Management and outcome of pediatric Wilms tumor with malignant inferior Vena cava thrombus: largest cohort of single-center experience

El‐Ayadi, Moatasem; Hammad, Mahmoud; Sallam, Kareem; Ahmed, Gehad; Ahmed, Soha; Ibrahim, Amany; Refaat, Amal; Elkinaai, Naglaa; Younes, Alaa; Graf, Norbert; Zekri, Wael

doi:10.1007/s10147-020-01667-0

Cited by 18 publications

(44 citation statements)

References 13 publications

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“…In WT, survival does not change due to thrombus presence and preoperative chemotherapy can modify the thrombus level and avoid the need for cardiac bypass. For this reason, different protocols indicate preoperative chemotherapy in such cases ( 8 , 9 , 14 , 15 ). Even with careful preoperative workup, it is advisable that surgeons palpate both the IVC and renal vein, in order to avoid the transection of an unidentified vascular extension during the surgical approach because it can upstage the tumor to stage III ( 16 ).…”

Section: Discussionmentioning

confidence: 99%

Management of Pediatric Tumors With Vascular Extension

et al. 2022

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Background: Pediatric tumors can present with vascular extension to the inferior vena cava and right atrium, which impacts the surgical strategy and can be challenging during surgical treatment. Wilms tumor (WT) is the most common retroperitoneal tumor that can present with vascular extension, but also adrenal tumors, clear cell tumors from the kidney, and hepatoblastomas can present with this situation. Surgical aims include obtaining complete tumor resection without risk for patients, to avoid severe bleeding, cardiac arrest, and embolization, and to avoid cardiac bypass if possible.Objective: To describe and discuss the surgical strategies to deal with pediatric tumors with vascular extension and propose a protocol.Method: Retrospectivly review the experience of treating patients with vascular extension in a single institution, describing different scenarios and a decision making fluxogram based on the preoperative evaluation regarding the surgical techniques and the need for cardiac bypass that are adequate for each situation. Image studies are important to guide the surgical strategy. Depending on the quality of image available, computerized tomography (CT) or magnetic resonance imaging (MRI) can be enough to give the information needed for surgical decisions. Ultrasonography (US) with Doppler is helpful to confirm diagnosis and describes factors to guide the adequate surgical strategy, like the upper level extension and presence or absence of blood flow around the thrombus. Neoadjuvant chemotherapy is indicated in most cases, in order to reduce the upper level of extension (and avoid the need for cardiac bypass) and to lower the risk of embolization. The approach is based on the upper level of the thrombus and can include cavotomy or cavectomy, sometimes with cardiac bypass and cardiac arrest with hypothermia, when the thrombus reaches the diaphragmatic level or above. Pathology analysis of the thrombus can guide staging and the need for radiotherapy postoperatively.Results: A decision making fluxogram protocol is presented focusing on the surgical treatment of such condition.Conclusion: Surgery strategy is highly impacted by the presence of vascular extension in pediatric tumors. Surgeons should be aware of potential complications and how to prevent them. Such cases should be treated in reference centers.

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Section: Discussionmentioning

confidence: 99%

Management of Pediatric Tumors With Vascular Extension

et al. 2022

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“…A previous meta-analysis found no survival benefits to extended courses of chemotherapy in Wilms patients with intravascular extension, confirmed by the present study 27 . These longer courses of chemotherapy may reflect the lack of radiological regression of thrombus, indicating the absence of macro- or microscopic response, although a single study reported no association between macroscopic appearances and microscopic response 25 . In the present study, no assumptions were made about the comparability of patients given standard or extended courses of chemotherapy.…”

