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.

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

“…It was also not possible to define fully how neoadjuvant radiotherapy influenced thrombus viability or to separate these patients from the series where they were reported, which made exclusion of these studies from the sensitivity analysis necessary. Resection margins were not often discussed, and it remains unclear whether incomplete thrombectomy increases the risk of disease relapse 1 , 2 , 14 , 15 , 24 , 43 , 51 . Adjuvant radiotherapy was usually given in these situations as mandated by tumour-staging protocols, but postoperative chemotherapy, relapse and mortality outcomes were often generalized, and it was difficult to define fully relationships between these and thrombus response and viability.…”

Outcomes of incomplete thrombectomy in Wilms tumour with intravascular extension: A commentary

“…Surgeons attempt thrombectomy with reportedly good outcomes, with relatively few studies alleging thrombus left in situ. [2][3][4][5] However, where full extirpative resection is considered unfavourable, disease is then upstaged with adjuvant caval irradiation mandated by current UK WT and Children's Oncology Group (COG) guidelines. It is currently unclear whether this strategy achieves sufficient disease control to make caval surgery unnecessary.…”

Inferior vena cava and right atrial thrombosis in children with embryonal tumors

“…Гистологически верифицирована нефробластома смешанного гистологического типа, промежуточная группа риска. Ребенок получил адъювантную химиотерапию и лучевую терапию в дозе 25,2 Гр с удовлетворительным результатом [30].…”