Guzy wilmsa
Epidemiologia

Guzy Wilmsa (nephroblastoma) stanowią najczęstszy złośliwy nowotwór nerki u dzieci, odpowiadając za 85-95% przypadków nowotworów nerek w tej grupie wiekowej oraz 5-7% wszystkich nowotworów dziecięcych. Roczna częstość występowania wynosi około 7-10,4 przypadków na milion dzieci poniżej 15. roku życia, z wyraźnymi różnicami geograficznymi i etnicznymi – najczęściej u osób pochodzenia afrykańskiego (>10/milion), rzadziej u rasy białej (6-9/milion) i najrzadziej u wschodnioazjatyckiej (3-4/milion). Mediana wieku rozpoznania to 3-4 lata, z wcześniejszym występowaniem w przypadkach obustronnych i zespołach genetycznych (np. WAGR, Denys-Drash, Beckwitha-Wiedemanna). Nadzór przesiewowy za pomocą USG nerek co 3-4 miesiące jest zalecany u dzieci z ryzykiem ≥1-5%, szczególnie do 5-7 roku życia, w zależności od zespołu predysponującego. Wskazane jest prowadzenie badań przesiewowych po konsultacji genetycznej, a wykryte zmiany powinny być leczone w ośrodkach specjalistycznych.

Epidemiologia guzów Wilmsa

Guzy Wilmsa (nephroblastoma) stanowią najczęstszy typ nowotworu złośliwego nerki u dzieci, odpowiadając za około 85-95% przypadków nowotworów nerki w tej grupie wiekowej. Nowotwór ten reprezentuje około 5-7% wszystkich nowotworów dziecięcych, co czyni go czwartym najczęstszym nowotworem złośliwym wieku dziecięcego.123

Każdego roku w Stanach Zjednoczonych diagnozuje się około 600-650 nowych przypadków guzów Wilmsa. Częstość występowania tego nowotworu wynosi około 7-10,4 przypadków na milion dzieci poniżej 15. roku życia.456 W Europie i Ameryce Północnej guzy Wilmsa dotykają około 1 na 10 000 dzieci.78

Zróżnicowanie geograficzne

Występowanie guzów Wilmsa wykazuje znaczne zróżnicowanie geograficzne i etniczne. Nowotwór ten występuje:910

  • Najczęściej u osób pochodzenia afrykańskiego (ponad 10 przypadków na milion osobolat)
  • Na średnim poziomie u osób rasy białej (6-9 przypadków na milion osobolat)
  • Najrzadziej u osób pochodzenia wschodnioazjatyckiego (3-4 przypadki na milion osobolat)

1112

Kraje o niskich dochodach mają wyższą medianę częstości występowania guzów Wilmsa (9,8 przypadków na milion, standaryzowanych według wieku) niż kraje o wysokich dochodach (8,6 przypadków na milion) i średnich dochodach (6,1 przypadków na milion).13 W Afryce częstość występowania szacuje się na 9,8 przypadków na milion, z gorszym wskaźnikiem przeżycia całkowitego wynoszącym około 25%.14

Rozkład wiekowy

Guzy Wilmsa występują głównie u małych dzieci. Około 80% przypadków diagnozuje się przed 5. rokiem życia. Średni lub mediana wieku w momencie rozpoznania wynosi 3-4 lata.151617

Istnieją jednak wyraźne różnice w zależności od typu guza i czynników etnicznych:1819

  • Mediana wieku dla jednostronnych, jednoogniskowych guzów wynosi 36,1 miesiąca (około 3 lat)
  • Mediana wieku dla synchronicznych obustronnych guzów Wilmsa jest niższa i wynosi 25,5 miesiąca (około 2,1 roku)
  • Przypadki sporadyczne z jednostronnymi guzami występują w starszym wieku (mediana 36 miesięcy) niż rodzinne synchroniczne obustronne guzy Wilmsa (mediana 26 miesięcy)
  • U dzieci azjatyckich guzy Wilmsa występują znacznie wcześniej, często w drugim roku życia, a nawet do 40% przypadków diagnozuje się u niemowląt poniżej 1. roku życia

2021

Gdy guz Wilmsa występuje jako część zespołu genetycznego, pojawia się jeszcze wcześniej, zazwyczaj między 2. a 24. miesiącem życia.22

Rozkład płci

W przypadku jednostronnych guzów Wilmsa stosunek płci męskiej do żeńskiej wynosi 0,92:1,00, co oznacza niewielką przewagę występowania u dziewczynek. Natomiast w przypadkach obustronnych guzów Wilmsa przewaga żeńska jest jeszcze bardziej wyraźna, ze stosunkiem płci męskiej do żeńskiej wynoszącym 0,60:1,00.232425

Ciekawe, że w Azji Południowo-Wschodniej obserwuje się nadmiar przypadków u chłopców, co stanowi wyjątek od ogólnego trendu.26

Zespoły predyspozycji i guzy Wilmsa

Chociaż większość guzów Wilmsa (około 90%) występuje sporadycznie u zdrowych dzieci bez żadnych predyspozycji do rozwoju raka, około 10-15% przypadków jest związanych z określonymi zespołami wad wrodzonych lub innymi nieprawidłowościami genetycznymi.272829

Główne zespoły predyspozycji

Do najważniejszych zespołów predyspozycji związanych z guzami Wilmsa należą:3031

  • Zespół WAGR (Wilms tumor, Aniridia, Genitourinary anomalies, mental Retardation) – związany z delecją genu supresorowego WT1 (11p13), charakteryzuje się najwyższym ryzykiem rozwoju guzów Wilmsa, sięgającym 45-60%
  • Zespół Denys-Drash – związany z mutacjami genu WT1, ryzyko wynosi około 90%
  • Zespół Beckwitha-Wiedemanna (BWS) – związany z mutacjami w klastrze genów WT2 (11p15.5), ryzyko wynosi około 10%, choć może się różnić w zależności od konkretnej zmiany genetycznej
  • Zespół Perlmana – ryzyko wynosi około 75%
  • Zespół Simpsona-Golabiego-Behmela (SGBS)
  • Izolowana hemihipertrofia (asymetryczny rozrost części ciała)
  • Sporadyczna aniridia (brak tęczówki)

323334

U pacjentów z tymi zespołami guzy Wilmsa mają tendencję do występowania we wcześniejszym wieku, częściej są obustronne i wieloogniskowe w porównaniu do przypadków sporadycznych.35

Nadzór i badania przesiewowe

Zalecenia dotyczące nadzoru

Regularny nadzór u dzieci uważanych za zagrożone zwiększonym ryzykiem rozwoju guzów Wilmsa stał się powszechny w Wielkiej Brytanii, USA i części Europy. Jednak nadal istnieje niewiele dowodów dotyczących skuteczności badań przesiewowych lub równowagi potencjalnych zagrożeń i korzyści.3637

Badania przesiewowe mają na celu wcześniejsze wykrycie małego i zlokalizowanego guza (stadium I lub II), poprawę rokowania i zastosowanie mniej intensywnego leczenia (np. umożliwienie operacji oszczędzającej nefron).3839

Obecne zalecenia obejmują:4041

  • Badania przesiewowe powinny być oferowane dzieciom z ryzykiem rozwoju guzów Wilmsa wynoszącym co najmniej 1-5%
  • Badania przesiewowe powinny być oferowane dopiero po konsultacji z genetykiem klinicznym
  • Nadzór powinien być prowadzony za pomocą ultrasonografii nerek co 3-4 miesiące
  • Badania przesiewowe powinny być kontynuowane do 5. roku życia w przypadku większości zespołów, a do 7. roku życia w przypadku zespołu Beckwitha-Wiedemanna, zespołu Simpsona-Golabiego-Behmela i niektórych rodzinnych przypadków guzów Wilmsa
  • Nadzór może być prowadzony w lokalnym ośrodku, ale powinien być wykonywany przez osobę doświadczoną w ultrasonografii pediatrycznej
  • Zmiany wykryte podczas badań przesiewowych powinny być leczone w ośrodku specjalistycznym

4243

Amerykańskie Stowarzyszenie Badań nad Rakiem (AACR) zaleca badania przesiewowe u wszystkich dzieci z ryzykiem rozwoju guzów Wilmsa większym niż 1%. Panel NCCN zaleca, aby badania przesiewowe obejmowały badanie fizykalne i ultrasonografię nerek co 3 miesiące do co najmniej 8. roku życia, na podstawie dostępnych danych i doświadczenia klinicznego.44

Kontrowersje dotyczące badań przesiewowych

Potencjalne ryzyko i korzyści związane z badaniami przesiewowymi w kierunku guzów Wilmsa są delikatnie zrównoważone i nie ma jednoznacznych dowodów na to, że badania przesiewowe zmniejszają śmiertelność lub chorobowość.45

Istnieją różnice w praktykach między USA a Europą dotyczące badań przesiewowych. Amerykańskie Stowarzyszenie Badań nad Rakiem (AACR) przyznaje, że istnieje różnica w środowiskach praktyki między Stanami Zjednoczonymi a Europą.4647

Niektóre ośrodki europejskie wdrożyły zróżnicowane podejście do badań przesiewowych w oparciu o różnice ryzyka między różnymi genetycznymi lub epigenetycznymi przyczynami każdego zespołu. Natomiast zalecenia amerykańskie są bardziej ujednolicone.48

Specjalne przypadki nadzoru

W przypadku zespołu WAGR, który charakteryzuje się wysokim ryzykiem guzów Wilmsa, zaleca się regularne badania przesiewowe w kierunku guzów Wilmsa od urodzenia lub momentu diagnozy. Badania te polegają na badaniach ultrasonograficznych jamy brzusznej co 3 miesiące do 8. roku życia.49

Chociaż rzadko, guzy Wilmsa zgłaszano u osób z zespołem WAGR nawet do 19. roku życia. Z tego powodu monitorowanie w kierunku guzów Wilmsa powinno być kontynuowane przez całe życie. Ten nadzór może obejmować okresowe badania ultrasonograficzne lub rezonans magnetyczny oraz monitorowanie objawów, takich jak masa w jamie brzusznej, wysokie ciśnienie krwi i krew w moczu.50

