Clear cell sarcoma of the kidney in Austrian children: Long-term survival after relapse.
Pediatr Blood Cancer
; 68(5): e28860, 2021 05.
Article
en En
| MEDLINE
| ID: mdl-33438324
ABSTRACT
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 ANDMETHODS:
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.Palabras clave
Texto completo:
1
Banco de datos:
MEDLINE
Asunto principal:
Sarcoma de Células Claras
/
Neoplasias Renales
Tipo de estudio:
Observational_studies
/
Prognostic_studies
Límite:
Child
/
Child, preschool
/
Female
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Humans
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Infant
/
Male
País/Región como asunto:
Europa
Idioma:
En
Revista:
Pediatr Blood Cancer
Asunto de la revista:
HEMATOLOGIA
/
NEOPLASIAS
/
PEDIATRIA
Año:
2021
Tipo del documento:
Article
País de afiliación:
Austria