Primary hepatic metastases in nephroblastoma—a report of the SIOP/GPOH Study

Szavay, Philipp; Luithle, Tobias; Graf, Norbert; Furtwängler, Rhoikos; Fuchs, Joerg

doi:10.1016/j.jpedsurg.2005.10.021

Cited by 23 publications

(15 citation statements)

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“…In a SIOP series of children with WT and primary hepatic metastases, Szavay et al 8 suggested that initial surgical resection improved survival. In our study, 2 children had isolated liver metastases, both underwent anatomic resection at the time of operation of the primary tumor, and both survived long term.…”

Section: Discussionmentioning

confidence: 99%

The Relationship Between the Site of Metastases and Outcome in Children With Stage IV Wilms Tumor

Berger

Fernández-Pineda

Cabello

et al. 2013

Journal of Pediatric Hematology/Oncology

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Summary:The aim of this study was to analyze in detail the site of metastasis of stage 4 Wilms tumor (WT) and its correlation with outcome. The databases from 3 major European pediatric cancer institutions were screened for children with WT between 1994 and 2011. Of 208 children identified, 31 (14.9%) had metastases at diagnosis. The lung was affected in 29 children (93.5%) and the liver in 6 children (19.4%). Twenty-seven children (87.1%) had metastases isolated to 1 organ, with the lung being the most common site (80.7%). Five-year overall survival was significantly better in those children with distant disease in either lung or liver (95.8%) compared with those affected in both lung and liver (57.1%, P = 0.028). Further, prognostic markers were the response of metastases to preoperative chemotherapy (P = 0.0138), high-risk histology (P = 0.024), and local stage (P = 0.026). Five-year overall survival was 82.1% and 5-year event-free survival was 67.9%. The overall follow-up time was 74.1 and 87.2 (2 to 151) months among survivors, and the treatment-related complication rate was 16.7%. In conclusion, in our series of stage 4 WT, prognosis was excellent if histology was favorable, metastatic disease was isolated to either lungs or liver, and if metastases responded to preoperative chemotherapy.Key Words: metastases, pediatric cancer, risk stratification, survival, Wilms tumor (J Pediatr Hematol Oncol 2013;35:518-524) W ilms tumor (WT) is the most common renal malignancy in childhood and the second most frequent intraabdominal pediatric cancer after neuroblastoma. 1 Many recent advances in clinical care and the biology of this tumor have pushed the overall survival (OS) to 90%. Naturally, for children with distant metastases the OS is lower. 1 Little is known on metastatic disease in stage 4 WT and its implication on therapy and outcome. 2,3 The lungs have long been recognized as a common site of distant tumor spread in stage 4 WT and several recent studies focus on this entity. 1,2 To our knowledge, no complete description of the metastatic profile of stage 4 WT at diagnosis exists at present. Therefore, in this study we reviewed children treated at 3 European pediatric cancer institutions for WT regarding their metastatic profile at diagnosis and its implication on long-term outcome. METHODS AND PATIENTS PatientsA retrospective analysis of the WT databases at the following 3 institutions was carried out: Hannover Medical School, Hannover (Germany); Dr. von Hauner Children's Hospital, Munich (Germany); and Virgen del Rocı´o Children's Hospital, Seville (Spain). Inclusion criteria were metastatic disease at diagnosis. Consequently, children with lower stage tumors who relapsed with metastatic disease were excluded. Additional exclusion criteria were other renal tumor entities like clear cell sarcoma, rhabdoid tumor, or renal cell carcinoma. TreatmentStaging criteria for WT was based on an upfront chemotherapy-based system developed by the International Society of Pediatric Oncology (SIOP). Treatment was accord...

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Section: Discussionmentioning

confidence: 99%

The Relationship Between the Site of Metastases and Outcome in Children With Stage IV Wilms Tumor

Berger

Fernández-Pineda

Cabello

et al. 2013

Journal of Pediatric Hematology/Oncology

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“…7 It is essential to note the SIOP patients receive preoperative chemotherapy before nephrectomy and or resection of metastatic lesions. This is in contrast to the patients in this report treated with immediate nephrectomy with or without resection of metastatic lesions before chemotherapy with or without radiation therapy is given.…”

Section: Discussionmentioning

confidence: 99%

“…6–8 The International Society of Pediatric Oncology (SIOP) and the German Pediatric Oncology Group (GPOH) studied 29 patients with liver metastases at diagnosis, including some adult patients with Wilms tumor. 7 The overall survival was less than 60%, but all patients who had complete resection of the hepatic lesions survived. Another report from Varan et al of 18 patients with liver metastases also noted a poorer outcome than for patients with pulmonary disease (50.2% vs. 16.6%).…”