W przypadku zespołu Beckwitha-Wiedemanna (BWS) z utratą metylacji w centrum imprintingu 2 (IC2), niektóre niedawne publikacje proponowały zaprzestanie nadzoru onkologicznego. Jednak stwierdzono, że ryzyko guzów Wilmsa w tej populacji nie jest tak niskie, jak wcześniej sądzono. Dlatego zaleca się, aby wszystkie dzieci z klinicznym lub molekularnym rozpoznaniem BWS były badane w kierunku guzów Wilmsa za pomocą ultrasonografii jamy brzusznej do 8. roku życia, niezależnie od klasyfikacji molekularnej.5152

Obserwacja po leczeniu

Po zakończeniu leczenia guzów Wilmsa, dzieci nadal wymagają regularnych wizyt w poradni. Podczas tych wizyt specjalista będzie poszukiwał wszelkich oznak nawrotu nowotworu (znanego jako wznowa). Stworzony zostaje zwykle spersonalizowany plan nadzoru, który określa konkretne długoterminowe obserwacje i badania, których dziecko będzie wymagało.5354

W obecnej dekadzie, wraz z pojawieniem się terapii wielomodalnej, ogólne wskaźniki przeżycia wynoszą około 90% dla dzieci z guzem Wilmsa, głównie ze względu na postęp w terapii chemoradioterapeutycznej oraz dbałość o długoterminową obserwację poprzez unikanie późnych efektów wtórnych.55

Children’s Oncology Group (COG) opracowała długoterminowe wytyczne dotyczące obserwacji osób, które przeżyły nowotwory dziecięce, takie jak guzy Wilmsa. Wytyczne te zostały stworzone, aby pomóc zwiększyć świadomość późnych efektów i poprawić opiekę po zakończeniu leczenia u osób, które przeżyły nowotwory dziecięce przez całe ich życie.56

Bardzo ważne jest omówienie możliwych długoterminowych powikłań z zespołem opieki zdrowotnej dziecka. Należy upewnić się, że istnieje plan monitorowania tych problemów i ich leczenia, jeśli to konieczne.5758

Kontrowersje dotyczące obserwacji obrazowej

Mimo zagrożeń i kosztów, stosowanie tomografii komputerowej (TK) do rutynowej obserwacji w celu wykrycia nawrotu u pacjentów z guzem Wilmsa wzrosło w ostatnich latach. Children’s Oncology Group opublikowała artykuł w Journal of Clinical Oncology badający wpływ obrazowania obserwacyjnego na pacjentów z guzem Wilmsa.59

Lekarze doszli do wniosku, że wyeliminowanie skanów TK z programów nadzoru w przypadku jednostronnego guza Wilmsa o korzystnej histologii prawdopodobnie nie zagrozi przeżyciu. Może to jednak skutkować znacznie mniejszą ekspozycją na promieniowanie i niższymi kosztami opieki zdrowotnej. Ogólnie rzecz biorąc, należy wziąć pod uwagę stosunek ryzyka do korzyści związanych z metodami obrazowania i formalnie zbadać go dla wszystkich nowotworów pediatrycznych.60

Wskaźniki przeżycia i rokowanie

Rokowanie w przypadku guzów Wilmsa znacznie się poprawiło w ciągu ostatnich dekad, a obecne podejścia terapeutyczne zapewniają doskonałe wyniki dla większości pacjentów. Ogólny wskaźnik przeżycia dla guzów Wilmsa jest wysoki – około 90% pacjentów przeżywa co najmniej 5 lat po rozpoznaniu. Można to przypisać skutecznym terapiom wielomodalnym, w tym chirurgii, chemioterapii i radioterapii.616263

Wskaźniki przeżycia różnią się w zależności od stadium i histologii guza:646566

  • Dzieci zdiagnozowane z guzem Wilmsa we wczesnym stadium o korzystnej histologii mają 4-letni wskaźnik przeżycia wynoszący 95-100%
  • Wskaźniki przeżycia według stadium wynoszą 95% u pacjentów w stadiach I i II, 75-80% w stadium III i 65-75% w stadium IV
  • Wskaźniki przeżycia dla pacjentów z guzem Wilmsa o korzystnej histologii zlokalizowanym w nerce przekraczają 90%
  • Pacjenci z anaplastycznymi, zlokalizowanymi guzami mają 5-letni wskaźnik przeżycia wynoszący około 70-75% po leczeniu chirurgicznym, chemioterapii i radioterapii

Oprócz zaawansowanego stadium choroby i histologii anaplastycznej, starszy wiek w momencie rozpoznania i niepełna odpowiedź na początkowe leczenie również są związane z gorszym rokowaniem. Pacjenci z tymi czynnikami mogą wymagać bardziej intensywnych terapii i ścisłego monitorowania w celu poprawy wyników.676869

Badanie z Korei wykazało 10-letni ogólny wskaźnik przeżycia wynoszący 89,1%, ze wskaźnikiem nawrotu wynoszącym 12%. Wskaźniki przeżycia w zależności od płci, stadium klinicznego, typu patologicznego i nawrotu były następujące: 88,6% u mężczyzn, 90,9% u kobiet, 100% w stadium I, 94,7% w stadium II, 92,1% w stadium III, 63,4% w stadium IV, 85,7% w stadium V, 95,3% w przypadku korzystnej histologii, 64,1% w przypadku niekorzystnej histologii, 94,8% bez nawrotu i 40,9% w przypadku nawrotu.70

Ostatnie badanie wykazało, że ogólne wskaźniki przeżycia dla całej kohorty w 5 i 10 lat wynoszą odpowiednio 93,6% i 92,5%. Zaobserwowano znaczny spadek przeżycia specyficznego dla raka (CSS) wraz z rosnącym rozprzestrzenianiem się guzów. Pacjenci z odległym rozsiewem mieli wyraźnie gorszy CSS, podobnie jak osoby z rozsiewem regionalnym i osoby z nieznanym stadium w porównaniu do pacjentów z guzami zlokalizowanymi.71

Zróżnicowanie wskaźników przeżycia

Przeżycie pacjentów z guzem Wilmsa jest silnie uzależnione od ich wieku i stadium nowotworu w momencie rozpoznania, przy czym wskaźniki przeżycia znacznie maleją wraz z postępem choroby do wyższych stadiów. Ponadto istotne różnice w wskaźnikach przeżycia utrzymują się między różnymi regionami i krajami, co wskazuje na potrzebę dalszych wysiłków w celu zmniejszenia tych rozbieżności.72

Pacjenci w wieku 15 lat i starsi wykazywali 10-letni CSS wynoszący 73,4%, w przeciwieństwie do pacjentów w wieku 0-3 lat, którzy wykazywali CSS wynoszący 95,5%.73

Mimo że ogólne wskaźniki przeżycia są doskonałe dla dzieci z guzem Wilmsa o korzystnej histologii, przeżycie pozostaje słabe dla dzieci z guzem Wilmsa o wyższym stadium z rozlaną anaplazją.74

Czynnik Odsetek przeżycia
Stadium I i II 95%
Stadium III 75-80%
Stadium IV 65-75%
Korzystna histologia 95,3%
Niekorzystna histologia 64,1%
Pacjenci bez nawrotu 94,8%
Pacjenci z nawrotem 40,9%
Wiek 0-3 lata 95,5% (10-letni CSS)
Wiek 15+ lat 73,4% (10-letni CSS)

Wnioski i przyszłe kierunki

Guzy Wilmsa stanowią istotny problem w onkologii dziecięcej, z wyraźnymi różnicami w częstości występowania i wynikach leczenia w różnych grupach etnicznych i regionach świata. Chociaż wskaźniki przeżycia poprawiły się znacznie dzięki wielomodalnym podejściom terapeutycznym, nadal istnieją wyzwania związane z optymalizacją protokołów nadzoru dla dzieci z predyspozycją oraz poprawą wyników w przypadkach niekorzystnej histologii i zaawansowanego stadium choroby.7576

Oczekuje się, że dalsze badania doprowadzą do modyfikacji obecnych zaleceń dotyczących nadzoru, z potencjalnym zróżnicowaniem w oparciu o konkretne ryzyko molekularne i genetyczne. Badanie zróżnicowanego obciążenia późnymi efektami toksycznymi leczenia w różnych grupach rasowych i etnicznych stanowi ważny obszar przyszłych badań.7778

Niedawne postępy w leczeniu, w tym terapie celowane i udoskonalone protokoły chemioterapii, jeszcze bardziej poprawiły przeżycie, chociaż niektóre podgrupy, takie jak przypadki histologii anaplastycznej, nadal wymagają lepszych strategii.79

Podejście międzyinstytucjonalne i współpraca międzynarodowa odgrywają kluczową rolę w optymalizacji leczenia, zwiększeniu rekrutacji do badań klinicznych i zmniejszeniu nierówności w wynikach leczenia guzów Wilmsa na całym świecie.8081

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  1. 13.04.2026
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Materiały źródłowe

