Clinical outcome and biological predictors of relapse following nephrectomy only for very low risk Wilms tumor (VLR WT): A report from Children’s Oncology Group AREN0532.

Fernandez, Conrad V.; Perlman, Elizabeth J.; Mullen, Elizabeth; Chi, Yueh‐Yun; Hamilton, Thomas E.; Gow, Kenneth W.; Ferrer, Fernando; Barnhart, Douglas C.; Ehrlich, Peter F.; Khanna, Geetika; Kalapurakal, John A.; Bocking, Tina; Huff, Vicki; An, Q.; Geller, James I.; Grundy, Paul E.; Anderson, James R.; Dome, Jeffrey S.; Shamberger, Robert C.

doi:10.1200/jco.2015.33.15_suppl.10023

Cited by 18 publications

(30 citation statements)

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“…It is not known if current treatment schema 'over-treat' these or are excessive, accounting for the disproportionately high survival rates seen in our cohort and that of the Japanese Wilms tumor Study (JWiTS) group, and whether such patients could benefit from a measured reduction in treatment intensity-akin to strategies for very low risk disease. 19,26,27 In ontogenic models of Wilms tumor, low risk molecular subgroups of patients have been proposed that are younger at diagnosis and without nephrogenic rests-similar to the clinical profile seen in our cohort-and are characterized by an absence of LOH 11p15, WT1 mutations and IGF2 aberrations. 28,29 However there are no established treatment selection criteria to identify this low-risk group, and current 1p and 16q LOH markers do not stratify them adequately.…”

Section: Ethnicity a A A A A A A A A A A A A A A N N A A A N A A A N supporting

confidence: 63%

“…28,29 However there are no established treatment selection criteria to identify this low-risk group, and current 1p and 16q LOH markers do not stratify them adequately. 26,27 Little else is known about the molecular signature of this lowrisk phenotype, and further characterization will be needed to appropriately stratify these cases and allocate treatment.…”

Section: Ethnicity a A A A A A A A A A A A A A A N N A A A N A A A N mentioning

confidence: 99%

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Clinical, pathological and loss of heterozygosity differences in Wilms tumors between Asian and non‐Asian children

Loke

Wong

Tawng

et al. 2018

Intl Journal of Cancer

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Wilms tumor demonstrates significant interethnic epidemiological, histological and outcome differences, and is rare and poorly studied among Asians. We compared the clinicopathological, and loss of heterozygosity (LOH) profile and survival outcomes of Asian and non-Asian patients with Wilms tumor. Clinical charts and histological slides from patients with malignant renal tumors over a period of 20 years were retrospectively reviewed. We adapted a genotyping assay to determine 1p36 and 16q21-22 LOH in formalin-fixed paraffin-embedded (FFPE) specimens, and compared these characteristics between Asian and non-Asian patients. Fifty-three (79.1%) Asian and 14 (20.9%) non-Asian patients had Wilms tumors. Compared to non-Asians, Asians were younger (mean 4.6 and 4.0 years, respectively), had more equal gender distribution (female: male = 1.8 and 1.0, respectively), fewer tumors with unfavorable histology (25.0% and 4.1%, respectively, p = 0.05), and less advanced disease at presentation, yet similar nodal metastases rates (16.7% and 18.4%, respectively). No Asian patients had bilateral tumors. Our adapted genotyping assay accurately determined LOH in FFPE specimens <10 years post-fixation. Among 30 Asian patients, 1p and 16q LOH were each detected in 5 (16.7%) patients, respectively-similar to rates reported in other ethnicities. Yet after similar treatment with National Wilms Tumor Study regimens, 15-year event-free and overall survival for Asian patients was 95.7% and 96.3% respectively. In summary, despite similar nodal metastasis and LOH rates, Asian patients had fewer unfavorable histology tumors, lower-stage disease, and better survival outcomes. The bases for these differences and implications on treatment strategy for these patients warrant further study.

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Section: Ethnicity a A A A A A A A A A A A A A A N N A A A N A A A N supporting

confidence: 63%

Section: Ethnicity a A A A A A A A A A A A A A A N N A A A N A A A N mentioning

confidence: 99%

Clinical, pathological and loss of heterozygosity differences in Wilms tumors between Asian and non‐Asian children

Loke

Wong

Tawng

et al. 2018

Intl Journal of Cancer

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“…Of the discordant cases, one was diagnosed as a cystic nephroma on IFS and then determined to be a cystic Wilms tumor on FP. Due to the age (<2 years) of the patient and weight of the specimen (<550 g), no additional invasive interventions were required as patient met the standards of a very low risk Wilms tumor (VLRWT) as per the recent Wilms tumor protocols of the Children's Oncology Group . This case was managed with surgery‐only and no need for long‐term central venous access for chemotherapy.…”

Section: Resultsmentioning

confidence: 99%

“…Although the majority of renal tumors suspicious for malignancy in children are Wilms tumors, other pathologies, both malignant and benign, can be encountered. Some benign and malignant renal tumors, including VLRWT, are managed with surgical resection alone, thus highlighting the importance of establishing a histologic diagnosis that can guide the need for adjunctive procedures at the time of tumor resection. In a patient who meets preoperative criteria for VLRWT (tumor weight <550 g, age <2 years, and no clinical evidence of metastatic disease), obtaining IFS may not be beneficial for confirming VLRWT classification since FP of the entire specimen is needed to confirm favorable histology and no lymph node involvement.…”

