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Management of Pediatric Tumors With Vascular Extension
Mayara Caroline Amorim Fanelli1, José Cícero Stocco Guilhen2, Alexandre Alberto Barros Duarte1
1Department of Pediatric Surgery, Pediatric Oncology Institute, Grupo de Apoio ao Adolescente e à Criança com Câncer (GRAACC), São Paulo, Brazil.
Insights
Pediatric tumors with vascular extension require tailored surgical strategies to ensure safe resection and avoid complications like embolization. A proposed protocol guides surgical decisions based on tumor extension and imaging, aiming to minimize the need for cardiac bypass.
Area of Science:
- Pediatric Surgery
- Surgical Oncology
- Vascular Surgery
Background:
- Pediatric tumors, including Wilms tumor, adrenal tumors, and hepatoblastomas, can extend into the inferior vena cava and right atrium, complicating surgical management.
- Vascular extension poses risks of severe bleeding, cardiac arrest, and embolization, necessitating careful surgical planning to ensure patient safety and optimize resection outcomes.
Purpose of the Study:
- To delineate effective surgical strategies for managing pediatric tumors with vascular extension.
- To propose a decision-making protocol to guide surgical approaches and minimize operative risks.
Main Methods:
- Retrospective review of patient cases with vascular tumor extension at a single institution.
- Utilized imaging studies (CT, MRI, Doppler US) for preoperative evaluation of tumor extent and thrombus characteristics.
- Developed a decision-making flowchart based on tumor thrombus level, guiding surgical techniques and the potential need for cardiopulmonary bypass.
Main Results:
- A comprehensive decision-making protocol was established for the surgical treatment of pediatric tumors with vascular extension.
- Neoadjuvant chemotherapy was frequently employed to reduce tumor extension and embolization risk, potentially avoiding cardiac bypass.
Conclusions:
- Vascular extension significantly impacts surgical strategy in pediatric tumors, requiring specialized awareness and management.
- Adherence to a structured protocol and treatment in reference centers are crucial for optimizing outcomes and preventing complications.
Abstract:
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.