  • #1 Key Statistics for Wilms Tumors | American Cancer Society
    https://www.cancer.org/cancer/types/wilms-tumor/about/key-statistics.html
    Wilms tumor is the most common type of kidney cancer in children. Each year, about 600 children are diagnosed with Wilms tumors in the United States. This number has been fairly stable for many years. About 5% of all cancers in children are Wilms tumors. […] Wilms tumors tend to occur in young children. The average age of children when they are diagnosed is about 3 to 4 years. These tumors become less common as children grow older. They’re very rare in adults. […] Wilms tumors are slightly more common in girls than in boys. The risk of Wilms tumor is slightly higher in Black children than in White children and is lower among Asian American children.
  • #2 Wilms tumor | Radiology Reference Article | Radiopaedia.org
    https://radiopaedia.org/articles/wilms-tumour?embed_domain=external.radpair.com%252527%25255B0%25255D%252527%25255B0%25255D&lang=us
    Wilms tumors are the most common pediatric renal mass, accounting for over 85% of cases and account for 7% of all childhood cancers. They typically occur in early childhood (1-11 years) with peak incidence between 3 and 4 years of age. Approximately 80% of these tumors are found before the age of 5 years. When part of a syndrome, they occur even earlier, typically between 2 and 24 months of age. […] There is no recognized gender predilection, however, presentation is a little later in females. The vast majority are unilateral with 5% occurring bilaterally.
  • #3 Wilms Tumor – Diagnosis & Disease Information
    https://www.cancertherapyadvisor.com/ddi/wilms-tumor/
    Wilms tumor also known as nephroblastoma is the most common kidney cancer in children, primarily affecting those younger than age 5 years. […] The incidence of Wilms tumor in the United States is approximately 10.4 cases for every 1 million children younger than age 15 years. Wilms tumor accounts for approximately 5% of all pediatric cancers. In the United States, 500 to 600 people are diagnosed with Wilms tumor each year. The average age at diagnosis is 3 to 4 years old; Wilms tumor is exceedingly rare in adults. […] The disease is diagnosed more frequently in children who are Black compared with those who are White, and least often in children who are Asian American, with a slight predominance in girls. Advances in multimodal treatment approaches, including surgery, chemotherapy, and radiation therapy, have greatly improved survival rates.
  • #4 Wilms Tumor and Other Childhood Kidney Tumors Treatment (PDQ®) – NCI
    https://www.cancer.gov/types/kidney/hp/wilms-treatment-pdq
    Wilms tumor is the most frequent tumor of the kidney in infants and children. The incidence of Wilms tumor is 9.7 cases for every 1 million children younger than 15 years and 13.5 cases per 1 million infants. Approximately 650 cases of Wilms tumor are diagnosed in the United States each year. The incidence is substantially lower in Asian people. […] The male-to-female ratio in unilateral cases of Wilms tumor is 0.92 to 1.00, but in bilateral cases, there is a female excess (0.60). The mean age at diagnosis is 44 months in unilateral cases and 31 months in bilateral cases of Wilms tumor. About 10% of children with Wilms tumor have an associated congenital malformation syndrome. […] Wilms tumor typically develops in otherwise healthy children without any predisposition to developing cancer. However, approximately 10% of children with Wilms tumor have been reported to have a congenital anomaly.
  • #5 Clinical presentation, diagnosis, and staging of Wilms tumor – UpToDate
    https://www.uptodate.com/contents/clinical-presentation-diagnosis-and-staging-of-wilms-tumor
    Wilms tumor is the most common kidney malignancy in children, with approximately 650 new cases diagnosed in the United States each year. […] In the United States, the annual incidence of kidney tumors is approximately 10 cases per 1 million children younger than 15 years, accounting for 5 percent of all childhood malignancies and approximately 650 new cases per year. Wilms tumor is the most common kidney malignancy in children <15 years old, accounting for approximately 95 percent of all cases. [...] In contrast, renal cell carcinoma (RCC) is more common in the 15- to 19-year-old age group. [...] The incidence of Wilms tumor is the lowest in Asian children and the highest in children of African descent. [...] Wilms tumor is primarily a sporadic disease, and only 1 to 2 percent of individuals with Wilms tumor have a relative with the disease. In approximately 10 to 15 percent of cases, Wilms tumor occurs as a part of a multiple malformation syndrome, including WAGR, Denys-Drash, and Beckwith-Wiedemann syndrome.
  • #6 Wilms Tumor: Practice Essentials, Background, Etiology
    https://emedicine.medscape.com/article/989398-overview
    Wilms tumor affects approximately 10 children and adolescents per 1 million before age 15 years. Therefore, it accounts for 6-7% of all childhood cancers in North America. As a result, about 450-500 new cases are diagnosed each year on this continent. In 5-10% of patients, both kidneys are affected at the same time (synchronous bilateral Wilms tumor) or one after the other (metachronous bilateral Wilms tumor). […] Wilms tumor appears to be relatively more common in Africa and least common in East Asia. The incidence in Europe is similar to that reported in North America. Low-income countries have a higher median incidence of Wilms tumor (9.8 age-standardized rate [ASR] per million) than high-income (8.6 ASR per million) and middle-income countries (6.1 ASR per million). […] Wilms tumor is relatively more common in Blacks than in Whites and is rare in East Asians. Estimates suggest 6-9 cases per million person years in Whites, 3-4 cases per million person years in East Asians, and more than 10 cases per million person years among Black populations. Among patients with unilateral Wilms tumor enrolled in all NWTSG protocols, the male-to-female ratio was 0.92:1. For patients with bilateral disease, the male-to-female ratio was 0.60:1. The median age at diagnosis of Wilms tumor is approximately 3.5 years. The median age is highest for patients with unilateral unicentric disease (36.1 mo) and lowest for those with synchronous bilateral Wilms tumors (25.5 mo). A study by Heck et al looked to determine whether the risk of childhood cancers among Hispanic children varies by maternal birthplace. The researchers found that for certain cancer types, such as glioma and astrocytoma, solid neuroblastoma, and Wilms tumor of the kidney, children of nonUS born Hispanic mothers had the lowest risk.
  • #7 Wilms tumor: MedlinePlus GeneticsLock
    https://medlineplus.gov/genetics/condition/wilms-tumor/
    Wilms tumor is the most common kidney cancer in children. In Europe and North America, Wilms tumor affects 1 in 10,000 children. In the United States, 500 children develop Wilms tumor each year. The incidence of Wilms tumor seems to vary among populations, with African Americans having a higher-than-average risk of developing this cancer and Asians having a lower-than-average risk. […] Most cases of Wilms tumor are not caused by inherited genetic factors and do not cluster in families. Approximately 90 percent of these cancers are due to somatic variants, which means that the variants are acquired during a person’s lifetime and are present only in the tumor cells. Variants that are present in cells throughout the body (called germline variants) are responsible for the remaining 10 percent of Wilms tumor cases and cause either Wilms tumor without any other signs or symptoms or syndromes in which Wilms tumor is one of multiple features. These cases follow autosomal dominant inheritance, which means one copy of the altered gene in each cell can cause a Wilms tumor-related syndrome or increase a person’s chance of developing the cancer alone. […] Surveillance Recommendations for Children with Overgrowth Syndromes and Predisposition to Wilms Tumors and Hepatoblastoma.
  • #8 Wilms’ tumor – Wikipedia
    https://en.wikipedia.org/wiki/Wilms%27_tumor
    Wilms tumor is the most common malignant renal tumor in children. […] There are a number of rare genetic syndromes that have been linked to an increased risk of developing Wilms Tumor. […] Screening guidelines vary between countries; however health care professionals are recommending regular ultrasound screening for people with associated genetic syndromes. […] Wilms’ tumor affects approximately one person per 10,000 worldwide before the age of 15 years. […] People of African descent may have slightly higher rates of Wilms’ tumor. […] The peak age of Wilms’ tumor is 3 to 4 years and most cases occur before the age of 10 years. […] A genetic predisposition to Wilms’ tumor in individuals with aniridia has been established, due to deletions in the p13 band on chromosome 11.
  • #9 Wilms Tumor: Practice Essentials, Background, Etiology
    https://emedicine.medscape.com/article/989398-overview
    Wilms tumor affects approximately 10 children and adolescents per 1 million before age 15 years. Therefore, it accounts for 6-7% of all childhood cancers in North America. As a result, about 450-500 new cases are diagnosed each year on this continent. In 5-10% of patients, both kidneys are affected at the same time (synchronous bilateral Wilms tumor) or one after the other (metachronous bilateral Wilms tumor). […] Wilms tumor appears to be relatively more common in Africa and least common in East Asia. The incidence in Europe is similar to that reported in North America. Low-income countries have a higher median incidence of Wilms tumor (9.8 age-standardized rate [ASR] per million) than high-income (8.6 ASR per million) and middle-income countries (6.1 ASR per million). […] Wilms tumor is relatively more common in Blacks than in Whites and is rare in East Asians. Estimates suggest 6-9 cases per million person years in Whites, 3-4 cases per million person years in East Asians, and more than 10 cases per million person years among Black populations. Among patients with unilateral Wilms tumor enrolled in all NWTSG protocols, the male-to-female ratio was 0.92:1. For patients with bilateral disease, the male-to-female ratio was 0.60:1. The median age at diagnosis of Wilms tumor is approximately 3.5 years. The median age is highest for patients with unilateral unicentric disease (36.1 mo) and lowest for those with synchronous bilateral Wilms tumors (25.5 mo). A study by Heck et al looked to determine whether the risk of childhood cancers among Hispanic children varies by maternal birthplace. The researchers found that for certain cancer types, such as glioma and astrocytoma, solid neuroblastoma, and Wilms tumor of the kidney, children of nonUS born Hispanic mothers had the lowest risk.
  • #10 Wilms Tumor and Other Childhood Kidney Tumors Treatment (PDQ®) – NCI
    https://www.cancer.gov/types/kidney/hp/wilms-treatment-pdq
    Wilms tumor is the most frequent tumor of the kidney in infants and children. The incidence of Wilms tumor is 9.7 cases for every 1 million children younger than 15 years and 13.5 cases per 1 million infants. Approximately 650 cases of Wilms tumor are diagnosed in the United States each year. The incidence is substantially lower in Asian people. […] The male-to-female ratio in unilateral cases of Wilms tumor is 0.92 to 1.00, but in bilateral cases, there is a female excess (0.60). The mean age at diagnosis is 44 months in unilateral cases and 31 months in bilateral cases of Wilms tumor. About 10% of children with Wilms tumor have an associated congenital malformation syndrome. […] Wilms tumor typically develops in otherwise healthy children without any predisposition to developing cancer. However, approximately 10% of children with Wilms tumor have been reported to have a congenital anomaly.
