Lessons from a patient with cardiac arrest due to massive pulmonary embolism as the initial presentation of Wilms tumor: a case report and literature review
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Finding an abdominal mass or hematuria is the initial step in diagnosing Wilms tumor. As the first manifestation of Wilms tumor, it is exceedingly rare for pulmonary tumor embolism to present with cardiac arrest. A case of a patient whose sudden cardiac arrest due to massive pulmonary tumor embolism of Wilms tumor was not responsive to resuscitation is presented.
The patient was a five-year-old girl who collapsed suddenly during activity in nursery school and went into cardiac arrest in the ambulance. Unfortunately, she was not responsive to conventional resuscitation. A judicial autopsy conducted at the local police department showed the main cause of her sudden cardiac arrest was attributed to multiple pulmonary tumor embolisms of stage IV Wilms tumor.
Except for one reported case, treatments were not successful in all eight cardiac arrest cases with pulmonary tumor embolism of Wilms tumor. These results indicate that it is challenging not only to make an accurate diagnosis, but also to provide proper specific treatment in the cardiac arrest setting. We propose that flexible triage and prompt transfer to a tertiary hospital are necessary as an oncologic emergency to get such patients to bridging therapy combined with extracorporeal membrane oxygenation or immediate surgical intervention under cardiopulmonary bypass.
KeywordsCase report Wilms tumor Massive pulmonary tumor embolism Cardiac arrest
Extracorporeal membrane oxygenation
Extracorporeal cardiopulmonary resuscitation
A judicial autopsy conducted at the local police department showed:  the weight of the Wilms tumor that originated in the right kidney was 885 g, while the left kidney weighed 100 g, and no further histological examination was performed (Fig. 1b);  tumor extended into the right renal vein, IVC, and entry portion of the right atrium (Fig. 1c); and  greyish or dark red small multiple emboli filled the right and left peripheral pulmonary arteries (Fig. 1d and e). Taken together, the main cause of her sudden cardiac arrest was attributed to multiple pulmonary tumor embolisms secondary to stage IV Wilms tumor.
Discussion and conclusions
Non-neonatal pediatric ECMO cases with a variety of indications have increased . In the past tumor-related reports, ECPR was performed for a patient with metastatic choriocarcinoma, a patient with lymphoma, and another patient with leukemia who developed PE [13, 14, 15]. The case with choriocarcinoma presented with severe dyspnea, massive hemoptysis, and decreased mean BP in the hospital. Venoarterial (VA) ECMO was used to restore hemodynamic stability. Subsequently, the patient was successfully cured with a pulmonary embolectomy and chemotherapy under ECMO . Although the third case was not successfully resuscitated even with ECMO support and emergency surgery in the hospital , both cases were started on ECMO before cardiac arrest. A patient with lymphoma who had a cardiac arrest for 8 min and achieved successful recovery of spontaneous circulation received bridging ECMO support, leading to discharge without any other serious complications . We suggest that ECMO at tertiary hospitals should be considered for potentially fatal cases of massive pulmonary tumor embolism of Wilms tumor before or immediately after cardiac arrest (Fig. 2b) [16, 17, 18]. Also, it is important to note that the femoral route as access for ECPR should be avoided in a patient with massive pulmonary tumor embolism because of infiltration of Wilms tumor into the IVC. Thus, V-A ECMO draining via the internal jugular venous cannula or immediate surgical intervention under cardiopulmonary bypass might have been a potential option for the present patient.
Some centers have developed PE teams involving hematology, ED staff, intensive care staff, cardiologists, and pediatric surgeons . Prompt treatment by the in-house multidisciplinary team is essential for the treatment of similar cases as an oncologic emergency . We also expect that development of immediate onsite triage combined with portable echocardiogram would provide a better strategy for initial selection and set up for potential use of ECPR systems [15, 21, 22, 23] (Fig. 2b). Ketelaars et al. showed that prehospital chest ultrasound on an air emergency medical service can flexibly alter the destination and improve treatment decisions for adult patients . Based on our experience, we suggest that the destination decision to a tertiary hospital and a prompt transfer system would be a critical first step for transporting the patient to an ECPR center or immediate surgery in a patient with fatal massive pulmonary tumor embolism of Wilms tumor (Fig. 2b). Further experience will be needed to determine how best to get a patient with this type of oncologic emergency to conventional therapy.
The authors would like to thank Dr. Yasuaki Koyama of the Department of Emergency and Critical Care Medicine at Tsukuba University and Dr. Masatoshi Takagi of the Department of Pediatrics at Tokyo Medical and Dental University for helpful discussion.
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All data are contained in the manuscript.
AF collected data. TI, NS, KN, and TO gave conceptual advice. AF and TI wrote the manuscript. All authors read and approved the final manuscript.
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