ObjectiveWilms tumour (WT) patients with a localised completely necrotic nephroblastoma after preoperative chemotherapy are a favourable outcome group. Since the introduction of the SIOP 2001 protocol, the SIOP-Renal Tumour Study Group (SIOP-RTSG) has omitted radiotherapy for such patients with low-risk, local stage III in an attempt to reduce treatment burden. However, for metastatic patients with local stage III, completely necrotic WT, the recommendations led to ambiguous use. The purpose of this descriptive study is to demonstrate the outcomes of patients with metastatic, completely necrotic and local stage III WT in relation to the application of radiotherapy or not.
Methods and materialsAll metastatic patients with local stage III, completely necrotic WT after 6 weeks of preoperative chemotherapy who were registered in the SIOP 2001 study were included in this analysis. The pattern of recurrence according to the usage of radiation treatment and 5 year event-free survival (EFS) and overall survival (OS) was analysed.
ResultsSeven hundred and three metastatic WT patients were registered in the SIOP 2001 database.Of them, 47 patients had a completely necrotic, local stage III WT: 45 lung metastases (11 combined localisations), 1 liver/peritoneal, and 1 tumour thrombus in the renal vein and the inferior vena cava with bilateral pulmonary arterial embolism. Abdominal radiotherapy was administered in 29 patients (62%; 29 flank/abdominal irradiation and 9 combined with lung irradiation). Eighteen patients did not receive radiotherapy. Median follow-up was 6.6 years (range 1-151 months). Two of the 47 patients (4%) developed disease recurrence in the lung (one combined with abdominal relapse) and eventually died of the disease. Both patients had received abdominal radiotherapy, one of them combined with lung irradiation. Five-year EFS and OS were 95% and 95%, respectively.
ConclusionsThe outcome of patients with stage IV, local stage III, completely necrotic Wilms tumours is excellent. Our results suggest that abdominal irradiation in this patient category may not be of added value in first-line treatment, consistent with the current recommendation in the SIOP-RTSG 2016 UMBRELLA protocol. Outcome of stage IV completely necrotic Wilms tumour and local stage III treated according to the SIOP-2001 protocol | 41 Whole abdominal RT:CTV: This includes the entire abdominal contents and peritoneum extending from the dome of the diaphragm to the pelvic floor (lower border of obturator foramen).
Pulmonary RT:CTV: This encompasses both lungs including the apices and costo-diaphragmatic recesses. If abdominal radiotherapy also has to be given, both fields should be matched in order to avoid any gap or overlap.
Liver RT:CTV: This includes the extent of incompletely resected tumour with a margin of 2 cm.
RT for brain metastases:CTV: the whole brain is treated.
RT for haematogenous metastases to bone:CTV: For bone metastases it is not necessary to treat the entire bone. The field includes the obvious disease visible on imaging ex...