Rationale for the treatment of Wilms tumour in the UMBRELLA SIOP–RTSG 2016 protocol

Heuvel‐Eibrink, Marry M. van den; Hol, Janna A.; Pritchard‐Jones, Kathy; Tinteren, Harm van; Furtwängler, Rhoikos; Verschuur, Arnauld; Vujanić, Gordan; Leuschner, Ivo; Brok, Jesper; Rübe, Christian; Smets, Anne; Janssens, Geert O.; Godziński, Jan; Ramírez-Villar, Gema L.; Camargo, Beatriz de; Segers, Heidi; Collini, Paola; Gessler, Manfred; Bergeron, Christophe; Spreafico, Filippo; Graf, Norbert

doi:10.1038/nrurol.2017.163

Cited by 286 publications

(414 citation statements)

References 58 publications

(72 reference statements)

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“…Lung metastases are commonly observed in MRTK, which occurs in young children . For this reason, the SIOP recently proposed a systematic biopsy of all renal tumors with lung metastases occurring in children under the age of two years at diagnosis . In our series, one third of the nine patients positive for this criterion had MRTK, the remaining having a WT.…”

Section: Discussionmentioning

confidence: 64%

“…The SIOP‐RTSG recommends going to upfront surgery in only a limited number of tumors: for purely cystic renal lesions (because needle sampling usually cannot provide representative material), and for infants less than 6 months of age because of the high prevalence of CMN in this age group. The SIOP recommends presumptive chemotherapy without biopsy for bilateral or multifocal tumors because it is usually challenging or even impossible to distinguish between WT and NRs, even for trained pathologists as illustrated by our results …”

Section: Discussionmentioning

confidence: 80%

“…Table shows the criteria used to indicate the diagnostic biopsy and distinguishes SIOP criteria defined in the SIOP93 and SIOP2001 guidelines from those currently proposed in the SIOP‐RTSG 2016 Umbrella study . Two criteria were present in 34/111 children.…”

Section: Resultsmentioning

confidence: 99%

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Indications and results of diagnostic biopsy in pediatric renal tumors: A retrospective analysis of 317 patients with critical review of SIOP guidelines

Monneraye

Michon

Pacquement

et al. 2019

Pediatric Blood & Cancer

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Objectives According to the Renal Tumor Study Group (RTSG) of the International Society of Paediatric Oncology (SIOP), diagnostic biopsy of renal tumors prior to neoadjuvant chemotherapy is not mandatory unless the presentation is atypical for a Wilms tumor (WT). This study addresses the relevance of this strategy as well as the accuracy and safety of image‐guided needle biopsy. Methods Clinical, radiological, and pathological data from 317 children (141 males/176 females, mean age: 4 years, range, 0–17.6) consecutively treated in one SIOP‐affiliated institution were retrospectively analyzed. Results Presumptive chemotherapy for WT was decided for 182 patients (57% of the cohort), 24 (8%) were operated upfront, and 111 (35%) were biopsied at diagnosis. A non‐WT was confirmed after surgery in 5/182 (3%), 11/24 (46%), and 28/111 (25%), respectively. Age at diagnosis was the most commonly (46%) used criterion to go for biopsy but a nine‐year threshold should be retrospectively considered more relevant. Tumor volumes of clear cell sarcoma of the kidney and WT were significantly higher than those of other tumors (P = 0.002). The agreement between core‐needle biopsy (CNB) and final histology was 99%. No significant morbidity was associated with CNB. Conclusion The use of SIOP criteria to identify patients eligible for presumptive WT neoadjuvant chemotherapy or upfront surgery avoided biopsy in 65% of children and led to a 97% rate of appropriate preoperative chemotherapy. Image‐guided CNB is a safe and accurate diagnostic procedure. The relevance of SIOP biopsy criteria might be improved by using an older age threshold (9 years instead of 6 years) and by adding initial tumor volume.

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Section: Discussionmentioning

confidence: 64%

Section: Discussionmentioning

confidence: 80%

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Indications and results of diagnostic biopsy in pediatric renal tumors: A retrospective analysis of 317 patients with critical review of SIOP guidelines

Monneraye

Michon

Pacquement

et al. 2019

Pediatric Blood & Cancer

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“…In this context, the SIOP-RTSG is aiming to offer all paediatric patients with renal tumours who are enrolled in SIOP protocols standardized, highquality diagnostics and treatment, independent of socioeconomic status or geographical region. To achieve these goals, the UMBRELLA SIOP-RTSG 2016 protocol (the UMBRELLA protocol), approved by the SIOP-RTSG and by the ethical committee in the country of the sponsor (Germany) (EudraCT number 2016-004180-39), is currently being implemented in over 50 countries 22,23 . The management of paediatric CCSK is addressed in the UMBRELLA protocol.…”

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confidence: 99%

Rationale for the treatment of children with CCSK in the UMBRELLA SIOP–RTSG 2016 protocol

et al. 2018

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“…In Europe, children are treated with neoadjuvant chemotherapy prior to surgery as per The International Society of Pediatric Oncology Renal Tumors Study Group (SIOP RTSG) guidelines [2]. Conversely, in North America, children undergo immediate surgery prior to chemotherapy in accordance with the Children's Oncology Group (COG).…”

Section: Introductionmentioning

confidence: 99%

Somatic TP53 Mutations Are Detectable in Circulating Tumor DNA from Children with Anaplastic Wilms Tumors

Treger

Chagtai

Butcher

et al. 2018

Translational Oncology

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BACKGROUND: Diffuse anaplastic Wilms tumor (DAWT) is a rare, high-risk subtype that is often missed on diagnostic needle biopsy. Somatic mutations in TP53 are associated with the development of anaplasia and with poorer survival, particularly in advanced-stage disease. Early identification of DAWT harboring TP53 abnormalities could improve risk stratification of initial therapy and monitoring for recurrence. METHODS: Droplet digital polymerase chain reaction (ddPCR) was used to evaluate 21 samples from 4 patients with DAWT. For each patient, we assessed TP53 status in frozen tumor, matched germline DNA, and circulating tumor DNA (ctDNA) from plasma, serum, and urine collected throughout treatment. RESULTS: Mutant TP53 was detectable in ctDNA from plasma and serum in all patients. We did not detect variant TP53 in the same volume (200 μl) of urine. One patient displayed heterogeneity of TP53 in the tumor despite both histological sections displaying anaplasia. Concentration of ctDNA from plasma/serum taken prenephrectomy varied significantly between patients, ranging from 0.44 (0.05-0.90) to 125.25 (109.75-140.25) copies/μl. We observed variation in ctDNA throughout treatment, and in all but one patient, ctDNA levels fell significantly following nephrectomy. CONCLUSION: We demonstrate for the first time that ddPCR is an effective method for detection of mutant TP53 in ctDNA from children with DAWT even when there is intratumoral somatic heterogeneity. This should be further explored in a larger cohort of patients, as early detection of circulating variant TP53 may have significant clinical impact on future risk stratification and surveillance.

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Rationale for the treatment of Wilms tumour in the UMBRELLA SIOP–RTSG 2016 protocol

Cited by 286 publications

References 58 publications

Indications and results of diagnostic biopsy in pediatric renal tumors: A retrospective analysis of 317 patients with critical review of SIOP guidelines

Indications and results of diagnostic biopsy in pediatric renal tumors: A retrospective analysis of 317 patients with critical review of SIOP guidelines

Rationale for the treatment of children with CCSK in the UMBRELLA SIOP–RTSG 2016 protocol

Somatic TP53 Mutations Are Detectable in Circulating Tumor DNA from Children with Anaplastic Wilms Tumors

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