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 < 0.0001), indicating marked chemosensitivity. The 5-year event-free survival (EFS) of the total group was 22% (95% CI: 15-33) and overall survival 26% (95% CI: 18-37). Most events (86%) occurred within the first 2 years after diagnosis. Younger age at diagnosis was an important adverse prognostic factors for survival. In contrast, tumour volume at diagnosis, nor volume reduction was associated with outcome. CONCLUSION: MRTK has a poor outcome especially in young and advanced-stage disease patients. Neither tumour volume at diagnosis, nor pre-operative chemosensitivity are prognostic factors for survival.
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 < 0.0001), indicating marked chemosensitivity. The 5-year event-free survival (EFS) of the total group was 22% (95% CI: 15-33) and overall survival 26% (95% CI: 18-37). Most events (86%) occurred within the first 2 years after diagnosis. Younger age at diagnosis was an important adverse prognostic factors for survival. In contrast, tumour volume at diagnosis, nor volume reduction was associated with outcome. CONCLUSION: MRTK has a poor outcome especially in young and advanced-stage disease patients. Neither tumour volume at diagnosis, nor pre-operative chemosensitivity are prognostic factors for survival.
Authors: Alicia M Waters; Jerry E Stewart; Venkatram R Atigadda; Elizabeth Mroczek-Musulman; Donald D Muccio; Clinton J Grubbs; Elizabeth A Beierle Journal: Mol Cancer Ther Date: 2016-02-12 Impact factor: 6.261
Authors: Benjamin A Farber; Neerav Shukla; Irene Isabel P Lim; Jennifer M Murphy; Michael P La Quaglia Journal: J Pediatr Surg Date: 2016-08-31 Impact factor: 2.545
Authors: Thomas J Jackson; Richard D Williams; Jesper Brok; Tanzina Chowdhury; Milind Ronghe; Mark Powis; Kathy Pritchard-Jones; Gordan M Vujanić Journal: Pediatr Blood Cancer Date: 2019-02-13 Impact factor: 3.167
Authors: Matthew E Sterling; Christopher J Long; Kristopher R Bosse; Rochelle Bagatell; Aseem R Shukla Journal: Urology Date: 2015-01-09 Impact factor: 2.649
Authors: Rajkumar Venkatramani; Yueh-Yun Chi; Max J Coppes; Marcio Malogolowkin; John A Kalapurakal; Jing Tian; Jeffrey S Dome Journal: Pediatr Blood Cancer Date: 2017-01-18 Impact factor: 3.167
Authors: Jeffrey S Dome; Conrad V Fernandez; Elizabeth A Mullen; John A Kalapurakal; James I Geller; Vicki Huff; Eric J Gratias; David B Dix; Peter F Ehrlich; Geetika Khanna; Marcio H Malogolowkin; James R Anderson; Arlene Naranjo; Elizabeth J Perlman Journal: Pediatr Blood Cancer Date: 2012-12-19 Impact factor: 3.167
Authors: Michael L Megison; Lauren A Gillory; Jerry E Stewart; Hugh C Nabers; Elizabeth Mrozcek-Musulman; Elizabeth A Beierle Journal: Mol Cancer Res Date: 2014-01-24 Impact factor: 5.852