{"entity":{"id":"wilms-risk-markers","kind":"term","name":"Wilms tumour risk markers (anaplasia, 1p/16q loss, 1q gain, SIOP and COG risk groups)","aka":["anaplastic Wilms tumour","diffuse anaplasia","focal anaplasia","favourable histology Wilms tumour","unfavourable histology","1p/16q loss of heterozygosity","LOH 1p and 16q","1q gain in Wilms tumour","blastemal-type Wilms tumour","SIOP risk group","SIOP UMBRELLA","COG renal tumour risk group","very low risk Wilms tumour","bilateral Wilms tumour","nephrogenic rests","11p15 loss of heterozygosity"],"tldr":"Wilms tumour is cured in nine of ten children, so its markers exist to decide who needs less treatment and who needs more: anaplastic cells under the microscope, loss of chromosome pieces 1p and 16q or gain of 1q in the tumour DNA, and, in Europe, how much blastemal tumour survives the pre-operative chemotherapy.","summary":"What is measured: the risk that a Wilms tumour will relapse. How: histology (favourable versus focal or diffuse anaplasia, the latter tied to TP53 mutation; in the SIOP system, which operates after four to six weeks of vincristine and actinomycin, the post-chemotherapy picture is graded low risk when completely necrotic, intermediate for regressive, epithelial, stromal, mixed and focal anaplastic types, and high risk for diffuse anaplasia and blastemal-type tumours), tumour DNA for loss of heterozygosity at 1p and 16q (in the COG system, loss of both in favourable-histology stages I to IV means augmented therapy), 1q gain (about 30 percent, adverse, now built into COG and SIOP UMBRELLA protocols), 11p15 loss of heterozygosity and TP53. Very low risk in COG is stage I favourable histology under age 2 with a tumour under 550 g, treated by nephrectomy alone. Germline testing (WT1, 11p15 imprinting disorders such as Beckwith-Wiedemann, DIS3L2) is offered when the tumour is bilateral, syndromic or in an infant, and bilateral disease is managed with nephron-sparing surgery. What a result changes: the intensity climbs from vincristine and actinomycin alone, through the addition of doxorubicin, to cyclophosphamide, carboplatin and etoposide with radiotherapy for anaplastic and other high-risk disease. Where it matters: Wilms tumour.","asOf":"2026-09-17","links":[],"tags":[],"related":["tp53-mutated","cytogenetics","vincristine","dactinomycin","doxorubicin","hereditary-cancer-syndromes","germline-testing"],"cancers":["wilms-tumor"],"sections":[],"technologies":[],"targets":[],"drugs":[],"companies":[],"institutions":[],"pathways":[],"terms":[],"trials":[],"people":[],"bottlenecks":[],"keyPapers":[],"journals":[],"dependsOn":[],"notes":[],"category":"Biomarkers"},"route":"/terms/wilms-risk-markers/","neighbours":{"term":[{"id":"cytogenetics","kind":"term","name":"Cytogenetics and karyotype","route":"/terms/cytogenetics/"},{"id":"hereditary-cancer-syndromes","kind":"term","name":"Hereditary cancer syndromes","route":"/terms/hereditary-cancer-syndromes/"},{"id":"tp53-mutated","kind":"term","name":"TP53-mutated (p53-abnormal)","route":"/terms/tp53-mutated/"}],"drug":[{"id":"dactinomycin","kind":"drug","name":"Dactinomycin (actinomycin D)","route":"/drugs/dactinomycin/"},{"id":"doxorubicin","kind":"drug","name":"Doxorubicin","route":"/drugs/doxorubicin/"},{"id":"vincristine","kind":"drug","name":"Vincristine","route":"/drugs/vincristine/"}],"technology":[{"id":"germline-testing","kind":"technology","name":"Germline (hereditary) testing","route":"/technologies/germline-testing/"}],"cancer":[{"id":"wilms-tumor","kind":"cancer","name":"Wilms tumour (nephroblastoma)","route":"/cancers/wilms-tumor/"}]}}