D'Hooghe, Ellen; Furtwängler, Rhoikos; Chowdhury, Tanzina; Vokuhl, Christian; Al-Saadi, Reem; Pritchard-Jones, Kathy; Graf, Norbert; D'Hooghe, Ellen; Furtwängler, Rhoikos; Chowdhury, Tanzina; Vokuhl, Christian; Al-Saadi, Reem; Pritchard-Jones, Kathy; Graf, Norbert; Vujanić, Gordan M; - view fewer (2023) Stage I epithelial or stromal type Wilms tumors are low risk tumors: An analysis of patients treated on the SIOP-WT-2001 protocol in the UK-CCLG and GPOH studies (2001-2020). Cancer , 129 (12) pp. 1930-1938. 10.1002/cncr.34734.

	Text (Article) Pritchard-Jones_01 - R1 - EtStWT Stage I main manuscript - clean.pdf Download (0B)
Preview	Text (Figure 1) Figure 1 - Event-free and overall survivals in our cohort.pdf - Accepted Version Download (610kB) | Preview
Preview	Text (Table 1) Pritchard-Jones_TABLE 1. Patients and tumors characteristics.pdf Download (9kB) | Preview
Preview	Text (Table 2) Pritchard-Jones_TABLE 2. Histologic characteristics of ET-WT and ST-WT.pdf Download (7kB) | Preview
Preview	Text (Table 3) Pritchard-Jones_TABLE 3. MVA of EFS by groups, age and gender.pdf Download (5kB) | Preview
Preview	Text (Table 4) Pritchard-Jones_TABLE 4. Events in our cohort.pdf Download (6kB) | Preview

Abstract

BACKGROUND: Patients treated with preoperative chemotherapy with stage I intermediate-risk Wilms tumor (IR-WT) represent the largest group of patients with Wilms tumor (WT), and they have excellent outcomes. METHODS: The authors performed a retrospective analysis of patients with stage I epithelial (ET-WT) or stromal type WT (ST-WT) treated pre- and postoperatively according to the International Society of Paediatric Oncology-WT-2001 protocol in the UK Children's Cancer and Leukaemia Group and Gesellschaft für Pädiatrische Onkologie und Hämatologie groups' participation in the relevant WT trials and studies (2001-2020). RESULTS: There were 880 patients with stage I IR-WT, including 124 with ET-WT, 156 with ST-WT, and 600 with other IR-WT (oIR-WT). Patients with stage I ET-WT or ST-WT were significantly younger than patients with oIR-WT, represented a large proportion of stage I WTs in their groups, and tumors showed poor histologic response to preoperative chemotherapy. The 5-year event-free survival (EFS) estimates for patients with stage I ET-WT (96.8% ± 1.8 SE) or ST-WT (96.8% ± 1.6 SE) were significantly better than for patients with oIR-WT (90.3% ± 1.3 SE) (p = .014 and p = .009, respectively). A multivariate analysis showed that histologic type (ET-WT or ST-WT) remained a significant factor for EFS when adjusted for age and gender (p = .032 and p = .022, respectively). In both groups, relapses occurred in 3.2% of patients, and the overall survival was 99.2%. CONCLUSIONS: The results suggest that stage I ET-WT or ST-WT could be regarded as low-risk WT, for which omission of postoperative chemotherapy should be considered. PLAIN LANGUAGE SUMMARY: Patients with pretreated intermediate-risk Wilms tumor (WT) represent the largest group of patients with WT. This study reports the outcomes of patients with stage I epithelial type (ET-WT) or stromal type WT (ST-WT). These patients were significantly younger and had a larger proportion of stage I cases than patients with other intermediate-risk WT (oIR-WT). The event-free survival for patients with stage I ET-WT and ST-WT was significantly better than for patients with oIR-WT. Rare relapses were curable resulting in 99.2% overall survival.

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