  • #11 Key Statistics for Wilms Tumors | American Cancer Society
    https://www.cancer.org/cancer/types/wilms-tumor/about/key-statistics.html
    Wilms tumor is the most common type of kidney cancer in children. Each year, about 600 children are diagnosed with Wilms tumors in the United States. This number has been fairly stable for many years. About 5% of all cancers in children are Wilms tumors. […] Wilms tumors tend to occur in young children. The average age of children when they are diagnosed is about 3 to 4 years. These tumors become less common as children grow older. They’re very rare in adults. […] Wilms tumors are slightly more common in girls than in boys. The risk of Wilms tumor is slightly higher in Black children than in White children and is lower among Asian American children.
  • #12 Inter-Ethnic Variations in the Clinical, Pathological, and Molecular Characteristics of Wilms Tumor
    https://www.mdpi.com/2072-6694/16/17/3051
    Wilms tumor is the most common pediatric primary renal malignancy globally but exhibits significant variations in epidemiological, clinical, and molecular aspects among different populations. […] Wilms tumor occurs at a lower incidence and at a younger age in Asians compared to Caucasians and Africans. […] Reports from the 1990s first described inter-ethnic variations in the incidence of Wilms tumors—Asian Wilms tumors had lower incidences and tended to occur at a younger age, with up to 40% occurring in infants less than 1 year old, compared to Caucasian and Black populations with peak incidences around the second year of life. […] The reduced incidence of Wilms tumor in the Asian population has been hypothesized to be due to the loss of imprinting (LOI) for the Insulin-like Growth Factor 2 gene (IGF2) occurring at a lower frequency in Asians.
  • #13 Wilms Tumor: Practice Essentials, Background, Etiology
    https://emedicine.medscape.com/article/989398-overview
    Wilms tumor affects approximately 10 children and adolescents per 1 million before age 15 years. Therefore, it accounts for 6-7% of all childhood cancers in North America. As a result, about 450-500 new cases are diagnosed each year on this continent. In 5-10% of patients, both kidneys are affected at the same time (synchronous bilateral Wilms tumor) or one after the other (metachronous bilateral Wilms tumor). […] Wilms tumor appears to be relatively more common in Africa and least common in East Asia. The incidence in Europe is similar to that reported in North America. Low-income countries have a higher median incidence of Wilms tumor (9.8 age-standardized rate [ASR] per million) than high-income (8.6 ASR per million) and middle-income countries (6.1 ASR per million). […] Wilms tumor is relatively more common in Blacks than in Whites and is rare in East Asians. Estimates suggest 6-9 cases per million person years in Whites, 3-4 cases per million person years in East Asians, and more than 10 cases per million person years among Black populations. Among patients with unilateral Wilms tumor enrolled in all NWTSG protocols, the male-to-female ratio was 0.92:1. For patients with bilateral disease, the male-to-female ratio was 0.60:1. The median age at diagnosis of Wilms tumor is approximately 3.5 years. The median age is highest for patients with unilateral unicentric disease (36.1 mo) and lowest for those with synchronous bilateral Wilms tumors (25.5 mo). A study by Heck et al looked to determine whether the risk of childhood cancers among Hispanic children varies by maternal birthplace. The researchers found that for certain cancer types, such as glioma and astrocytoma, solid neuroblastoma, and Wilms tumor of the kidney, children of nonUS born Hispanic mothers had the lowest risk.
  • #14 One year overall survival of wilms tumor cases and its predictors, among children diagnosed at a teaching hospital in South Western Uganda: a retrospective cohort study | BMC Cancer | Full Text
    https://bmccancer.biomedcentral.com/articles/10.1186/s12885-023-10601-2
    The number of children diagnosed in Africa is equally high, with an incidence of 9.8 age-specific rates per million (ASR/million), conversely with a poor OS of 25%. […] In Nigeria, the stage of WT at diagnosis, age at diagnosis, histology type, frequent treatment interruptions, and size of the tumor were factors predicting OS of children diagnosed with WT. […] Unfavorable histology type was found to increase the risk of death from WT by 5.1 times (95%CI: 1.7792.50), while tumor size above 15 cm was found to increase the risk of death from WT by 6 times (1.3234.95). […] Overall survival of WT at MRRH was found to be 59.3%, and predictive significant factors noted were unfavorable histology and tumor size greater than 115 cm. Unfavorable WT histology and late stages of WT correlated with poor OS.
  • #15 Key Statistics for Wilms Tumors | American Cancer Society
    https://www.cancer.org/cancer/types/wilms-tumor/about/key-statistics.html
    Wilms tumor is the most common type of kidney cancer in children. Each year, about 600 children are diagnosed with Wilms tumors in the United States. This number has been fairly stable for many years. About 5% of all cancers in children are Wilms tumors. […] Wilms tumors tend to occur in young children. The average age of children when they are diagnosed is about 3 to 4 years. These tumors become less common as children grow older. They’re very rare in adults. […] Wilms tumors are slightly more common in girls than in boys. The risk of Wilms tumor is slightly higher in Black children than in White children and is lower among Asian American children.
  • #16 Wilms tumor | Radiology Reference Article | Radiopaedia.org
    https://radiopaedia.org/articles/wilms-tumour?embed_domain=external.radpair.com%252527%25255B0%25255D%252527%25255B0%25255D&lang=us
    Wilms tumors are the most common pediatric renal mass, accounting for over 85% of cases and account for 7% of all childhood cancers. They typically occur in early childhood (1-11 years) with peak incidence between 3 and 4 years of age. Approximately 80% of these tumors are found before the age of 5 years. When part of a syndrome, they occur even earlier, typically between 2 and 24 months of age. […] There is no recognized gender predilection, however, presentation is a little later in females. The vast majority are unilateral with 5% occurring bilaterally.
  • #17 Wilms’ tumor epidemiology and demographics – wikidoc
    https://www.wikidoc.org/index.php/Wilms%27_tumor_epidemiology_and_demographics
    Wilms tumors are the most common pediatric renal mass. The incidence of Wilms tumor is estimated to be 0.71 cases per 10,00,000 children younger than 15 years. There is no significant gender predilection among unilateral cases, but the male to female ratio in bilateral cases is 0.60:1.00. It typically occurs in early childhood with peak incidence between 3 and 4 years of age. Asian individuals are less likely to develop Wilms tumor. […] Wilms tumours are the most common paediatic renal mass, accounting for over 85% of cases and accounts for 6% of all childhood cancers. […] The incidence of Wilms tumor is 7.1 cases per 1 million children younger than 15 years. Approximately 500 cases of Wilms tumor are diagnosed in the United States each year. […] It typically occurs in early childhood (1-11 years) with peak incidence between 3 and 4 years of age. Approximately 80% of these tumors are found before the age of 5 years. When part of a syndrome they occur even earlier, typically between 2 and 24 months of age. The mean age at diagnosis is 44 months in unilateral cases of Wilms tumor and 31 months in bilateral cases. […] The male to female ratio in unilateral cases of Wilms tumor is 0.92:1.00, but in bilateral cases it is 0.60:1.00. […] The incidence is substantially lower in Asians.
  • #18 Wilms Tumor and Other Childhood Kidney Tumors Treatment (PDQ®) – NCI
    https://www.cancer.gov/types/kidney/hp/wilms-treatment-pdq
    Wilms tumor is the most frequent tumor of the kidney in infants and children. The incidence of Wilms tumor is 9.7 cases for every 1 million children younger than 15 years and 13.5 cases per 1 million infants. Approximately 650 cases of Wilms tumor are diagnosed in the United States each year. The incidence is substantially lower in Asian people. […] The male-to-female ratio in unilateral cases of Wilms tumor is 0.92 to 1.00, but in bilateral cases, there is a female excess (0.60). The mean age at diagnosis is 44 months in unilateral cases and 31 months in bilateral cases of Wilms tumor. About 10% of children with Wilms tumor have an associated congenital malformation syndrome. […] Wilms tumor typically develops in otherwise healthy children without any predisposition to developing cancer. However, approximately 10% of children with Wilms tumor have been reported to have a congenital anomaly.
  • #19 Wilms Tumor: Practice Essentials, Background, Etiology
    https://emedicine.medscape.com/article/989398-overview
    Wilms tumor affects approximately 10 children and adolescents per 1 million before age 15 years. Therefore, it accounts for 6-7% of all childhood cancers in North America. As a result, about 450-500 new cases are diagnosed each year on this continent. In 5-10% of patients, both kidneys are affected at the same time (synchronous bilateral Wilms tumor) or one after the other (metachronous bilateral Wilms tumor). […] Wilms tumor appears to be relatively more common in Africa and least common in East Asia. The incidence in Europe is similar to that reported in North America. Low-income countries have a higher median incidence of Wilms tumor (9.8 age-standardized rate [ASR] per million) than high-income (8.6 ASR per million) and middle-income countries (6.1 ASR per million). […] Wilms tumor is relatively more common in Blacks than in Whites and is rare in East Asians. Estimates suggest 6-9 cases per million person years in Whites, 3-4 cases per million person years in East Asians, and more than 10 cases per million person years among Black populations. Among patients with unilateral Wilms tumor enrolled in all NWTSG protocols, the male-to-female ratio was 0.92:1. For patients with bilateral disease, the male-to-female ratio was 0.60:1. The median age at diagnosis of Wilms tumor is approximately 3.5 years. The median age is highest for patients with unilateral unicentric disease (36.1 mo) and lowest for those with synchronous bilateral Wilms tumors (25.5 mo). A study by Heck et al looked to determine whether the risk of childhood cancers among Hispanic children varies by maternal birthplace. The researchers found that for certain cancer types, such as glioma and astrocytoma, solid neuroblastoma, and Wilms tumor of the kidney, children of nonUS born Hispanic mothers had the lowest risk.
  • #20 Inter-Ethnic Variations in the Clinical, Pathological, and Molecular Characteristics of Wilms Tumor
    https://www.mdpi.com/2072-6694/16/17/3051
    Wilms tumor is the most common pediatric primary renal malignancy globally but exhibits significant variations in epidemiological, clinical, and molecular aspects among different populations. […] Wilms tumor occurs at a lower incidence and at a younger age in Asians compared to Caucasians and Africans. […] Reports from the 1990s first described inter-ethnic variations in the incidence of Wilms tumors—Asian Wilms tumors had lower incidences and tended to occur at a younger age, with up to 40% occurring in infants less than 1 year old, compared to Caucasian and Black populations with peak incidences around the second year of life. […] The reduced incidence of Wilms tumor in the Asian population has been hypothesized to be due to the loss of imprinting (LOI) for the Insulin-like Growth Factor 2 gene (IGF2) occurring at a lower frequency in Asians.