Section: Discussionmentioning

confidence: 99%

Impact of neoadjuvant chemotherapy on thrombus viability in patients with Wilms tumour and caval extension: systematic review with meta-analysis

et al. 2021

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Background Inferior vena cava (IVC) tumour thrombus in children with Wilms tumour is typically managed with neoadjuvant chemotherapy with the intention of achieving thrombus regression in order to minimize the risks associated with complex vascular surgery. Methods A systematic review of Medline and Embase databases was undertaken to identify all eligible studies with reference to thrombus viability in Wilms tumour index cases with caval/cardiac extension. A meta-analysis of proportions was utilized for pooled thrombus viability data across studies. Logistic regression was used to analyse the relationship between thrombus viability and duration of chemotherapy. Results Thirty-five eligible observational studies and case reports met inclusion criteria describing a total of 236 patients with thrombus viability data. The pooled proportion of patients with viable tumour thrombus after neoadjuvant chemotherapy was 0.53 (0.43–0.63). Logistic regression analysis of 54 patients receiving either a standard (4–6 weeks) or extended (more than 6 weeks) course of neoadjuvant chemotherapy resulted in an odds ratio of 3.14 (95 per cent c.i. 0.97 to 10.16), P = 0.056, with extended course therapy trending towards viable tumour thrombus. Conclusion Preoperative chemotherapy is successful in achieving non-viability of caval and cardiac thrombi in around 50 per cent of children, without added benefit from extended cycles of neoadjuvant chemotherapy. Risks versus benefits of extirpative vascular surgery must be considered, therefore, for these high-risk patients.

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“…In 4-10% of patients with WT, the tumor extends into the renal vein or inferior vena cava, whereas an extension into the right atrium or ventricle occurs in around 1-3% of cases (43,(54)(55)(56)(57). A complete picture of a possible intravascular extension is essential for planning anesthesia and surgery.…”

Section: Intravascular Extensionmentioning

confidence: 99%

Multidisciplinary Treatment Strategies for Wilms Tumor: Recent Advances, Technical Innovations and Future Directions

et al. 2022

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Significant progress has been made in the management of Wilms tumor (WT) in recent years, mostly as a result of collaborative efforts and the implementation of protocol-driven, multimodal therapy. This article offers a comprehensive overview of current multidisciplinary treatment strategies for WT, whilst also addressing recent technical innovations including nephron-sparing surgery (NSS) and minimally invasive approaches. In addition, surgical concepts for the treatment of metastatic disease, advances in tumor imaging technology and potentially prognostic biomarkers will be discussed. Current evidence suggests that, in experienced hands and selected cases, laparoscopic radical nephrectomy and laparoscopic-assisted partial nephrectomy for WT may offer the same outcome as the traditional open approach. While NSS is the standard procedure for bilateral WT, NSS has evolved as an alternative technique in patients with smaller unilateral WT and in cases with imminent renal failure. Metastatic disease of the lung or liver that is associated with WT is preferably treated with a three-drug chemotherapy and local radiation therapy. However, surgical sampling of lung nodules may be advisable in persistent nodules before whole lung irradiation is commenced. Several tumor markers such as loss of heterozygosity of chromosomes 1p/16q, 11p15 and gain of function at 1q are associated with an increased risk of recurrence or a decreased risk of overall survival in patients with WT. In summary, complete resection with tumor-free margins remains the primary surgical aim in WT, while NSS and minimally invasive approaches are only suitable in a subset of patients with smaller WT and low-risk disease. In the future, advances in tumor imaging technology may assist the surgeon in defining surgical resection margins and additional biomarkers may emerge as targets for development of new diagnostic tests and potential therapies.

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Management and outcome of pediatric Wilms tumor with malignant inferior Vena cava thrombus: largest cohort of single-center experience

Cited by 18 publications

References 13 publications

Management of Pediatric Tumors With Vascular Extension

Management of Pediatric Tumors With Vascular Extension

Impact of neoadjuvant chemotherapy on thrombus viability in patients with Wilms tumour and caval extension: systematic review with meta-analysis

Multidisciplinary Treatment Strategies for Wilms Tumor: Recent Advances, Technical Innovations and Future Directions

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