{"frequencyBands":{"HP:0040280":[100,100],"HP:0040281":[80,99],"HP:0040282":[30,79],"HP:0040283":[5,29],"HP:0040284":[1,4],"HP:0040285":[0,0]},"page":{"columns":[{"gene":"","key":"MONDO:0008679/OMIM:194070","label":"Wilms tumor 1","mondoId":"MONDO:0008679","onsetQualifier":null,"recordKeys":["a00a08cda39cdbf188c1b4c1eb67fa2981f456fa96ebb3045561c96de0563ad8"],"recordQualifiers":{},"scopeLabel":"OMIM:194070","sourceIds":["OMIM:194070"],"sourceScope":"This column retains the native source entry. It is not a pooled estimate for the umbrella condition."},{"gene":"","key":"MONDO:0008680/OMIM:194071","label":"Multiple tumor-associated chromosome region 1","mondoId":"MONDO:0008680","onsetQualifier":null,"recordKeys":["6d531fbc4ff1ab83599f91dac6ec25ad3d188bb54381de21db15a1adbdedaf79"],"recordQualifiers":{},"scopeLabel":"OMIM:194071","sourceIds":["OMIM:194071"],"sourceScope":"This column retains the native source entry. It is not a pooled estimate for the umbrella condition."},{"gene":"","key":"MONDO:0008683/OMIM:194090","label":"Wilms tumor 3","mondoId":"MONDO:0008683","onsetQualifier":null,"recordKeys":["aab13896e39710e4dce1ec75a2ffc815f818a873c64137e435ef7e6b2c1388a0"],"recordQualifiers":{},"scopeLabel":"OMIM:194090","sourceIds":["OMIM:194090"],"sourceScope":"This column retains the native source entry. It is not a pooled estimate for the umbrella condition."},{"gene":"","key":"MONDO:0011056/OMIM:601363","label":"Wilms tumor 4","mondoId":"MONDO:0011056","onsetQualifier":null,"recordKeys":["2ca261b0bfa5138ef541c7023affd5221fa918e09bef606fa61584c8b94408a4"],"recordQualifiers":{},"scopeLabel":"OMIM:601363","sourceIds":["OMIM:601363"],"sourceScope":"This column retains the native source entry. It is not a pooled estimate for the umbrella condition."},{"gene":"","key":"MONDO:0011112/OMIM:601583","label":"Wilms tumor 5","mondoId":"MONDO:0011112","onsetQualifier":null,"recordKeys":["5dd1430a4e909f3233eeaf585212ea9f5c8297bd0d127708e879121ba0b4e69f"],"recordQualifiers":{},"scopeLabel":"OMIM:601583","sourceIds":["OMIM:601583"],"sourceScope":"This column retains the native source entry. It is not a pooled estimate for the umbrella condition."},{"gene":"","key":"MONDO:0014779/OMIM:616806","label":"Wilms tumor 6","mondoId":"MONDO:0014779","onsetQualifier":null,"recordKeys":["37fa770f3c496ac43264b1dba64191daa50e2c890b930fb39223c1fc62d4979b"],"recordQualifiers":{},"scopeLabel":"OMIM:616806","sourceIds":["OMIM:616806"],"sourceScope":"This column retains the native source entry. It is not a pooled estimate for the umbrella condition."},{"gene":"","key":"MONDO:0979876/OMIM:621332","label":"Wilms tumor 7","mondoId":"MONDO:0979876","onsetQualifier":null,"recordKeys":["3ab1185997c91ee512cb583b306a9a80723e040bfc96374635e60345ba9b082c"],"recordQualifiers":{},"scopeLabel":"OMIM:621332","sourceIds":["OMIM:621332"],"sourceScope":"This column retains the native source entry. It is not a pooled estimate for the umbrella condition."