  • #21 Inter-Ethnic Variations in the Clinical, Pathological, and Molecular Characteristics of Wilms Tumor
    https://www.mdpi.com/2072-6694/16/17/3051
    Another area where epidemiological characteristics differ significantly by ethnicity is gender distribution. […] Multiple studies have consistently indicated an earlier peak age of diagnosis of Wilms tumor in Asian children, typically in the second year of life. […] In summary, Wilms tumor occurs at a lower incidence and at a younger age in Asians compared to Caucasian and African populations.
  • #22 Wilms tumor | Radiology Reference Article | Radiopaedia.org
    https://radiopaedia.org/articles/wilms-tumour?embed_domain=external.radpair.com%252527%25255B0%25255D%252527%25255B0%25255D&lang=us
    Wilms tumors are the most common pediatric renal mass, accounting for over 85% of cases and account for 7% of all childhood cancers. They typically occur in early childhood (1-11 years) with peak incidence between 3 and 4 years of age. Approximately 80% of these tumors are found before the age of 5 years. When part of a syndrome, they occur even earlier, typically between 2 and 24 months of age. […] There is no recognized gender predilection, however, presentation is a little later in females. The vast majority are unilateral with 5% occurring bilaterally.
  • #23 Wilms Tumor and Other Childhood Kidney Tumors Treatment (PDQ®) – NCI
    https://www.cancer.gov/types/kidney/hp/wilms-treatment-pdq
    Wilms tumor is the most frequent tumor of the kidney in infants and children. The incidence of Wilms tumor is 9.7 cases for every 1 million children younger than 15 years and 13.5 cases per 1 million infants. Approximately 650 cases of Wilms tumor are diagnosed in the United States each year. The incidence is substantially lower in Asian people. […] The male-to-female ratio in unilateral cases of Wilms tumor is 0.92 to 1.00, but in bilateral cases, there is a female excess (0.60). The mean age at diagnosis is 44 months in unilateral cases and 31 months in bilateral cases of Wilms tumor. About 10% of children with Wilms tumor have an associated congenital malformation syndrome. […] Wilms tumor typically develops in otherwise healthy children without any predisposition to developing cancer. However, approximately 10% of children with Wilms tumor have been reported to have a congenital anomaly.
  • #24 Wilms’ tumor epidemiology and demographics – wikidoc
    https://www.wikidoc.org/index.php/Wilms%27_tumor_epidemiology_and_demographics
    Wilms tumors are the most common pediatric renal mass. The incidence of Wilms tumor is estimated to be 0.71 cases per 10,00,000 children younger than 15 years. There is no significant gender predilection among unilateral cases, but the male to female ratio in bilateral cases is 0.60:1.00. It typically occurs in early childhood with peak incidence between 3 and 4 years of age. Asian individuals are less likely to develop Wilms tumor. […] Wilms tumours are the most common paediatic renal mass, accounting for over 85% of cases and accounts for 6% of all childhood cancers. […] The incidence of Wilms tumor is 7.1 cases per 1 million children younger than 15 years. Approximately 500 cases of Wilms tumor are diagnosed in the United States each year. […] It typically occurs in early childhood (1-11 years) with peak incidence between 3 and 4 years of age. Approximately 80% of these tumors are found before the age of 5 years. When part of a syndrome they occur even earlier, typically between 2 and 24 months of age. The mean age at diagnosis is 44 months in unilateral cases of Wilms tumor and 31 months in bilateral cases. […] The male to female ratio in unilateral cases of Wilms tumor is 0.92:1.00, but in bilateral cases it is 0.60:1.00. […] The incidence is substantially lower in Asians.
  • #25 Pediatric Oncology Education Materials
    http://www.pedsoncologyeducation.com/wilmstumor_epidemiology.asp
    Wilms tumor is also called nephroblastoma. This is the fourth most common form of childhood cancer and the most common primary renal tumor in children (6% of all childhood cancer). […] Summary of Wilms Epidemiology: Annual incidence (In patients 16 years) 7 per million children. Proportion of pediatric cancers 6%. Age affected Mostly 5 years old. […] Sporadic cases with unilateral tumors present at an older age (median 36 months) than familial synchronous bilateral Wilms tumor (median age 26 months). […] Proportion of patients with various anomalies: Musculoskeletal: 2.9% Genitourinary: 4.4%. […] Peak incidence 3-4 years. Median age at diagnosis is 3.5 years. […] Gender bias: Generally female predominance for both unilateral and bilateral tumors. The male to female ratio is 0.92 for unilateral tumors. The male to female ratio is 0.6 for bilateral tumors. In South East Asia excess of cases in males.
  • #26 Pediatric Oncology Education Materials
    http://www.pedsoncologyeducation.com/wilmstumor_epidemiology.asp
    Wilms tumor is also called nephroblastoma. This is the fourth most common form of childhood cancer and the most common primary renal tumor in children (6% of all childhood cancer). […] Summary of Wilms Epidemiology: Annual incidence (In patients 16 years) 7 per million children. Proportion of pediatric cancers 6%. Age affected Mostly 5 years old. […] Sporadic cases with unilateral tumors present at an older age (median 36 months) than familial synchronous bilateral Wilms tumor (median age 26 months). […] Proportion of patients with various anomalies: Musculoskeletal: 2.9% Genitourinary: 4.4%. […] Peak incidence 3-4 years. Median age at diagnosis is 3.5 years. […] Gender bias: Generally female predominance for both unilateral and bilateral tumors. The male to female ratio is 0.92 for unilateral tumors. The male to female ratio is 0.6 for bilateral tumors. In South East Asia excess of cases in males.
  • #27 Clinical presentation, diagnosis, and staging of Wilms tumor – UpToDate
    https://www.uptodate.com/contents/clinical-presentation-diagnosis-and-staging-of-wilms-tumor
    Wilms tumor is the most common kidney malignancy in children, with approximately 650 new cases diagnosed in the United States each year. […] In the United States, the annual incidence of kidney tumors is approximately 10 cases per 1 million children younger than 15 years, accounting for 5 percent of all childhood malignancies and approximately 650 new cases per year. Wilms tumor is the most common kidney malignancy in children <15 years old, accounting for approximately 95 percent of all cases. [...] In contrast, renal cell carcinoma (RCC) is more common in the 15- to 19-year-old age group. [...] The incidence of Wilms tumor is the lowest in Asian children and the highest in children of African descent. [...] Wilms tumor is primarily a sporadic disease, and only 1 to 2 percent of individuals with Wilms tumor have a relative with the disease. In approximately 10 to 15 percent of cases, Wilms tumor occurs as a part of a multiple malformation syndrome, including WAGR, Denys-Drash, and Beckwith-Wiedemann syndrome.
  • #28 Wilms Tumor and Other Childhood Kidney Tumors Treatment (PDQ®) – NCI
    https://www.cancer.gov/types/kidney/hp/wilms-treatment-pdq
    Wilms tumor is the most frequent tumor of the kidney in infants and children. The incidence of Wilms tumor is 9.7 cases for every 1 million children younger than 15 years and 13.5 cases per 1 million infants. Approximately 650 cases of Wilms tumor are diagnosed in the United States each year. The incidence is substantially lower in Asian people. […] The male-to-female ratio in unilateral cases of Wilms tumor is 0.92 to 1.00, but in bilateral cases, there is a female excess (0.60). The mean age at diagnosis is 44 months in unilateral cases and 31 months in bilateral cases of Wilms tumor. About 10% of children with Wilms tumor have an associated congenital malformation syndrome. […] Wilms tumor typically develops in otherwise healthy children without any predisposition to developing cancer. However, approximately 10% of children with Wilms tumor have been reported to have a congenital anomaly.
  • #29 Bukenya Ali – Renal Pathology assignment.pptx
    https://www.slideshare.net/slideshow/bukenya-ali-renal-pathology-assignment-pptx/278841240
    3. WILMS TUMOR. A) Malignant kidney tumor comprised of blastema (immature kidney mesenchyme), primitive glomeruli and tubules, and stromal cells. 1. Most common malignant renal tumor in children; average age is 3 years. B) Presents as a large, unilateral flank mass with hematuria and hypertension (due to renin secretion). C) Most cases (90%) are sporadic; syndromic tumors may be seen with; 1. WAGR syndromeWilms tumor, Aniridia, Genital abnormalities, and mental and motor Retardation; associated with deletion of WT1 tumor suppressor gene (located at llpl3). 2. Denys-Drash syndromeWilms tumor, progressive renal (glomerular) disease, and male pseudo hermaphroditism; associated with mutations of WT1. 3. Beckwith-Wiedemann syndromeWilms tumor, neonatal hypoglycemia, muscular hemihypertrophy, and organomegaly (including tongue); associated with mutations in WT2 gene cluster (imprinted genes at 1lpl5.5), particularly IGF-2
  • #30 Syndromic Wilms tumor: a review of predisposing conditions, surveillance and treatment – Liu – Translational Andrology and Urology
    https://tau.amegroups.org/article/view/39654/html
    Predisposing syndromes associated with an increased risk of Wilms tumor (WT) are responsible for 917% of all cases of the malignancy. […] General surveillance guidelines include screening renal or abdominal ultrasound every 34 months until the age of 5 or 7, depending on the syndrome. […] Due to the differences mentioned above, including increased frequency, earlier age at diagnosis, and higher incidence of bilateral disease, approaches to management differ when compared to sporadic WT when an underlying predisposing syndrome is present. These patients should be offered nephron-sparing surgeries, cautious chemotherapeutic and radiation therapy, and familial genetic counseling. […] The first step in an effective screening protocol for syndromic WT is to identify who has a syndrome that predisposes patients to the malignancy.
  • #31 Wilms’ tumor – Wikipedia
    https://en.wikipedia.org/wiki/Wilms%27_tumor
    Wilms tumor is the most common malignant renal tumor in children. […] There are a number of rare genetic syndromes that have been linked to an increased risk of developing Wilms Tumor. […] Screening guidelines vary between countries; however health care professionals are recommending regular ultrasound screening for people with associated genetic syndromes. […] Wilms’ tumor affects approximately one person per 10,000 worldwide before the age of 15 years. […] People of African descent may have slightly higher rates of Wilms’ tumor. […] The peak age of Wilms’ tumor is 3 to 4 years and most cases occur before the age of 10 years. […] A genetic predisposition to Wilms’ tumor in individuals with aniridia has been established, due to deletions in the p13 band on chromosome 11.
  • #32 Wilms Tumor (Nephroblastoma), Version 2.2021, NCCN Clinical Practice Guidelines in Oncology in: Journal of the National Comprehensive Cancer Network Volume 19 Issue 8 (2021)
    https://jnccn.org/abstract/journals/jnccn/19/8/article-p945.xml
