Clinical and genetic risk factors define two risk groups of extracranial malignant rhabdoid tumours (eMRT/RTK)

Current and Emerging Therapeutic Approaches for Extracranial Malignant Rhabdoid Tumors

“…Certain cytostatics such as anthracyclines, alkylating, platinating agents and vinca alkaloids are employed in all current approaches. 6 , 8–10 , 32–34 …”

“… [ 8 ] SIOP RTSG 107 (RTK=107) 13 (0–9 years) 25% 27/107 age, stage 26% (5 year) 22% (5 year) [ 9 ] SEER 229 (ATRT=81, eMRT=103, RTK=45) 24 (0–89 years) 43% 58/134* age, stage, RTx 33% (5 year)** n.a. [ 32 ] EpSSG NRSTS 2005 100 (eMRT=83, RTK=17) 16.8 (3 d - 10.9 years) 23% 23/100 age, gender*** 38.4% (3 year) 32.3% (3 year) [ 10 ] Beijing Children’s Hospital 53 (eMRT=21, RTK=32) 16 (0–8.75) 36% (19/53) age, stage 23.7% (3 year) 14.5% (3 year) [ 34 ] EU-RHAB 100 (eMRT=70, RTK=30) 11.6 (0–17.2 years) 35% 35/100 GLM, stage, surgery 45.8% (5 year) 35.2% (5 year) [ 6 ] Notes : § Distant metastasis or synchronous tumor, *n=14 patients without staging not included, **5-year OS for entire group of patients, ***Only in patients with advanced stage. …”

“…3 , 4 The 5-year overall- and disease-free survival for extracranial MRT are significantly better than for ATRT, due to a higher percentage of gross total resection (GTR), radiotherapy (RTx) and older age at diagnosis. 5 , 6

Figure 1 Localization of extracranial MRT (EU-RHAB registry data). ( A ) Anatomical localization of patients with extracranial MRT (n=185) registered between 2007 and 2020.

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Fifty years of clinical and research studies for childhood renal tumors within the International Society of Pediatric Oncology (SIOP)

“…Radiology Diffusion weighted MRI is helpful in characterizing renal tumors Outcome of patients with CT-only lung metastasis is inferior to no metastasis Specific renal tumors require biopsy before preoperative chemotherapy ( 14), ( The basis for the current UMBRELLA study is summarized in several reviews on Nephroblastoma, 13,37,38 relapse treatment, 39,40 pathology and biology, 41 but also on different non-WTs. [42][43][44][45][46] Over the last decades collaboration and exchange of knowledge between SIOP-RTSG and the Children's Oncology Group Renal Tumor Committee has developed resulting in a number of papers such as renal tumors of early age, 47 a meta-analysis of high dose chemotherapy, 48 late relapses, 49 the advances of international collaboration 50 and new approaches to risk stratification for WT. 51 In 2015 the task force HARMONICA (HARMONIzation and CollAboration for pediatric renal tumors) was established as an exchange platform, building on the expertise of both large study groups, enhancing international collaboration and supporting Young Investigators interested in renal tumors in childhood.…”

SMARCA4‐deficient rhabdoid tumours show intermediate molecular features between SMARCB1‐deficient rhabdoid tumours and small cell carcinomas of the ovary, hypercalcaemic type

“…Malignant rhabdoid tumours (RTs) are rare malignancies characterized by ‘rhabdoid’ histomorphology, highly aggressive clinical behaviour, and early onset, with a median age at diagnosis of less than 2 years [1–3]. RTs occur in the central nervous system, where they are referred to as atypical teratoid/rhabdoid tumours (ATRTs), or in extracranial tissues (extracranial rhabdoid tumours, ECRTs) including the kidney, liver, and miscellaneous soft tissues.…”