},{"gene":"","key":"MONDO:0019004/ORPHA:654","label":"Nephroblastoma","mondoId":"MONDO:0019004","onsetQualifier":null,"recordKeys":["0ba1c96da4028c7c68aee5df434a5ac458c05acb58192a5cf505dde29f397a01","18024a9de7be3cca046aa6f278943010f235d4daa63c984b78c8fa1c7dfa9e07","19a9766e860348dd4d88dc7fca4042bb40a67075f9bb967c0509835a0bc86ae6","1fd2d39335bd9ae8989b3b1c351ce016e4639591041d490e8c2b576be1dca573","20c3763df8b2b3eccb8f968e7c1c3bb2a5361dae88660b40c14ac76f326433b0","22a5a0e96fd0def1c1016562654fc9d70f2f29c6a2158a316bc02a7820fe0400","38877d69bcb676890c71fddd5a6d08a8794e8a8c169571030e6e0b67cd1edb94","3c2286463693cfb13650e86e60d779fc1d7c6f704c42947f1b9cff8fb90798e3","5053b9b4a7cd38ce47256db090357bcc45d37fdbc1231a3c847b57ac44e0a5ae","522466ce673d5501d7ce40a4e834b02a45457b49eac532f8f7bf0544abc2e82a","55532340ecfe6d8ee8772d048e5459ce544c480ac88003597afc371259c29cf9","6ad81d5fe51b5bd01596d7a712a86a853ddd91771a84dd14609315b185b56129","6bed644dcb85a51d390b3fdcf6652dcf91873382016b057e8b0917b511f71e24","6ff079730bad0a16441685da2dac6d1b030561d3ebfd52327fe0f6d220108db6","7a0775017a0bcc45360dacc80e25e537c5a06d4a49d4dc70d85f13ad359c47e4","894cbffdef088545b146d40ad6bd611b9b768a997b06bc8fa439037db722b4a5","9259215bb789ce3de4ffa74936f120d963b7f77587f3013508f8d52374bd3854","933a29de97f5ddeb80957200d2cd14e404646f696ebb1328ede7928062e0fb09","9a04df77a46517f5cbc0f05b9cb595cc6ae1e91fd91c23e9809db4997b1625c8","a35ee1ec62a328c3c4b949f32ee62ab31cfead942fd77014d29a57db8f0111df","a78c8f617d29c33ea42b07c7a340cc5f5723b3534eb38d89b73d98c05b48bf7f","aee7dd90c707b8e8e46b02fa035654dda4bb0ff54bc9d20a2baac546710936db","c6b7c0db8bb601b774d871bd9fecb4d527b9cdbcf8b7311efb4caa0b409916d0","c756d6747480463544e6a2de0d8cd58922e64e082e631285685e994e7fd38eb1","da610b3fc159236105e2e367b1cab04188651cb3bcb6a8efce45e0e21310ec46","e77b312d5ebbcc1927e8b3a996afa366f4d8c1d9a9e0e1db8bc625e5ac779001","eeafadbee1ae0778ccb214f2ad1a0d6e35e7012ab5365baf479bd7d838308223"],"recordQualifiers":{},"scopeLabel":"ORPHA:654","sourceIds":["ORPHA:654"],"sourceScope":"This column retains the native source entry. It is not a pooled estimate for the umbrella condition."}],"comparison":{"collapseColumnsLabel":"Show initial comparisons","defaultColumnKeys":["MONDO:0008679/OMIM:194070","MONDO:0008680/OMIM:194071","MONDO:0008683/OMIM:194090","MONDO:0011056/OMIM:601363"],"expandColumnsLabel":"Compare all source entries","intro":"Compare the original source entries. Different entries may describe different patient groups.","primaryFeatureIds":[],"scopeNoteHtml":"<p>Each column keeps its native condition scope. Initial features are displayed alphabetically, not ranked by clinical importance. Counts remain separate, and an empty cell does not mean a feature cannot occur.</p>"},"coverage":{"countRecords":1,"emptyColumnKeys":[],"hpoRelease":"2026-06-23","narrativeSourceChanges":[],"records":34,"sourceGroups":{"omim":5,"orphanet":27,"published":2}},"features":[{"hpoId":"HP:0031500","label":"Abdominal mass","primary":false},{"hpoId":"HP:0002027","label":"Abdominal pain","primary":false},{"hpoId":"HP:0031105","label":"Abnormal uterus morphology","primary":false},{"hpoId":"HP:0001903","label":"Anemia","primary":false},{"hpoId":"HP:0000526","label":"Aniridia","primary":false},{"hpoId":"HP:0000028","label":"Cryptorchidism","primary":false},{"hpoId":"HP:0005580","label":"Duplication of renal pelvis","primary":false},{"hpoId":"HP:0002094","label":"Dyspnea","primary":false},{"hpoId":"HP:0000086","label":"Ectopic kidney","primary":false},{"hpoId":"HP:0001945","label":"Fever","primary":false},{"hpoId":"HP:0001528","label":"Hemihypertrophy","primary":false},{"hpoId":"HP:0000085","label":"Horseshoe kidney","primary":false},{"hpoId":"HP:0003072","label":"Hypercalcemia","primary":false},{"hpoId":"HP:0000822","label":"Hypertension","primary":false},{"hpoId":"HP:0000047","label":"Hypospadias","primary":false},{"hpoId":"HP:0002716","label":"Lymphadenopathy","primary":false},{"hpoId":"HP:0012587","label":"Macroscopic