The management of nephroblastoma with cavo-atrial disease at presentation: experience from a developing country

To describe the management of cavoatrial extension of nephroblastoma in a developing country. Of 406 consecutive children with Wilms tumour, 40 (10%) presented with tumour extension into the inferior vena cava (IVC) (30), right atrium or right ventricle and form the inception cohort. Of this group, 17 (43%) had visceral metastases and two had bilateral synchronous tumours. Neoadjuvant chemotherapy appropriate to the pre-operative stage caused reduction in intravascular tumour in 18 (45%) patients, allowing 3 children who had atrial tumour at presentation to be surgically managed without recourse to cardiopulmonary bypass. Neoadjuvant chemotherapy also provided a window of opportunity to assess and manage co-morbidity especially malnutrition. Of the inception cohort, 31 (78%) underwent surgical resection; 23 had simple cavotomy, one caval resection and seven resection under cardiopulmonary bypass. There was one post-operative death. 77% of resected specimen contained viable malignant cells despite one or more cycles of neoadjuvant chemotherapy. Retrograde extension into caval tributaries threatened the completeness of resection in all patients necessitating the addition of post-operative radiotherapy. Of nine patients who did not undergo resection, five died pre-operatively. Three of these children died of chemotherapy induced neutropaenic sepsis. Four patients refused surgical treatment. Twenty-three patients completed the post-operative treatment protocol (1,203 characters).