinomycin - therapeutic use; Disease-Free Survival; Female; Follow-Up Studies; Humans; Infant; kidney; Kidney Neoplasms - drug therapy; Kidney Neoplasms - mortality; Kidney Neoplasms - pathology; Male; Medicin och hälsovetenskap; Neoplasm Metastasis; Neoplasm Staging; Registries; Rhabdoid Tumor - drug therapy; Rhabdoid Tumor - mortality; Rhabdoid Tumor - pathology; rhabdoid tumour; Survival Rate; Treatment Outcome; Vincristine - therapeutic use
• ISSN: 1545-5009; 1545-5017; 1545-5017
• DOI: 10.1002/pbc.22922

Background Survival data of malignant rhabdoid tumour of the kidney (MRTK) registered in SIOP trials, advocating preoperative chemotherapy, are not available. Aim To evaluate characteristics, response and survival of MRTK patients registered in recent SIOP protocols. Methods An evaluation of all MRTK patients treated from 1993 to 2005 (SIOP trials 93‐01 and 2001) was performed. Data were obtained from study specific case record forms and entered centrally in a database. Results Hundred and seven patients were identified (57 male), with a median age at diagnosis of 13 months (interquartile range 6–27 months), and a median follow‐up time of 60 months. Left and right kidneys were equally affected. Tumour stage distribution was stage I (6%), stage II (22%), stage III (43%), stage IV (22%) and stage V (3%). Stage IV patients included 17 with pulmonary metastasis (8 lung‐only) and 12 with multiple organ metastases (bone, brain and liver). Primary surgery was the upfront treatment approach in 22/107 patients (21%), by which 19 patients reached a complete remission (CR). Median difference in tumour volume before and after preoperative chemotherapy was 69 ml (interquartile range: 4.5–158.0, P