hematuria","primary":false},{"hpoId":"HP:0033834","label":"Malaise","primary":false},{"hpoId":"HP:0002907","label":"Microscopic hematuria","primary":false},{"hpoId":"HP:0002664","label":"Neoplasm","primary":false},{"hpoId":"HP:0002896","label":"Neoplasm of the liver","primary":false},{"hpoId":"HP:0100526","label":"Neoplasm of the lung","primary":false},{"hpoId":"HP:0002667","label":"Nephroblastoma","primary":false},{"hpoId":"HP:0001901","label":"Polycythemia","primary":false},{"hpoId":"HP:0008330","label":"Reduced von Willebrand factor activity","primary":false},{"hpoId":"HP:0012871","label":"Varicocele","primary":false},{"hpoId":"HP:0001824","label":"Weight loss","primary":false}],"ledeHtml":"Wilms tumor is a form of kidney cancer that primarily develops in children. Nearly all cases of Wilms tumor are diagnosed before the age of 10, with two-thirds being found before age 5.","mondoId":"MONDO:0006058","records":[{"frequencyKind":"range","key":"0ba1c96da4028c7c68aee5df434a5ac458c05acb58192a5cf505dde29f397a01","raw":{"aspect":"P","body_system":"blood","disease_id":"ORPHA:654","disease_name":"Nephroblastoma","evidence":"TAS","frequency_raw":"HP:0040283","hpo_id":"HP:0008330","is_excluded":false,"label":"Reduced von Willebrand factor activity","onset_id":null,"reference":"ORPHA:654","source_release":"2026-06-23"},"sourceGroups":["orphanet"]},{"frequencyKind":"range","key":"18024a9de7be3cca046aa6f278943010f235d4daa63c984b78c8fa1c7dfa9e07","raw":{"aspect":"P","body_system":"genitourinary","disease_id":"ORPHA:654","disease_name":"Nephroblastoma","evidence":"TAS","frequency_raw":"HP:0040283","hpo_id":"HP:0000047","is_excluded":false,"label":"Hypospadias","onset_id":null,"reference":"ORPHA:654","source_release":"2026-06-23"},"sourceGroups":["orphanet"]},{"frequencyKind":"range","key":"19a9766e860348dd4d88dc7fca4042bb40a67075f9bb967c0509835a0bc86ae6","raw":{"aspect":"P","body_system":"other","disease_id":"ORPHA:654","disease_name":"Nephroblastoma","evidence":"TAS","frequency_raw":"HP:0040283","hpo_id":"HP:0033834","is_excluded":false,"label":"Malaise","onset_id":null,"reference":"ORPHA:654","source_release":"2026-06-23"},"sourceGroups":["orphanet"]},{"frequencyKind":"range","key":"1fd2d39335bd9ae8989b3b1c351ce016e4639591041d490e8c2b576be1dca573","raw":{"aspect":"P","body_system":"genitourinary","disease_id":"ORPHA:654","disease_name":"Nephroblastoma","evidence":"TAS","frequency_raw":"HP:0040283","hpo_id":"HP:0012871","is_excluded":false,"label":"Varicocele","onset_id":null,"reference":"ORPHA:654","source_release":"2026-06-23"},"sourceGroups":["orphanet"]},{"frequencyKind":"range","key":"20c3763df8b2b3eccb8f968e7c1c3bb2a5361dae88660b40c14ac76f326433b0","raw":{"aspect":"P","body_system":"digestive","disease_id":"ORPHA:654","disease_name":"Nephroblastoma","evidence":"TAS","frequency_raw":"HP:0040282","hpo_id":"HP:0005580","is_excluded":false,"label":"Duplication of renal