Treatment of Pulmonary Metastases in Children With Stage IV Nephroblastoma With Risk-Based Use of Pulmonary Radiotherapy

Abstract

Following the SIOP protocol, pulmonary RT can be omitted for a majority of patients with PM and results in a relatively good outcome.

Rationale for the treatment of Wilms tumour in the UMBRELLA SIOP–RTSG 2016 protocol

“…Overall, ~17% of patients with Wilms tumours present with stage IV disease at diagnosis, which is defined as haematogenous metastases to the lungs, liver, or other sites, or extra-abdominal lymph node metastases. Pulmonary metastases are by far the most frequently observed [21][22][23] . The increasing use of chest CT as routine imaging for staging has resulted in the detection of small pulmonary nodules not visible on chest radiography (CT-only nodules).…”

“…Similar to SIOP−2001, preoperative treatment for metastatic (stage IV) disease in the UMBRELLA protocol includes a combined vincristine, actinomycin D, and doxo rubicin regimen for 6 weeks, followed by reassessment imaging of local tumour (using MRI) and metastatic sites (using CT and/or MRI) before surgery. With this preoperative regimen, 61-67% of patients have complete metastatic response before surgery 21,23 . Detailed guidelines are provided for the stratification of postoperative chemotherapy, in which the cumulative dose of doxo rubicin has been lowered in order to reduce cardiac toxicity.…”

“…Pulmonary radiotherapy is administered for lung metastases lacking complete response by postoperative week 10. Evidence suggests that the majority of patients achieving a complete response after induction chemotherapy with or without surgery do not need radiotherapy to the lungs, as they have excellent survival even without radiotherapy (5-year EFS 84%, 5-year OS 92%) 21 . Patients with viable metastases at surgery or highrisk histology, both of which are asso ciated with poor survival of <40%, are the exception and receive radiotherapy to the lungs 23 .…”

Wilms Tumor: An Example Of Risk-Adapted and Well Tolerated Therapy

“…The current approach for treatment of WT in North America is to perform surgery followed by risk-based chemotherapy. The treatment strategy in Europe consists of initial neoadjuvant chemotherapy, then nephrectomy and postoperative chemotherapy, and sometimes radiotherapy [7]. Radiation is very effective, but now used only in higher risk situations.…”

A Large Mass in Lung in a 5-Year-Old Child ? Wilm’s Tumour with Pulmonary Metastasis

“…3 According to the International Society of Paediatrics Oncology (SIOP), treatment strategy consists of multiple agents chemotherapy, surgery and sometimes radiotherapy. 4 In pulmonary metastasis disease free survival may vary from two to three years after initial treatment. …”