The management of nephroblastoma with cavo-atrial disease at presentation: experience from a developing country

Hadley, G. P.; Sheik-Gafoor, Mahomed Hoosen; Buckels, N J

doi:10.1007/s00383-010-2667-5

Cited by 18 publications

(18 citation statements)

References 15 publications

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“…[7] In cases of Wilms tumor with cavo-atrial thrombus, the recommended surgical method is extraction of the primary renal and thrombus masses simultaneously in one session under cardiopulmonary bypass. [3][4][5][6][7] The overall prognosis of these patients with cavo-atrial thrombus is the same with the same histologic subtype without cavo-atrial thrombus. But as the surgical method is much more complicated, surgical morbidity and mortality is higher in patients with cavo-atrial thrombus.…”

Section: Discussionmentioning

confidence: 85%

“…[5] In the study of Hadley et al 1 of 7 patients with cavo-atrial thrombus died in the early postoperative period. [6] Murthi et al did not report any surgical mortality in 8 cases with intracardiac thrombus. [7] Following surgical resection, patients should continue on chemotherapy regimen.…”

Section: Discussionmentioning

confidence: 92%

“…[5] In the report of Hadley et al in 10 of 406 (2.4%) Wilms tumor patients, extension of thrombus mass into the right atrium and ventricle was observed. [6] At the time of diagnosis, if there is tumor thrombus above the level of hepatic vein, neo-adjuvant chemotherapy for 5-7 weeks and re-evaluation is recommended. [1-3, 9, 10] This also gives a time for the treatment of accompanying diseases and complications and stabilization of the patient before the major surgery.…”

Section: Discussionmentioning

confidence: 99%

“…[1,4] In cases with intracardiac extension of tu-mor thrombus, the preferred surgical method is total excision of primary mass and intracardiac thrombus simultaneously under cardiopulmonary bypass. [5][6][7] …”

Section: Introductionmentioning

confidence: 99%

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A case of Wilms tumor with right ventricular extension of tumor thrombus

Çakan

Tokuç

Cerit

et al. 2017

CRIM

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Primary renal tumors comprise 6% of all childhood cancers. Wilms tumor is the most common primary renal tumor in pediatric age group and the peak age of diagnosis is 3-4 years. In 10% of cases tumor extension into hepatic vein and inferior vena cava can be seen. But tumor extension into whole inferior vena cava, right atrium and right ventricle is only seen in less than 1% of patients. A 2-year-old girl was admitted to the hospital because of abdominal distension that was noticed by the parents two weeks ago. Imaging studies revealed that she had a mass at the right renal lodge which was favoring to Wilms tumor and on thorax tomography tumor thrombus was seen in the whole inferior vena cava, right atrium and right ventricle. Neoadjuvant chemotherapy was given for 7 weeks. On the 8th week of diagnosis, under cardiopulmonary bypass, surgical operation by pediatric and cardiovascular surgery teams for primary renal tumor and for cavo-atrial tumor thrombus was performed. Pathological examination of the mass was reported as stage 3 diffuse anaplastic Wilms tumor. The patient completed 24 weeks of chemotherapy protocol and she is being followed for 15 months without any morbidity. We present our case to emphasize the importance of multidisciplinary approach in Wilms tumor with cardiac extension.

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Section: Discussionmentioning

confidence: 85%

Section: Discussionmentioning

confidence: 92%

Section: Discussionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

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A case of Wilms tumor with right ventricular extension of tumor thrombus

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Tokuç

Cerit

et al. 2017

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“…Venous involvement of WT in the childhood is frequent and occurs in up to 45% of cases. In IVC, tumor thrombosis is present in 4–10% of patients, with 1–3% of right atrium involvement [ 7 – 10 ]. Although these figures well correspond with IVC tumor thrombosis rate in adult renal cell carcinoma (RCC), the incidence of venous involvement in adult WT is unknown.…”

Section: Discussionmentioning

confidence: 99%

Adult Wilms tumor with inferior vena cava thrombus and distal deep vein thrombosis – a case report and literature review