pelvis","onset_id":null,"reference":"ORPHA:654","source_release":"2026-06-23"},"sourceGroups":["orphanet"]},{"frequencyKind":"range","key":"22a5a0e96fd0def1c1016562654fc9d70f2f29c6a2158a316bc02a7820fe0400","raw":{"aspect":"P","body_system":"immune","disease_id":"ORPHA:654","disease_name":"Nephroblastoma","evidence":"TAS","frequency_raw":"HP:0040283","hpo_id":"HP:0002716","is_excluded":false,"label":"Lymphadenopathy","onset_id":null,"reference":"ORPHA:654","source_release":"2026-06-23"},"sourceGroups":["orphanet"]},{"frequencyKind":"reported","key":"2ca261b0bfa5138ef541c7023affd5221fa918e09bef606fa61584c8b94408a4","raw":{"aspect":"P","body_system":"neoplasm","disease_id":"OMIM:601363","disease_name":"Wilms tumor 4","evidence":"IEA","frequency_raw":null,"hpo_id":"HP:0002667","is_excluded":false,"label":"Nephroblastoma","onset_id":null,"reference":"OMIM:601363","source_release":"2026-06-23"},"sourceGroups":["omim"]},{"frequencyKind":"reported","key":"37fa770f3c496ac43264b1dba64191daa50e2c890b930fb39223c1fc62d4979b","raw":{"aspect":"P","body_system":"neoplasm","disease_id":"OMIM:616806","disease_name":"Wilms tumor 6","evidence":"PCS","frequency_raw":null,"hpo_id":"HP:0002667","is_excluded":false,"label":"Nephroblastoma","onset_id":null,"reference":"PMID:26551668","source_release":"2026-06-23"},"sourceGroups":["published"]},{"frequencyKind":"range","key":"38877d69bcb676890c71fddd5a6d08a8794e8a8c169571030e6e0b67cd1edb94","raw":{"aspect":"P","body_system":"neoplasm","disease_id":"ORPHA:654","disease_name":"Nephroblastoma","evidence":"TAS","frequency_raw":"HP:0040281","hpo_id":"HP:0002664","is_excluded":false,"label":"Neoplasm","onset_id":null,"reference":"ORPHA:654","source_release":"2026-06-23"},"sourceGroups":["orphanet"]},{"frequencyKind":"count","key":"3ab1185997c91ee512cb583b306a9a80723e040bfc96374635e60345ba9b082c","raw":{"aspect":"P","body_system":"neoplasm","disease_id":"OMIM:621332","disease_name":"Wilms tumor 7","evidence":"PCS","frequency_raw":"5/5","hpo_id":"HP:0002667","is_excluded":false,"label":"Nephroblastoma","onset_id":null,"reference":"PMID:29912901","source_release":"2026-06-23"},"sourceGroups":["published"]},{"frequencyKind":"range","key":"3c2286463693cfb13650e86e60d779fc1d7c6f704c42947f1b9cff8fb90798e3","raw":{"aspect":"P","body_system":"genitourinary","disease_id":"ORPHA:654","disease_name":"Nephroblastoma","evidence":"TAS","frequency_raw":"HP:0040283","hpo_id":"HP:0031105","is_excluded":false,"label":"Abnormal uterus morphology","onset_id":null,"reference":"ORPHA:654","source_release":"2026-06-23"},"sourceGroups":["orphanet"]},{"frequencyKind":"range","key":"5053b9b4a7cd38ce47256db090357bcc45d37fdbc1231a3c847b57ac44e0a5ae","raw":{"aspect":"P","body_system":"cardiovascular","disease_id":"ORPHA:654","disease_name":"Nephroblastoma","evidence":"TAS","frequency_raw":"HP:0040283","hpo_id":"HP:0000822","is_excluded":false,"label":"Hypertension","onset_id":null,"reference":"ORPHA:654","source_release":"2026-06-23"},"sourceGroups":["orphanet"]},{"frequencyKind":"range","key":"522466ce673d5501d7ce40a4e834b02a45457b49eac532f8f7bf0544abc2e82a","raw":{"aspect":"P","body_system":"genitourinary","disease_id":"ORPHA:654","disease_name":"Nephroblastoma","evidence":"TAS","frequency_raw":"HP:0040283","hpo_id":"HP:0000028","is_excluded":false,"label":"Cryptorchidism","onset_id":null,"reference":"ORPHA:654","source_release":"2026-06-23"},"sourceGroups":["orphanet"]},{"frequencyKind":"range","key":"55532340ecfe6d8ee8772d048e5459ce544c480ac88003597afc371259c29cf9","raw":{"aspect":"P","body_system":"respiratory","disease_id":"ORPHA:654","disease_name":"Nephroblastoma","evidence":"TAS","frequency_raw":"HP:0040284","hpo_id":"HP:0002094","is_excluded":false,"label":"Dyspnea","onset_id":null,"reference":"ORPHA:654","source_release":"2026-06-23"},"sourceGroups":["orphanet"]},{"frequencyKind":"reported","key":"5dd1430a4e909f3233eeaf585212ea9f5c8297bd0d127708e879121ba0b4e69f","raw":{"aspect":"P","body_system":"neoplasm","disease_id":"OMIM:601583","disease_name":"Wilms