    Children with genetic predisposition syndromes should receive routine screening for possible development of WT. The goal is to identify and treat the WT at an early stage when the tumor is small and asymptomatic; this may hopefully be accomplished by partial nephrectomy, preserving renal tissue. It is important to note that the presence of a genetic predisposition syndrome does not mean that a child will develop WT. The different genetic syndromes are associated with various levels of risk for WT. Children with Denys-Drash have approximately a 90% risk of developing WT; Perlman syndrome, approximately a 75% risk; and WAGR syndrome, approximately a 50% risk. Approximately 10% of children with Beckwith-Wiedemann syndrome will develop WT, but the risk varies with the genetic alteration. […] The American Association for Cancer Research recommends screening in all children with a greater than 1% risk of developing WT. The NCCN Panel recommends that screening include physical examination and renal ultrasound every 3 months until children are at least 8 years of age based on the available data and clinical experience. Children who present at a younger age are more likely to have multifocal/bilateral disease than children without a predisposition syndrome and often have been identified as part of a surveillance program.
  • #33 Wilms Tumor: Symptoms, Causes, Stages, Diagnosis and Treatment – OncoDaily
    https://oncodaily.com/oncolibrary/cancer-types/234450
    Wilms tumor, or nephroblastoma, is a rare yet significant type of kidney cancer primarily affecting children under the age of 5. It accounts for approximately 6-7% of all childhood cancers, making it one of the most common pediatric renal malignancies. […] Early detection and treatment have dramatically improved outcomes, with a 5-year survival rate exceeding 90% for cases localized to the kidney, according to the American Cancer Society and the Childrens Oncology Group. […] These statistics emphasize the need for prompt evaluation of early signs to improve treatment outcomes. […] According to data from MedlinePlus Genetics, children with WAGR syndrome face a 45-60% lifetime risk of developing Wilms tumor. […] This syndrome is often accompanied by features such as macroglossia (enlarged tongue) and hemihypertrophy (asymmetric body growth), which alert clinicians to the need for regular tumor monitoring.
  • #34 Wilms Tumor: Symptoms, Causes, Stages, Diagnosis and Treatment – OncoDaily
    https://oncodaily.com/oncolibrary/cancer-types/234450
    Nearly 90% of children with this syndrome develop Wilms tumor, making it one of the highest-risk genetic conditions and highlighting the importance of proactive monitoring. […] According to data from the Childrens Oncology Group and the American Cancer Society, favorable histology Wilms tumor is the most common type, accounting for approximately 90% of all Wilms tumor cases. […] Survival rates for favorable histology Wilms tumor are highly encouraging. For localized cases, the 5-year survival rate exceeds 90%. […] According to data from the Childrens Oncology Group, the 5-year survival rate for children with localized anaplastic tumors is approximately 70-75% when treated with surgery, chemotherapy, and radiation therapy. […] The prognosis of Wilms tumor depends on tumor stage, histology, and patient health. Early-stage tumors (Stages I and II) have a 5-year survival rate exceeding 90%, while Stage IV cases have a 4-year survival rate of approximately 86%.
  • #35 Syndromic Wilms tumor: a review of predisposing conditions, surveillance and treatment – Liu – Translational Andrology and Urology
    https://tau.amegroups.org/article/view/39654/html
    Predisposing syndromes associated with an increased risk of Wilms tumor (WT) are responsible for 917% of all cases of the malignancy. […] General surveillance guidelines include screening renal or abdominal ultrasound every 34 months until the age of 5 or 7, depending on the syndrome. […] Due to the differences mentioned above, including increased frequency, earlier age at diagnosis, and higher incidence of bilateral disease, approaches to management differ when compared to sporadic WT when an underlying predisposing syndrome is present. These patients should be offered nephron-sparing surgeries, cautious chemotherapeutic and radiation therapy, and familial genetic counseling. […] The first step in an effective screening protocol for syndromic WT is to identify who has a syndrome that predisposes patients to the malignancy.
  • #36 Surveillance for Wilms tumour in at‐risk children: pragmatic recommendations for best practice
    https://pmc.ncbi.nlm.nih.gov/articles/PMC2083016/
    Most Wilms tumours occur in otherwise healthy children, but a small proportion occur in children with genetic syndromes associated with increased risks of Wilms tumour. Surveillance for Wilms tumour has become widespread, despite a lack of clarity about which children are at increased risk of these tumours and limited evidence of the efficacy of screening or guidance as to how screening should be implemented. […] The potential risks and benefits of Wilms tumour surveillance are finely balanced and there is no clear evidence that screening reduces mortality or morbidity. […] Regular surveillance in children thought to be at increased risk of Wilms tumour has become widespread in the UK, US and parts of Europe. However, there is little evidence available regarding the efficacy of screening or the balance of potential risks and benefits.
  • #37 Surveillance for Wilms tumour in at‐risk children: pragmatic recommendations for best practice
    https://pmc.ncbi.nlm.nih.gov/articles/PMC2083016/
    We formed a working group of clinical geneticists, paediatricians, paediatric oncologists and radiologists to formulate recommendations for Wilms tumour surveillance based on a review of the available evidence from the literature, current practice and expert opinion. […] Surveillance should be offered to children at 5% risk of Wilms tumour. […] Surveillance should only be offered after review by a clinical geneticist. […] Surveillance should be carried out by renal ultrasonography every 3-4 months. […] Surveillance should continue until 5 years of age in all conditions except Beckwith-Wiedemann syndrome, Simpson-Golabi-Behmel syndrome and some familial Wilms tumour pedigrees where it should continue until 7 years. […] Surveillance can be undertaken at a local centre, but should be carried out by someone with experience in paediatric ultrasonography. […] Screen-detected lesions should be managed at a specialist centre.
  • #38 Wilms Tumor and Other Childhood Kidney Tumors Treatment (PDQ®) – NCI
    https://www.cancer.gov/types/kidney/hp/wilms-treatment-pdq
    It is important to recognize that the absolute risk of developing Wilms tumor varies with the underlying condition or anomaly. For example, most patients with hemihypertrophy will not develop Wilms tumor. […] The primary purpose of screening is to enable earlier detection of a small and localized tumor (stage I or II), improve prognosis, and use less intensive treatment (such as to facilitate nephron-sparing surgery). Children with a significant increased predisposition to develop Wilms tumor (e.g., most children with Beckwith-Wiedemann syndrome or other overgrowth syndromes, WAGR syndrome, Denys-Drash syndrome, sporadic aniridia, or isolated hemihypertrophy) are usually screened with ultrasonography every 3 months until they reach at least age 8 years. […] Screening for Wilms tumor usually continues until age 8 years. Physical examination by a specialist (geneticist or pediatric oncologist) is recommended twice per year, and ongoing education regarding tumor manifestations, reinforcing the rationale for screening and compliance with the screening regimen, is discussed.
  • #39 Wilms Tumor and Other Childhood Kidney Tumors Treatment (PDQ®): Treatment – Health Professional Information [NCI] | Kaiser Permanente
    https://healthy.kaiserpermanente.org/health-wellness/health-encyclopedia/he.wilms-tumor-and-other-childhood-kidney-tumors-treatment-pdq%C2%AE-treatment-health-professional-information-nci.ncicdr0000062789
    Wilms tumor is the most frequent tumor of the kidney in infants and children. The incidence of Wilms tumor is 10.4 cases for every 1 million children younger than 15 years, and 0.2 cases per 10,000 infants. Approximately 650 cases of Wilms tumor are diagnosed in the United States each year. The incidence is substantially lower in Asian people. […] The male to female ratio in unilateral cases of Wilms tumor is 0.92 to 1.00, but in bilateral cases, there is a female excess (0.60). The mean age at diagnosis is 44 months in unilateral cases and 31 months in bilateral cases of Wilms tumor. About 10% of children with Wilms tumor have an associated congenital malformation syndrome. […] The primary purpose of screening is to enable earlier detection of a small and localized tumor (stage I or II), improve prognosis, and use less intensive treatment (such as to facilitate nephron-sparing surgery). Children with a significant increased predisposition to develop Wilms tumor (e.g., most children with Beckwith-Wiedemann syndrome or other overgrowth syndromes, WAGR syndrome, Denys-Drash syndrome, sporadic aniridia, or isolated hemihypertrophy) are usually screened with ultrasonography every 3 months until they reach at least age 8 years.
  • #40 Surveillance for Wilms tumour in at‐risk children: pragmatic recommendations for best practice
    https://pmc.ncbi.nlm.nih.gov/articles/PMC2083016/
    We formed a working group of clinical geneticists, paediatricians, paediatric oncologists and radiologists to formulate recommendations for Wilms tumour surveillance based on a review of the available evidence from the literature, current practice and expert opinion. […] Surveillance should be offered to children at 5% risk of Wilms tumour. […] Surveillance should only be offered after review by a clinical geneticist. […] Surveillance should be carried out by renal ultrasonography every 3-4 months. […] Surveillance should continue until 5 years of age in all conditions except Beckwith-Wiedemann syndrome, Simpson-Golabi-Behmel syndrome and some familial Wilms tumour pedigrees where it should continue until 7 years. […] Surveillance can be undertaken at a local centre, but should be carried out by someone with experience in paediatric ultrasonography. […] Screen-detected lesions should be managed at a specialist centre.
  • #41 Syndromic Wilms tumor: a review of predisposing conditions, surveillance and treatment – Liu – Translational Andrology and Urology
    https://tau.amegroups.org/article/view/39654/html
    Because of the increased risks of WT associated with various syndromes, screening is recommended to identify tumors earlier. […] General screening recommendations of serial ultrasound every 3 months until the age of 7 apply, although it should be noted that DDS patients, on average, are diagnosed just over a year of age. […] WAGR has the highest reported rates of WT, with one report of 54 patients describing 57% with the malignancy. It is recommended that patients undergo serial renal ultrasound until the age of 7, approximately every 3 months. […] The American Association for Cancer Research (AACR) acknowledged these findings and admits a distinction in practice environments between the United States and Europe. […] Children with SGBS syndrome should undergo screening abdominal ultrasound every 3 months until the age of 7 because of the risk of WT and hepatoblastoma. […] Because of the malignancy risks besides WT, screening recommendations for Bloom and Li Fraumeni syndromes are more inclusive than most of the other predisposing conditions.
  • #42 Surveillance Recommendations for Children with Overgrowth Syndromes and Predisposition to Wilms Tumors and Hepatoblastoma
    https://www.repository.cam.ac.uk/items/4bf7136b-63c3-4094-81ed-4d1d11d3e045
    A number of genetic syndromes have been linked to increased risk for Wilms tumor (WT), hepatoblastoma (HB), and other embryonal tumors. […] Specifically, for syndromes with increased risk for WT, we recommend renal ultrasounds every 3 months from birth (or the time of diagnosis) through the seventh birthday. […] Further study is expected to lead to modifications of these recommendations.
  • #43 Wilms Tumor (Nephroblastoma), Version 2.2021, NCCN Clinical Practice Guidelines in Oncology in: Journal of the National Comprehensive Cancer Network Volume 19 Issue 8 (2021)