Ratajczyk¹,

Czekaj²,

Rogala³

et al. 2018

World J Surg Onc

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BackgroundAdult Wilms tumor (WT, nephroblastoma) is a rare, but well-described renal neoplasm. Although inferior vena cava tumor thrombosis is present in up to 10% of Wilms tumors in childhood, only few cases of this clinical manifestation in adults have been reported. To the best of our knowledge, this is the first case of adult WT infiltrating into inferior vena cava (IVC) with concomitant distal deep vein thrombosis.Case presentationA 28-year-old male patient with gross hematuria and right flank pain was diagnosed with right kidney tumor penetrating to IVC. Preoperatively, acute distal thrombosis in inferior vena cava and lower extremities veins occurred. Right radical nephrectomy with tumor thrombectomy via cavotomy was performed. In order to prevent pulmonary embolism, IVC was ligated below left renal vein level. Histopathological examination revealed a triphasic nephroblastoma without anaplastic features. Postoperatively, patient was diagnosed with metastatic liver disease, which was treated with two lines of chemotherapy followed by radiotherapy with achievement of complete response.ConclusionsAdult WT occurs usually in young patients, under 40 years of age. Neoadjuvant chemotherapy proved to be effective in children, resulting with tumor shrinkage and venous tumor thrombus regression. Therefore, percutaneous biopsy should be always considered in young patients presenting with renal tumor invading venous system. IVC ligation is a safe treatment option in the event of complete inferior vena cava occlusion due to distal thrombosis concomitant to tumor thrombus, provided collateral venous pathways are well-developed.

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Optimal neoadjuvant chemotherapy duration in Wilms tumour with intravascular thrombus: A literature review and evidence from SIOP WT 2001 trial

Morris

Squire

Sznajder

et al. 2019

Pediatric Blood & Cancer

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Introduction:Preoperative chemotherapy is recommended for children with Wilms tumour with intravascular extension. Extended chemotherapy may improve resectability, but increase tumour adherence to vascular endothelium, precluding complete resection. To evaluate the optimal length of preoperative treatment, we report a two-part review comprising systematic review of the literature and investigation of patients treated in the International Society of Paediatric Oncology (SIOP) WT 2001 trial. Methods: Studies were identified using Medline and Embase databases from 1996 to present. English language titles reporting management of intravascular Wilms tumour were analysed. Patients with Wilms tumour and thrombus were identified from the SIOP WT 2001 trial. Overall survival (OS) and event-free survival (EFS), tumour regression, completeness of resection and cavectomy were investigated. Results: The search retrieved 43 articles documenting 498 children. Note that 72% of the patients received neoadjuvant chemotherapy: 101 received standard course (4-6 weeks, standard course neoadjuvant chemotherapy [StC]) and 62 extended course (> 6 weeks, extended course neoadjuvant chemotherapy [EC]). There was no significant difference between the groups in terms of thrombus regression or completeness of resection. EFS was greater in the StC group (78 vs 54%; P = .04). Of 4511 patients registered in the SIOP WT 2001 trial, 166 had thrombus. Note that 97% of the patients received neoadjuvant chemotherapy: 63 StC and 67 EC. There was no significant difference between the groups with regard to tumour regression, complete resection, or cavectomy. Survival was significantly higher in those receiving StC than EC (OS: 95% vs 82%, P = .025; EFS: 88% vs 72%, P = .047). Conclusion:There is no evidence that prolonged courses of neoadjuvant chemotherapy beyond the recommended protocols confer any additional benefit in treating intravascular extension of Wilms tumour.

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The management of nephroblastoma with cavo-atrial disease at presentation: experience from a developing country

Cited by 18 publications

References 15 publications

A case of Wilms tumor with right ventricular extension of tumor thrombus

A case of Wilms tumor with right ventricular extension of tumor thrombus

Adult Wilms tumor with inferior vena cava thrombus and distal deep vein thrombosis – a case report and literature review

Optimal neoadjuvant chemotherapy duration in Wilms tumour with intravascular thrombus: A literature review and evidence from SIOP WT 2001 trial

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