tumor 5","evidence":"IEA","frequency_raw":null,"hpo_id":"HP:0002667","is_excluded":false,"label":"Nephroblastoma","onset_id":null,"reference":"OMIM:601583","source_release":"2026-06-23"},"sourceGroups":["omim"]},{"frequencyKind":"range","key":"6ad81d5fe51b5bd01596d7a712a86a853ddd91771a84dd14609315b185b56129","raw":{"aspect":"P","body_system":"metabolic","disease_id":"ORPHA:654","disease_name":"Nephroblastoma","evidence":"TAS","frequency_raw":"HP:0040283","hpo_id":"HP:0002907","is_excluded":false,"label":"Microscopic hematuria","onset_id":null,"reference":"ORPHA:654","source_release":"2026-06-23"},"sourceGroups":["orphanet"]},{"frequencyKind":"range","key":"6bed644dcb85a51d390b3fdcf6652dcf91873382016b057e8b0917b511f71e24","raw":{"aspect":"P","body_system":"genitourinary","disease_id":"ORPHA:654","disease_name":"Nephroblastoma","evidence":"TAS","frequency_raw":"HP:0040283","hpo_id":"HP:0000085","is_excluded":false,"label":"Horseshoe kidney","onset_id":null,"reference":"ORPHA:654","source_release":"2026-06-23"},"sourceGroups":["orphanet"]},{"frequencyKind":"reported","key":"6d531fbc4ff1ab83599f91dac6ec25ad3d188bb54381de21db15a1adbdedaf79","raw":{"aspect":"P","body_system":"neoplasm","disease_id":"OMIM:194071","disease_name":"Multiple tumor-associated chromosome region 1","evidence":"IEA","frequency_raw":null,"hpo_id":"HP:0002667","is_excluded":false,"label":"Nephroblastoma","onset_id":null,"reference":"OMIM:194071","source_release":"2026-06-23"},"sourceGroups":["omim"]},{"frequencyKind":"range","key":"6ff079730bad0a16441685da2dac6d1b030561d3ebfd52327fe0f6d220108db6","raw":{"aspect":"P","body_system":"blood","disease_id":"ORPHA:654","disease_name":"Nephroblastoma","evidence":"TAS","frequency_raw":"HP:0040283","hpo_id":"HP:0001901","is_excluded":false,"label":"Polycythemia","onset_id":null,"reference":"ORPHA:654","source_release":"2026-06-23"},"sourceGroups":["orphanet"]},{"frequencyKind":"range","key":"7a0775017a0bcc45360dacc80e25e537c5a06d4a49d4dc70d85f13ad359c47e4","raw":{"aspect":"P","body_system":"metabolic","disease_id":"ORPHA:654","disease_name":"Nephroblastoma","evidence":"TAS","frequency_raw":"HP:0040283","hpo_id":"HP:0001945","is_excluded":false,"label":"Fever","onset_id":null,"reference":"ORPHA:654","source_release":"2026-06-23"},"sourceGroups":["orphanet"]},{"frequencyKind":"range","key":"894cbffdef088545b146d40ad6bd611b9b768a997b06bc8fa439037db722b4a5","raw":{"aspect":"P","body_system":"digestive","disease_id":"ORPHA:654","disease_name":"Nephroblastoma","evidence":"TAS","frequency_raw":"HP:0040281","hpo_id":"HP:0031500","is_excluded":false,"label":"Abdominal mass","onset_id":null,"reference":"ORPHA:654","source_release":"2026-06-23"},"sourceGroups":["orphanet"]},{"frequencyKind":"range","key":"9259215bb789ce3de4ffa74936f120d963b7f77587f3013508f8d52374bd3854","raw":{"aspect":"P","body_system":"other","disease_id":"ORPHA:654","disease_name":"Nephroblastoma","evidence":"TAS","frequency_raw":"HP:0040282","hpo_id":"HP:0002027","is_excluded":false,"label":"Abdominal