Results of children with renal tumors treated in the Austrian–Hungarian Wilms Tumor Study 1989 and the International Society of Pediatric Oncology (SIOP) 93-01/GPOH trial in Austria
“…All trials included additional irradiation to the flank for local stages II and III (AHWTS89: 14.4 Gy; SIOP93‐01: 30‐35 Gy; and SIOP2001: 25.2‐36 Gy) 6,7,9 …”
Section: Methodsmentioning
confidence: 99%
“…According to the treatment schedule for localised unilateral kidney tumours without visible distant metastases (stages I‐III), all but one patient received 4‐week preoperative chemotherapy with actinomycin‐D and vincristine (AV). The cumulative doses were the same in all studies 7,9 …”
Section: Methodsmentioning
confidence: 99%
“…One patient had primary surgery. Staging was done according to the Revised SIOP working classification of renal tumours in childhood (retrospectively for the AHWTS study) 9–11 …”
Introduction
Clear cell sarcoma of the kidney (CCSK) is a rare malignant childhood renal tumour. Recently, the central nervous system (CNS) was found to be the most frequent site of relapse associated with a poor outcome. Optimal treatment strategies are scarce.
Patients and Methods
Retrospective data analysis of all Austrian children with CCSK. They were enrolled in the Austrian‐Hungarian Wilms Tumour Study (AHWTS) 1989, the SIOP93‐01 or the SIOP2001 study between 1990 and 2019. Demographic, diagnostic, treatment‐related variables and survival data were analysed.
Results
We identified 12 children with CCSK (M = 7, F = 5; median age 1.6 years). All had localised disease (stage I: 2; stage II: 2; stage III: 8) at diagnosis, and a first complete remission (CR1) was achieved in 12/12. Six patients are in an ongoing CR1 (median follow‐up 10 years). Six other patients had a relapse (local 1; brain 5) a median time of 2.4 years from diagnosis. Two patients died of the disease 4 months and 2.8 years after first relapse. Four of five patients with CNS relapse are in CR2 with a median follow‐up time of 9.3 years after relapse diagnosis. Relapse treatment included a combination of chemotherapy, radiation and surgery. Two children received high‐dose chemotherapy followed by autologous stem cell rescue, and one child received intrathecal mafosphamide. Long‐term side effects after treatment were impaired tubular renal function (n = 4), cardiomyopathy (n = 1) and growth disorders (n = 1).
Conclusions
In this series, the brain was the most common site of relapse. Long‐term survival after recurrence was achievable with intensive multimodal therapy.
“…All trials included additional irradiation to the flank for local stages II and III (AHWTS89: 14.4 Gy; SIOP93‐01: 30‐35 Gy; and SIOP2001: 25.2‐36 Gy) 6,7,9 …”
Section: Methodsmentioning
confidence: 99%
“…According to the treatment schedule for localised unilateral kidney tumours without visible distant metastases (stages I‐III), all but one patient received 4‐week preoperative chemotherapy with actinomycin‐D and vincristine (AV). The cumulative doses were the same in all studies 7,9 …”
Section: Methodsmentioning
confidence: 99%
“…One patient had primary surgery. Staging was done according to the Revised SIOP working classification of renal tumours in childhood (retrospectively for the AHWTS study) 9–11 …”
Introduction
Clear cell sarcoma of the kidney (CCSK) is a rare malignant childhood renal tumour. Recently, the central nervous system (CNS) was found to be the most frequent site of relapse associated with a poor outcome. Optimal treatment strategies are scarce.
Patients and Methods
Retrospective data analysis of all Austrian children with CCSK. They were enrolled in the Austrian‐Hungarian Wilms Tumour Study (AHWTS) 1989, the SIOP93‐01 or the SIOP2001 study between 1990 and 2019. Demographic, diagnostic, treatment‐related variables and survival data were analysed.
Results
We identified 12 children with CCSK (M = 7, F = 5; median age 1.6 years). All had localised disease (stage I: 2; stage II: 2; stage III: 8) at diagnosis, and a first complete remission (CR1) was achieved in 12/12. Six patients are in an ongoing CR1 (median follow‐up 10 years). Six other patients had a relapse (local 1; brain 5) a median time of 2.4 years from diagnosis. Two patients died of the disease 4 months and 2.8 years after first relapse. Four of five patients with CNS relapse are in CR2 with a median follow‐up time of 9.3 years after relapse diagnosis. Relapse treatment included a combination of chemotherapy, radiation and surgery. Two children received high‐dose chemotherapy followed by autologous stem cell rescue, and one child received intrathecal mafosphamide. Long‐term side effects after treatment were impaired tubular renal function (n = 4), cardiomyopathy (n = 1) and growth disorders (n = 1).
Conclusions
In this series, the brain was the most common site of relapse. Long‐term survival after recurrence was achievable with intensive multimodal therapy.
At the moment no evidence from RCTs is available which underscores the use of anthracyclines in ALL. However, 'no evidence of effect', as identified in this review, is not the same as 'evidence of no effect'. For Wilms' tumour, rhabdomyosarcoma and undifferentiated sarcoma, Ewing's sarcoma, non-Hodgkin lymphoma, hepatoblastoma and AML only one RCT was available for each type and, therefore, no definitive conclusions can be made about the antitumour efficacy of treatment with or without anthracyclines in these tumours. For other childhood cancers no RCTs were identified and therefore no conclusions can be made about the antitumour efficacy of treatment with or without anthracyclines in these tumours.
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