Section: Discussionmentioning

confidence: 99%

“…Some benign and malignant renal tumors, including VLRWT, are managed with surgical resection alone, thus highlighting the importance of establishing a histologic diagnosis that can guide the need for adjunctive procedures at the time of tumor resection. In a patient who meets preoperative criteria for VLRWT (tumor weight <550 g, age <2 years, and no clinical evidence of metastatic disease), obtaining IFS may not be beneficial for confirming VLRWT classification since FP of the entire specimen is needed to confirm favorable histology and no lymph node involvement. However, IFS may rule out other malignant tumor types that may require adjuvant chemotherapy (e.g., rhabdoid tumor and clear cell sarcoma of the kidney), and thus further supporting any decision for or against vascular access placement at the same operative setting.…”

Section: Discussionmentioning

confidence: 99%

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Reliability of intraoperative frozen section for the diagnosis of renal tumors suspicious for malignancy in children and adolescents

Carrasco

Caldwell

Cost

et al. 2017

Pediatric Blood & Cancer

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Background The ability of Intraoperative Frozen Section (IFS) to reliably diagnose renal tumors in children and adolescents is largely unknown. The objective of our study is to evaluate the ability of IFS to establish a histologic diagnosis for renal tumors in this population. Methods We reviewed our experience with patients who underwent IFS at the time of surgery for a renal tumor suspicious for malignancy from 2005–15. The IFS was compared to the final pathology (FP). Data on concordance and reliability were analyzed. Results 130 patients underwent surgical interventions for a renal tumor suspicious for malignancy, and 32 (25%) patients underwent IFS. Median turnaround time for IFS was 20 minutes (range 13 – 44). The histologic IFS diagnosis correlated with FP in 26 (81.2%) cases, was discrepant in 3 (9.4%) cases, and IFS was deferred to FP in 3 (9.4%) cases (kappa 0.71, 95% CI 0.52–0.899, p<0.001). The IFS correctly distinguished between Wilms tumor and non-Wilms tumor in 30 (94%) cases (kappa 0.874, 95% CI 0.705–1, p<0.001). A total of 17 of 19 (89.5%) Wilms tumors were correctly diagnosed by IFS, yielding a sensitivity of 0.89 (95% CI 0.67–0.99) and a specificity of 1 (95% CI 0.75–1). Conclusion IFS is a reliable tool to establish a histologic diagnosis, and to differentiate between Wilms and non-Wilms tumors in children and adolescents with renal tumors. The use of IFS should be encouraged in cases where obtaining a diagnosis will provide guidance for important “real-time” medical decision making, specifically additional adjunctive surgical procedures.

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Outcome analysis of stage I epithelial‐predominant favorable‐histology Wilms tumors: A report from Children's Oncology Group study AREN03B2

Parsons

Mullen

Geller

et al. 2020

Cancer

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BACKGROUND: Stage I epithelial-predominant favorable-histology Wilms tumors (EFHWTs) have long been suspected to have an excellent outcome. This study investigates the clinical and pathologic features of patients with stage I EFHWTs to better evaluate the potential for a reduction of chemotherapy and its associated toxicity. METHODS: All patients registered in the Children's Oncology Group (COG) AREN03B2 study between 2006 and 2017 with stage I EFHWTs were identified. EFHWTs were defined as tumors with at least 66% epithelial differentiation, regardless of the degree of differentiation. Clinical information was abstracted from COG records. Event-free survival (EFS) and overall survival (OS) were calculated and compared between groups based on age and therapy. RESULTS: The 4-year EFS rate was 96.2% (95% confidence interval, 92%-100%), and the OS rate was 100%; EFS and OS did not statistically significantly differ with the age at diagnosis (<48 vs ≥48 months; P = .37) or treatment (EE4A vs observation only; P = .55). Six events were reported. Three patients developed contralateral tumors and did not otherwise relapse; none of these had nephrogenic rests or a recognized predisposition syndrome. Three patients developed metastatic recurrence; all 3 had received EE4A as their primary therapy after nephrectomy. CONCLUSIONS: These findings demonstrate an excellent outcome for stage I EFHWTs with >95% EFS and OS. These data support the utility of investigating the treatment of stage I EFHWTs with observation alone after nephrectomy. Cancer 2020;126: 2866-2871.

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Clinical outcome and biological predictors of relapse following nephrectomy only for very low risk Wilms tumor (VLR WT): A report from Children’s Oncology Group AREN0532.

Cited by 18 publications

References 0 publications

Clinical, pathological and loss of heterozygosity differences in Wilms tumors between Asian and non‐Asian children

Clinical, pathological and loss of heterozygosity differences in Wilms tumors between Asian and non‐Asian children

Reliability of intraoperative frozen section for the diagnosis of renal tumors suspicious for malignancy in children and adolescents

Outcome analysis of stage I epithelial‐predominant favorable‐histology Wilms tumors: A report from Children's Oncology Group study AREN03B2

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