pain","onset_id":null,"reference":"ORPHA:654","source_release":"2026-06-23"},"sourceGroups":["orphanet"]},{"frequencyKind":"range","key":"933a29de97f5ddeb80957200d2cd14e404646f696ebb1328ede7928062e0fb09","raw":{"aspect":"P","body_system":"growth","disease_id":"ORPHA:654","disease_name":"Nephroblastoma","evidence":"TAS","frequency_raw":"HP:0040283","hpo_id":"HP:0001824","is_excluded":false,"label":"Weight loss","onset_id":null,"reference":"ORPHA:654","source_release":"2026-06-23"},"sourceGroups":["orphanet"]},{"frequencyKind":"range","key":"9a04df77a46517f5cbc0f05b9cb595cc6ae1e91fd91c23e9809db4997b1625c8","raw":{"aspect":"P","body_system":"eye","disease_id":"ORPHA:654","disease_name":"Nephroblastoma","evidence":"TAS","frequency_raw":"HP:0040283","hpo_id":"HP:0000526","is_excluded":false,"label":"Aniridia","onset_id":null,"reference":"ORPHA:654","source_release":"2026-06-23"},"sourceGroups":["orphanet"]},{"frequencyKind":"reported","key":"a00a08cda39cdbf188c1b4c1eb67fa2981f456fa96ebb3045561c96de0563ad8","raw":{"aspect":"P","body_system":"neoplasm","disease_id":"OMIM:194070","disease_name":"Wilms tumor 1","evidence":"IEA","frequency_raw":null,"hpo_id":"HP:0002667","is_excluded":false,"label":"Nephroblastoma","onset_id":null,"reference":"OMIM:194070","source_release":"2026-06-23"},"sourceGroups":["omim"]},{"frequencyKind":"range","key":"a35ee1ec62a328c3c4b949f32ee62ab31cfead942fd77014d29a57db8f0111df","raw":{"aspect":"P","body_system":"musculoskeletal","disease_id":"ORPHA:654","disease_name":"Nephroblastoma","evidence":"TAS","frequency_raw":"HP:0040283","hpo_id":"HP:0001528","is_excluded":false,"label":"Hemihypertrophy","onset_id":null,"reference":"ORPHA:654","source_release":"2026-06-23"},"sourceGroups":["orphanet"]},{"frequencyKind":"range","key":"a78c8f617d29c33ea42b07c7a340cc5f5723b3534eb38d89b73d98c05b48bf7f","raw":{"aspect":"P","body_system":"blood","disease_id":"ORPHA:654","disease_name":"Nephroblastoma","evidence":"TAS","frequency_raw":"HP:0040283","hpo_id":"HP:0001903","is_excluded":false,"label":"Anemia","onset_id":null,"reference":"ORPHA:654","source_release":"2026-06-23"},"sourceGroups":["orphanet"]},{"frequencyKind":"reported","key":"aab13896e39710e4dce1ec75a2ffc815f818a873c64137e435ef7e6b2c1388a0","raw":{"aspect":"P","body_system":"neoplasm","disease_id":"OMIM:194090","disease_name":"Wilms tumor 3","evidence":"IEA","frequency_raw":null,"hpo_id":"HP:0002667","is_excluded":false,"label":"Nephroblastoma","onset_id":null,"reference":"OMIM:194090","source_release":"2026-06-23"},"sourceGroups":["omim"]},{"frequencyKind":"range","key":"aee7dd90c707b8e8e46b02fa035654dda4bb0ff54bc9d20a2baac546710936db","raw":{"aspect":"P","body_system":"neoplasm","disease_id":"ORPHA:654","disease_name":"Nephroblastoma","evidence":"TAS","frequency_raw":"HP:0040281","hpo_id":"HP:0002667","is_excluded":false,"label":"Nephroblastoma","onset_id":null,"reference":"ORPHA:654","source_release":"2026-06-23"},"sourceGroups":["orphanet"]},{"frequencyKind":"range","key":"c6b7c0db8bb601b774d871bd9fecb4d527b9cdbcf8b7311efb4caa0b409916d0","raw":{"aspect":"P","body_system":"metabolic","disease_id":"ORPHA:654","disease_name":"Nephroblastoma","evidence":"TAS","frequency_raw":"HP:0040283","hpo_id":"HP:0003072","is_excluded":false,"label":"Hypercalcemia","onset_id":null,"reference":"ORPHA:654","source_release":"2026-06-23"},"sourceGroups":["orphanet"]},{"frequencyKind":"range","key":"c756d6747480463544e6a2de0d8cd58922e64e082e631285685e994e7fd38eb1","raw":{"aspect":"P","body_system":"respiratory","disease_id":"ORPHA:654","disease_name":"Nephroblastoma","evidence":"TAS","frequency_raw":"HP:0040283","hpo_id":"HP:0100526","is_excluded":false,"label":"Neoplasm