    https://jnccn.org/abstract/journals/jnccn/19/8/article-p945.xml
    Children with genetic predisposition syndromes should receive routine screening for possible development of WT. The goal is to identify and treat the WT at an early stage when the tumor is small and asymptomatic; this may hopefully be accomplished by partial nephrectomy, preserving renal tissue. It is important to note that the presence of a genetic predisposition syndrome does not mean that a child will develop WT. The different genetic syndromes are associated with various levels of risk for WT. Children with Denys-Drash have approximately a 90% risk of developing WT; Perlman syndrome, approximately a 75% risk; and WAGR syndrome, approximately a 50% risk. Approximately 10% of children with Beckwith-Wiedemann syndrome will develop WT, but the risk varies with the genetic alteration. […] The American Association for Cancer Research recommends screening in all children with a greater than 1% risk of developing WT. The NCCN Panel recommends that screening include physical examination and renal ultrasound every 3 months until children are at least 8 years of age based on the available data and clinical experience. Children who present at a younger age are more likely to have multifocal/bilateral disease than children without a predisposition syndrome and often have been identified as part of a surveillance program.
  • #44 Wilms Tumor (Nephroblastoma), Version 2.2021, NCCN Clinical Practice Guidelines in Oncology in: Journal of the National Comprehensive Cancer Network Volume 19 Issue 8 (2021)
    https://jnccn.org/abstract/journals/jnccn/19/8/article-p945.xml
    Children with genetic predisposition syndromes should receive routine screening for possible development of WT. The goal is to identify and treat the WT at an early stage when the tumor is small and asymptomatic; this may hopefully be accomplished by partial nephrectomy, preserving renal tissue. It is important to note that the presence of a genetic predisposition syndrome does not mean that a child will develop WT. The different genetic syndromes are associated with various levels of risk for WT. Children with Denys-Drash have approximately a 90% risk of developing WT; Perlman syndrome, approximately a 75% risk; and WAGR syndrome, approximately a 50% risk. Approximately 10% of children with Beckwith-Wiedemann syndrome will develop WT, but the risk varies with the genetic alteration. […] The American Association for Cancer Research recommends screening in all children with a greater than 1% risk of developing WT. The NCCN Panel recommends that screening include physical examination and renal ultrasound every 3 months until children are at least 8 years of age based on the available data and clinical experience. Children who present at a younger age are more likely to have multifocal/bilateral disease than children without a predisposition syndrome and often have been identified as part of a surveillance program.
  • #45 Surveillance for Wilms tumour in at‐risk children: pragmatic recommendations for best practice
    https://pmc.ncbi.nlm.nih.gov/articles/PMC2083016/
    Most Wilms tumours occur in otherwise healthy children, but a small proportion occur in children with genetic syndromes associated with increased risks of Wilms tumour. Surveillance for Wilms tumour has become widespread, despite a lack of clarity about which children are at increased risk of these tumours and limited evidence of the efficacy of screening or guidance as to how screening should be implemented. […] The potential risks and benefits of Wilms tumour surveillance are finely balanced and there is no clear evidence that screening reduces mortality or morbidity. […] Regular surveillance in children thought to be at increased risk of Wilms tumour has become widespread in the UK, US and parts of Europe. However, there is little evidence available regarding the efficacy of screening or the balance of potential risks and benefits.
  • #46 Syndromic Wilms tumor: a review of predisposing conditions, surveillance and treatment – Liu – Translational Andrology and Urology
    https://tau.amegroups.org/article/view/39654/html
    Because of the increased risks of WT associated with various syndromes, screening is recommended to identify tumors earlier. […] General screening recommendations of serial ultrasound every 3 months until the age of 7 apply, although it should be noted that DDS patients, on average, are diagnosed just over a year of age. […] WAGR has the highest reported rates of WT, with one report of 54 patients describing 57% with the malignancy. It is recommended that patients undergo serial renal ultrasound until the age of 7, approximately every 3 months. […] The American Association for Cancer Research (AACR) acknowledged these findings and admits a distinction in practice environments between the United States and Europe. […] Children with SGBS syndrome should undergo screening abdominal ultrasound every 3 months until the age of 7 because of the risk of WT and hepatoblastoma. […] Because of the malignancy risks besides WT, screening recommendations for Bloom and Li Fraumeni syndromes are more inclusive than most of the other predisposing conditions.
  • #47 Clinical Cancer Research: Surveillance Recommendations for Children with Overgrowth Syndromes and Predisposition to Wilms Tumors and Hepatoblastoma – Greehey Children’s Cancer
    https://gccri.uthscsa.edu/2017/07/01/clinical-cancer-research-surveillance-recommendations-for-children-with-overgrowth-syndromes-and-predisposition-to-wilms-tumors-and-hepatoblastoma/
    A number of genetic syndromes have been linked to increased risk for Wilms tumor (WT), hepatoblastoma (HB), and other embryonal tumors. Here, we outline these rare syndromes with at least a 1% risk to develop these tumors and recommend uniform tumor screening recommendations for North America. Specifically, for syndromes with increased risk for WT, we recommend renal ultrasounds every 3 months from birth (or the time of diagnosis) through the seventh birthday. […] We recommend that when possible, these patients be evaluated and monitored by cancer predisposition specialists. At this time, these recommendations are not based on the differential risk between different genetic or epigenetic causes for each syndrome, which some European centers have implemented. This differentiated approach largely represents distinct practice environments between the United States and Europe, and these guidelines are designed to be a broad framework within which physicians and families can work together to implement specific screening. Further study is expected to lead to modifications of these recommendations.
  • #48 Clinical Cancer Research: Surveillance Recommendations for Children with Overgrowth Syndromes and Predisposition to Wilms Tumors and Hepatoblastoma – Greehey Children’s Cancer
    https://gccri.uthscsa.edu/2017/07/01/clinical-cancer-research-surveillance-recommendations-for-children-with-overgrowth-syndromes-and-predisposition-to-wilms-tumors-and-hepatoblastoma/
    A number of genetic syndromes have been linked to increased risk for Wilms tumor (WT), hepatoblastoma (HB), and other embryonal tumors. Here, we outline these rare syndromes with at least a 1% risk to develop these tumors and recommend uniform tumor screening recommendations for North America. Specifically, for syndromes with increased risk for WT, we recommend renal ultrasounds every 3 months from birth (or the time of diagnosis) through the seventh birthday. […] We recommend that when possible, these patients be evaluated and monitored by cancer predisposition specialists. At this time, these recommendations are not based on the differential risk between different genetic or epigenetic causes for each syndrome, which some European centers have implemented. This differentiated approach largely represents distinct practice environments between the United States and Europe, and these guidelines are designed to be a broad framework within which physicians and families can work together to implement specific screening. Further study is expected to lead to modifications of these recommendations.
  • #49 About WAGR – W is for Wilms Tumor
    https://wagr.org/wilms-tumor
    Wilms tumor is a solid cancerous tumor of the kidney. It is the most common form of kidney cancer in children. […] Children with WAGR syndrome are at increased risk for developing Wilms tumor. This increased risk is the result of a mutation in the WT1 gene, which is located within the region of the genetic abnormality that causes WAGR syndrome. About 50 percent of children with WAGR syndrome develop Wilms tumor. […] It is not yet possible to predict which children with WAGR syndrome will develop Wilms tumor. For this reason, periodic screening for Wilms tumor should begin at birth or upon diagnosis of WAGR syndrome. Screening consists of abdominal ultrasound examinations every 3 months until age 8. […] Although rare, Wilms tumor has been reported in individuals with WAGR syndrome up to age 19. For this reason, monitoring for Wilms tumor should continue throughout life. This surveillance may involve periodic ultrasound examination or MRI, and monitoring for symptoms such as abdominal mass, high blood pressure, and blood in the urine. […] Care management guidelines published in December 2022 outline specific recommendations for screening for Wilms tumor throughout the lifespan. These recommendations include ultrasound surveillance intervals depending on the patient’s age and medical history.
  • #50 About WAGR – W is for Wilms Tumor
    https://wagr.org/wilms-tumor
    Wilms tumor is a solid cancerous tumor of the kidney. It is the most common form of kidney cancer in children. […] Children with WAGR syndrome are at increased risk for developing Wilms tumor. This increased risk is the result of a mutation in the WT1 gene, which is located within the region of the genetic abnormality that causes WAGR syndrome. About 50 percent of children with WAGR syndrome develop Wilms tumor. […] It is not yet possible to predict which children with WAGR syndrome will develop Wilms tumor. For this reason, periodic screening for Wilms tumor should begin at birth or upon diagnosis of WAGR syndrome. Screening consists of abdominal ultrasound examinations every 3 months until age 8. […] Although rare, Wilms tumor has been reported in individuals with WAGR syndrome up to age 19. For this reason, monitoring for Wilms tumor should continue throughout life. This surveillance may involve periodic ultrasound examination or MRI, and monitoring for symptoms such as abdominal mass, high blood pressure, and blood in the urine. […] Care management guidelines published in December 2022 outline specific recommendations for screening for Wilms tumor throughout the lifespan. These recommendations include ultrasound surveillance intervals depending on the patient’s age and medical history.
  • #51 Wilms tumour in Beckwith–Wiedemann Syndrome and loss of methylation at imprinting centre 2: revisiting tumour surveillance guidelines | European Journal of Human Genetics
    https://www.nature.com/articles/ejhg2017102
    BeckwithWiedemann Syndrome (BWS) is an overgrowth syndrome caused by a variety of molecular changes on chromosome 11p15.5. Children with BWS have a significant risk of developing Wilms tumours with the degree of risk being dependent on the underlying molecular mechanism. […] Discontinuation of tumour surveillance for children with BWS and loss of methylation at IC2 has been proposed in several recent publications. […] The confirmed cases of loss of methylation at IC2 also suggest that the risk of Wilms tumour in this population is not as low as previously thought. Therefore, we recommend that for now, all children with a clinical or molecular diagnosis of BWS be screened for Wilms tumour by abdominal ultrasonography until the age of eight years regardless of the molecular classification.
