Prognosis

Favorable-histology Wilms tumors are associated with very high 5-year overall survival rates of >90%.[82][103] Patients with tumor weight <550 g and age <2 years also have excellent outcomes.[104][105] However, there is an increased risk of treatment-related morbidity and mortality 25 years from diagnosis.[106]

Presence of anaplasia, aneuploidy, distant metastatic disease (especially to the lymph node and/or hepatic metastasis and/or intravascular tumor thrombus), and loss of heterozygosity (LOH) at 1p and 16q confers poor prognosis.[44][61][82][95]​​​[107][108]​​​​[109][110][111]

Lymph-node involvement and the presence of microscopic residual disease are highly predictive of event-free survival and overall survival in patients with stage III favorable-histology disease.[112]

Four-year event free survival for all stage IV favorable-histology Wilms tumor patients treated on NWTSG-5 is 75%.[82] The 5-year relapse-free survival for stage IV patients enrolled on the SIOP-9 trial receiving preoperative chemotherapy is 73%.[113] Prognosis is considered excellent in patients with stage IV disease if histology was favorable, metastatic disease was isolated to either the liver or lungs, and metastases responded to treatment with preoperative chemotherapy.[114] Prognosis is inferior for patients with high-risk stage IV tumors; the overall 5-year survival rates are 17% for blastermal type stage IV Wilms tumor and 16% for diffuse anaplastic stage IV Wilms tumor.[115]

Recurrence rates are low.[82] Adverse prognostic factors for patients with recurrent tumors include previous treatment with doxorubicin, relapses that occur <12 months after diagnosis and intra-abdominal relapse after abdominal irradiation.[82]​​[97][116]​​ Prognosis for patients with recurrence is poor.

Poor outcomes are observed in patients with Denys-Drash syndrome as the disease is often bilateral and associated with progressive renal failure.[47]​[77]​​[117] However, overall survival is improved with successful renal transplant.[118]

One study found that Hispanic ethnicity may be associated with poorer prognosis; however, further research on ethnic disparities in Wilms tumor is required.[119]

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