of the lung","onset_id":null,"reference":"ORPHA:654","source_release":"2026-06-23"},"sourceGroups":["orphanet"]},{"frequencyKind":"range","key":"da610b3fc159236105e2e367b1cab04188651cb3bcb6a8efce45e0e21310ec46","raw":{"aspect":"P","body_system":"genitourinary","disease_id":"ORPHA:654","disease_name":"Nephroblastoma","evidence":"TAS","frequency_raw":"HP:0040283","hpo_id":"HP:0000086","is_excluded":false,"label":"Ectopic kidney","onset_id":null,"reference":"ORPHA:654","source_release":"2026-06-23"},"sourceGroups":["orphanet"]},{"frequencyKind":"range","key":"e77b312d5ebbcc1927e8b3a996afa366f4d8c1d9a9e0e1db8bc625e5ac779001","raw":{"aspect":"P","body_system":"digestive","disease_id":"ORPHA:654","disease_name":"Nephroblastoma","evidence":"TAS","frequency_raw":"HP:0040283","hpo_id":"HP:0002896","is_excluded":false,"label":"Neoplasm of the liver","onset_id":null,"reference":"ORPHA:654","source_release":"2026-06-23"},"sourceGroups":["orphanet"]},{"frequencyKind":"range","key":"eeafadbee1ae0778ccb214f2ad1a0d6e35e7012ab5365baf479bd7d838308223","raw":{"aspect":"P","body_system":"metabolic","disease_id":"ORPHA:654","disease_name":"Nephroblastoma","evidence":"TAS","frequency_raw":"HP:0040282","hpo_id":"HP:0012587","is_excluded":false,"label":"Macroscopic hematuria","onset_id":null,"reference":"ORPHA:654","source_release":"2026-06-23"},"sourceGroups":["orphanet"]}],"referenceLabels":{},"references":{"causeHtml":"<ul><li><a href=\"https://medlineplus.gov/genetics/condition/wilms-tumor\">Wilms tumor</a></li></ul>","identityNote":"MONDO cross-references identify native condition entries. Ancestor membership does not transfer phenotype values between groups.","importantHtml":""},"sections":[{"bodyHtml":"<p>Changes in any of several genes are involved in the formation of Wilms tumor. Wilms tumor is often associated with variants (also called mutations) in the WT1 gene, CTNNB1 gene, or AMER1 gene. These genes provide instructions for making proteins that regulate gene activity and promote the growth and division (proliferation) of cells. WT1, CTNNB1, and AMER1 gene variants all lead to the unchecked proliferation of cells, allowing tumor development.</p><p>Changes on the short (p) arm of chromosome 11 are also associated with developing Wilms tumor. Two genes in this area, IGF2 and H19, are either turned on or off depending on whether the copy of the gene was inherited from the mother or the father. This parent-specific difference in gene activation is a phenomenon called genomic imprinting. In some cases of Wilms tumor, abnormalities in the process of genomic imprinting on chromosome 11 lead to a loss of H19 gene activity and increased activity of the IGF2 gene in kidney cells. The resulting loss of H19 gene activity, which normally restrains cell growth, and increase in IGF2 gene activity, which promotes cell growth, together lead to uncontrolled cell growth and tumor development in people with Wilms tumor.</p><p>In most cases of Wilms tumors involving one kidney and nearly all cases involving both kidneys, the tumors are thought to arise from immature kidney tissue that never developed properly. These immature tissues are known as nephrogenic rests. It is likely that genetic changes are involved in the presence of nephrogenic rests and that additional genetic changes trigger nephrogenic rests to develop into a tumor.