Intracardiac Extension of Wilms Tumor: A Case of a 2.5-Year-Old Girl Presenting with Upper Venous Congestion Caused by Tumor Growth into the Right Cardiac Ventricle

Abstract

While Wilms tumors (WT) typically present solely with an abdominally palpable mass, rare cases exhibiting vascular tumor growth can also present with circulatory problems. Here, we report the case of a 2.5-year-old girl presenting with upper venous congestion and arterial hypertension as the primary symptoms of intraventricular tumor growth exhibiting remarkable tubular and perfused morphology. Clinical situation stabilized after initiation of neoadjuvant chemotherapy (NAC) with actinomycin D and vincristine, followed by surgical resection via laparotomy and sternotomy supported by cardiopulmonary bypass and deep hypothermia. Our results highlight the previously reported feasibility of this approach, even in primarily unstable patients.

Keywords: Cardiopulmonary bypass; Intracardiac tumor extent; Intravascular tumor extent; Neoadjuvant chemotherapy; Wilms Tumor.

Fig. 1

A–C. Coronal CT reconstruction in early venous phase after i.v. contrast application, depicting primary tumor and vascular extent. D–F. Ultrasound images in Doppler mode highlighting hepatoatrial transition (D) as well as atrial dilation and movement of the sacciforme intravascular tumor extension over the tricuspid valve into the right cardiac ventricle (E and F). All depicted images represent the initial diagnosis.

Fig. 2

A. Intravascular tumor extensions along centimeter scale: * subhepatic VCI; ** intrahepatic VCI; *** intracardiac. B. Dissected kidney along centimeter scale.