Section: Introductionmentioning

confidence: 97%

Hepatic Metastasis at Diagnosis in Patients With Wilms Tumor is not an Independent Adverse Prognostic Factor for Stage IV Wilms Tumor

Ehrlich¹,

Ferrer²,

Ritchey³

et al. 2009

Annals of Surgery

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Objective To determine event free survival (EFS) of children with Wilms tumor (WT) and metastatic liver disease at diagnosis. Summary and Background data We reviewed patients with stage IV Wilms tumor treated on National Wilms Tumor Study 4 and -5 to ascertain if they have a worse prognosis than other Stage IV disease. Methods 742 patients (pts) with stage IV disease were assessed for EFS (95% confidence interval [CI]) at 5 years after diagnosis. Cohorts included those who underwent resection of the liver lesions compared with those who received only chemotherapy and radiotherapy (XRT). Results Seven hundred and forty two patients with stage IV Wilms tumor were enrolled on NWTS-4 and 5, 111 of who had liver metastases. Of these, 96 had favorable histology disease and are the focus of this analysis. Twenty-two patients had a primary liver resection (wedge resection - 18 and lobectomy – 4). After chemotherapy and/or radiation, 13 patients underwent liver resection (wedge resection - 7, lobectomy - 5 and trisegmentectomy – 1). Seventy-one patients (67%) did not undergo surgery for their liver disease. In 14 patients the liver disease disappeared with chemotherapy only. Eighty-two patients received abdominal radiation. EFS for the patients with metastatic FH Wilms tumor was 75% (95% confidence interval [CI]: (71%, 78%), EFS by Stage IV category was: lung only 76% (95% CI: 72%, 80%)(513 patients); liver, not lung 76% (95% CI: 58%, 87%)(34 patients); liver and lung 70% (95% CI: 57%, 80%)(62 patients) and other sites 64% (95% CI: 42%, 79%)(25 patients). There were no significant differences among stage IV groups (p=0.60). EFS (95% CI) for the patients with primary resection of the liver metastases (22 patients) was 86% (63%, 95%) compared to 68% (56%, 78%) (p=0.09) for the 74 with no primary resection of liver metastases. There was no significant difference in EFS for patients with FH Wilms tumor treated with chemotherapy compared to that of patients treated with chemotherapy and radiation (p=0.63). The EFS (95% CI) for each of the subsets was; no abdominal radiation: 64% (34%, 83%); abdominal radiation, no boost: 77% (55%, 89%); abdominal radiation, boost: 72% (58%, 82%) (p=0.05). Conclusion Liver metastasis at diagnosis is not an adverse prognostic factor for stage IV metastatic FH WT.

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“…The most common sites of recurrence are the lungs, pleura, tumor bed, and the liver. Among all patients with Wilms’ tumor, those with liver involvement have a poorer prognosis than those with lung metastasis [40, 71]. …”

Section: Prognosismentioning

confidence: 99%

Wilms’ Tumor in Children: An Overview

Varan

2008

Nephron Clin Pract

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Wilms’ tumor is the most frequently occurring renal tumor in children and is one of the most treatment-responsive tumors. A tumor-suppressor gene and other genetic abnormalities have been implicated in its etiology. In addition, patients with several congenital anomalies, such as Beckwith-Wiedemann syndrome, WAGR syndrome, and Denys-Drash syndrome, have an increased risk of Wilms’ tumor. Previously, a three-drug chemotherapy regimen with surgery and radiotherapy was used with patients in all stages. Now, patients with early-stage Wilms’ tumor are treated with a two-drug regimen without radiotherapy, whereas those in advanced stages still receive the three-drug regimen and radiotherapy. Two large collaborative groups – the National Wilms’ Tumor Study Group (NWTS) in the United States and the International Society of Pediatric Oncology (SIOP) in Europe – are involved in Wilms’ tumor management, which differs in some aspects. Multimodality treatment has been used successfully, and in Europe preoperative strategies are used as well. As the survival rate has now reached 90%, the primary objectives of the physician are to perform nephron-sparing surgery in selected cases and to reduce the dosage and duration of chemotherapy and radiotherapy in appropriate cases. Other renal tumors occur rarely, but have also been treated successfully in the last decade.

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Primary hepatic metastases in nephroblastoma—a report of the SIOP/GPOH Study

Cited by 23 publications

References 11 publications

The Relationship Between the Site of Metastases and Outcome in Children With Stage IV Wilms Tumor

The Relationship Between the Site of Metastases and Outcome in Children With Stage IV Wilms Tumor

Hepatic Metastasis at Diagnosis in Patients With Wilms Tumor is not an Independent Adverse Prognostic Factor for Stage IV Wilms Tumor

Wilms’ Tumor in Children: An Overview

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