</p><p>Genetic conditions that share a genetic cause with Wilms tumor can also have this cancer as a feature. These conditions include WAGR syndrome, Denys-Drash syndrome, and Frasier syndrome, which are caused by variants in the WT1 gene. Wilms tumor has also been seen in individuals with Beckwith-Wiedemann syndrome, which can be caused by changes in the genomic imprinting of the IGF2 and H19 genes. Wilms tumor can be a feature of other genetic conditions caused by variants in other genes.</p><p>Many children with Wilms tumor do not have identified variants in any of the known genes. In these cases, the cause of the condition is unknown. It is likely that other, unknown genes are also associated with the development of Wilms tumor.</p><p class=\"section-source\"><a href=\"https://medlineplus.gov/genetics/condition/wilms-tumor/#causes\">MedlinePlus Genetics: Causes</a></p>","key":"forms","title":"What causes Wilms tumor?"},{"bodyHtml":"<p>Wilms tumor is often first noticed because of abdominal swelling or a mass in the kidney that can be felt upon physical examination. Some affected children have abdominal pain, fever, a low number of red blood cells (anemia), blood in the urine (hematuria), or high blood pressure (hypertension). Additional signs of Wilms tumor can include loss of appetite, weight loss, nausea, vomiting, and tiredness (lethargy).</p><p>Wilms tumor can develop in one or both kidneys. About 5 to 10 percent of affected individuals develop multiple tumors in one or both kidneys. Wilms tumor may spread from the kidneys to other parts of the body (metastasize). In rare cases, Wilms tumor does not involve the kidneys and occurs instead in the genital tract, bladder, abdomen, chest, or lower back. It is unclear how Wilms tumor develops in these tissues.</p><p>With proper treatment, children with Wilms tumor have a 90 percent survival rate. However, the risk that the cancer will come back (recur) is between 15 and 50 percent, depending on traits of the original tumor. Tumors usually recur in the first 2 years following treatment and develop in the kidneys or other tissues, such as the lungs. Individuals who have had Wilms tumor may experience related health problems or late effects of their treatment in adulthood, such as decreased kidney function, heart disease, and development of additional cancers.</p><p class=\"section-source\">Source: <a href=\"https://medlineplus.gov/genetics/condition/wilms-tumor\">MedlinePlus Genetics</a></p>","key":"overview","title":"Condition overview"}],"sharedSources":[],"slug":"wilms-tumor","sourceDocuments":[{"name":"Wilms tumor","reviewed":"2018-09","sections":{"causes":{"fetchedAt":"2026-10-20T20:25:50.218607+00:00","label":"Causes","pageSha256":"f2a0e2b70e0d4bb3ad34ed7cea65d72f693a6ca460628922da6bc9671ac9c709","sourceUrl":"https://medlineplus.gov/genetics/condition/wilms-tumor/#causes","status":"available"}},"source_release":"2026-10-01","url":"https://medlineplus.gov/genetics/condition/wilms-tumor"}],"sourceNote":"MedlinePlus descriptions and Causes; original HPO annotations.","takeawayHtml":"Cause explanations are from MedlinePlus Genetics. Feature values below retain their original sources and patient groups.","title":"Wilms tumor"},"schemaVersion":1}