11.12Prognosis

•Before area of radiotherapy and chemotherapy, surgery only: survival rate 20–40%

•After multidisciplinary approaches according to tumor stage and standard therapy, 85–90% cure rate

•Prognosis depends on age, stage, histology, cytogenetics, and response to chemotherapy

Prognosis according to stage

•Unfavorable factors:

–Diffuse anaplasia

–Viable malignant cells after preoperative chemotherapy

–Infiltration of tumor capsule

–Invasion of tumor cells into vessels

–Non-radical surgical resection of tumor

–Tumor rupture (also after biopsy)

–Metastatic spread

–Large tumor volume

–Histology: rhabdoid tumor

–Molecular genetics: alteration of loss of heterozygosity on 1p, 11q, 16q, and 22q; p53 mutations

–Children with lung metastasis have a better prognosis than those with other metastases

11.13 Metastatic Nephroblastoma

•Ten percent of children present with stage 4 and bilateral synchronous tumors occur in 4–7% of them

•Kidney-sparing surgery after initial chemotherapy is the recommended procedure

•Prognosis 70–80% event-free survival after 4 years. Renal failure may lead to kidney transplantation

11.14Subtypes

11.14.1Bilateral Wilms Tumor (Stage 5)

•Incidence 4–7%

•Often initially bilateral tumor characterized by:

– Mean age: 15 months (unilateral Wilms tumor: 42 months)