  • #52 Wilms tumour in Beckwith–Wiedemann Syndrome and loss of methylation at imprinting centre 2: revisiting tumour surveillance guidelines | European Journal of Human Genetics
    https://www.nature.com/articles/ejhg2017102
    BWS confers a risk for embryonal tumour development in the first eight years of life of ~7.5% (range of 421%). This risk includes a 35% risk of developing Wilms tumour (WT), a renal malignancy of embryonal origin. Tumour surveillance recommendations vary amongst centres; however, for WT, these have typically included abdominal ultrasound every 3 months to the age of 8 years. […] The highest risk for tumour development, especially WT, occurs with patUPD of 11p15.5 or GOM at IC1. Tumour risk is significantly lower for LOM at IC2 or mutations in CDKN1C. Multiple investigators have concluded that LOM at IC2 does not confer an increased risk for WT. […] The fact that one case of BWS and LOM IC2 was reclassified to patUPD at 11p15.5 also points to challenges inherent in current clinical testing modalities suggesting that they are not always definitive. […] These findings support continued screening for WT in all children with a clinical diagnosis of BWS regardless of their underlying molecular alteration until such time that clinical testing can more accurately classify BWS cases and further data on prospectively ascertained cohorts are available to determine the true risk for WT in BWS and IC2 LOM.
  • #53 Diagnosis & Treatment | Wilms Foundation
    https://www.wilmsfoundation.org/diagnosisandtreatment
    Diagnosing Wilms is fairly straightforward and early indicators can be picked up from simple blood, urine and tissue samples. […] The disease has a good survival rate if detected early but if diagnosed late the cancer can be fatal. […] Children in Europe are usually treated with chemotherapy to shrink the tumor and then surgery. […] After your treatment is complete your child will still need to be seen regularly at an outpatient clinic. At this clinic, your childs specialist will be looking for any signs that the cancer has returned (this is known as a relapse). […] A personalized surveillance plan is usually created that outlines the specific long term follow up and on-going investigations that your child will require.
  • #54 What Happens After Treatment for Wilms Tumors? | American Cancer Society
    https://www.cancer.org/cancer/types/wilms-tumor/after-treatment/follow-up.html
    After your child finishes treatment for Wilms tumor, their cancer care team will talk to you about a follow-up schedule. This includes physical exams and imaging tests (like chest x-rays, ultrasounds, and CT scans). […] These exams and tests will look for the growth or return of the tumor, or any problems related to treatment. […] Its important to discuss these possible effects with your childs cancer care team. Make sure you understand what to watch for and what to report to the care team. The earlier problems are found, the more likely they can be treated effectively. […] The Childrens Oncology Group (COG) has developed long-term follow-up guidelines for survivors of childhood cancers, such as Wilms tumors. These guidelines were created to help increase awareness of late effects and improve follow-up care of childhood cancer survivors throughout their lives. […] Its very important to discuss possible long-term complications with your childs health care team. Make sure theres a plan in place to watch for these problems and treat them, if needed.
  • #55 Post-Therapy Surveillance of Wilms’ Tumor Survivors | SpringerLink
    https://link.springer.com/chapter/10.1007/978-981-19-3428-5_37
    In the present decade, with the advent of multimodal therapy, overall survival rates are about 90% for children with Wilms tumor mainly because of the advancement in chemoradiation therapy and also taking care of the long-term follow-up by avoiding the late secondary effect. […] Hence, a protocolized postoperative care and follow-up is very much important, and parents must be counseled and consent obtained about the same prior to starting the treatment. […] Learning objectives of this chapter include post-therapy follow-up surveillance protocol and its importance in early identification of any recurrence, secondary effects of chemoradiation therapy, avoiding unwanted recurrent exposure to radiation, and cost-effectiveness of the follow-up therapy.
  • #56 What Happens After Treatment for Wilms Tumors? | American Cancer Society
    https://www.cancer.org/cancer/types/wilms-tumor/after-treatment/follow-up.html
    After your child finishes treatment for Wilms tumor, their cancer care team will talk to you about a follow-up schedule. This includes physical exams and imaging tests (like chest x-rays, ultrasounds, and CT scans). […] These exams and tests will look for the growth or return of the tumor, or any problems related to treatment. […] Its important to discuss these possible effects with your childs cancer care team. Make sure you understand what to watch for and what to report to the care team. The earlier problems are found, the more likely they can be treated effectively. […] The Childrens Oncology Group (COG) has developed long-term follow-up guidelines for survivors of childhood cancers, such as Wilms tumors. These guidelines were created to help increase awareness of late effects and improve follow-up care of childhood cancer survivors throughout their lives. […] Its very important to discuss possible long-term complications with your childs health care team. Make sure theres a plan in place to watch for these problems and treat them, if needed.
  • #57 What Happens After Treatment for Wilms Tumors? | American Cancer Society
    https://www.cancer.org/cancer/types/wilms-tumor/after-treatment/follow-up.html
    After your child finishes treatment for Wilms tumor, their cancer care team will talk to you about a follow-up schedule. This includes physical exams and imaging tests (like chest x-rays, ultrasounds, and CT scans). […] These exams and tests will look for the growth or return of the tumor, or any problems related to treatment. […] Its important to discuss these possible effects with your childs cancer care team. Make sure you understand what to watch for and what to report to the care team. The earlier problems are found, the more likely they can be treated effectively. […] The Childrens Oncology Group (COG) has developed long-term follow-up guidelines for survivors of childhood cancers, such as Wilms tumors. These guidelines were created to help increase awareness of late effects and improve follow-up care of childhood cancer survivors throughout their lives. […] Its very important to discuss possible long-term complications with your childs health care team. Make sure theres a plan in place to watch for these problems and treat them, if needed.
  • #58 Wilms Tumor (Kidney Tumor) | Children’s Hospital of Philadelphia
    https://www.chop.edu/conditions-diseases/wilms-tumor-kidney-tumor
    Regular follow-up care is essential for a child diagnosed with Wilms tumor to monitor for tumor recurrence and long-term side effects of radiation and chemotherapy. […] Some children treated for Wilms tumor develop complications many years later. Our Cancer Survivorship Program provides you and your child with information about the potential long-term effects of the specific treatment your child received and ways of monitoring and treating these effects.
  • #59 The impact of surveillance imaging to detect relapse in Wilms tumor patients – Children’s National
    https://innovationdistrict.childrensnational.org/the-impact-of-surveillance-imaging-to-detect-relapse-in-wilms-tumor-patients/
    The Childrens Oncology Group published an article in the Journal of Clinical Oncology looking at the impact that surveillance imaging has on patients with Wilms tumor (WT), the most common kidney cancer in children. […] Despite the risks and costs, the use of computed tomography (CT) for routine surveillance to detect recurrence in patients with WT has increased in recent years. […] In conclusion, the doctors found that the elimination of CT scans from surveillance programs for unilateral favorable histology Wilms tumor is unlikely to compromise survival. However, it could result in substantially less radiation exposure and lower health care costs. Overall, the risk-benefit ratio associated with imaging modalities should be considered and formally studied for all pediatric cancers.
  • #60 The impact of surveillance imaging to detect relapse in Wilms tumor patients – Children’s National
    https://innovationdistrict.childrensnational.org/the-impact-of-surveillance-imaging-to-detect-relapse-in-wilms-tumor-patients/
    The Childrens Oncology Group published an article in the Journal of Clinical Oncology looking at the impact that surveillance imaging has on patients with Wilms tumor (WT), the most common kidney cancer in children. […] Despite the risks and costs, the use of computed tomography (CT) for routine surveillance to detect recurrence in patients with WT has increased in recent years. […] In conclusion, the doctors found that the elimination of CT scans from surveillance programs for unilateral favorable histology Wilms tumor is unlikely to compromise survival. However, it could result in substantially less radiation exposure and lower health care costs. Overall, the risk-benefit ratio associated with imaging modalities should be considered and formally studied for all pediatric cancers.
  • #61 Wilms Tumor – Diagnosis & Disease Information
    https://www.cancertherapyadvisor.com/ddi/wilms-tumor/
    The prognosis of Wilms tumor has dramatically improved over the past decades, with current treatment approaches achieving excellent outcomes for most patients. The overall survival rate for Wilms tumor is high approximately 90% of patients survive at least 5 years after diagnosis. This can be attributed to effective multimodal therapies, including surgery, chemotherapy, and radiation therapy. […] Wilms tumor prognosis varies by stage at diagnosis, and histology of the tumor. Children diagnosed with early-stage Wilms tumor with a favorable histology have a 4-year survival rate of 95% to 100%. In contrast, patients with advanced disease and/or unfavorable Wilms tumor histology (anaplastic, with poorly differentiated cells) have slightly lower survival rates, but generally favorable outcomes compared with many other childhood cancers. The survival rates by stage are 95% in patients in stages I and II, 75% to 80% in stage III, and 65% to 75% in stage IV. […] In addition to advanced-stage disease and anaplastic histology, older age at diagnosis and incomplete response to initial treatment also are associated with a worse prognosis. Patients with these factors may require more intensive therapies and close monitoring to improve outcomes.
  • #62 Post-Therapy Surveillance of Wilms’ Tumor Survivors | SpringerLink
    https://link.springer.com/chapter/10.1007/978-981-19-3428-5_37
    In the present decade, with the advent of multimodal therapy, overall survival rates are about 90% for children with Wilms tumor mainly because of the advancement in chemoradiation therapy and also taking care of the long-term follow-up by avoiding the late secondary effect. […] Hence, a protocolized postoperative care and follow-up is very much important, and parents must be counseled and consent obtained about the same prior to starting the treatment. […] Learning objectives of this chapter include post-therapy follow-up surveillance protocol and its importance in early identification of any recurrence, secondary effects of chemoradiation therapy, avoiding unwanted recurrent exposure to radiation, and cost-effectiveness of the follow-up therapy.
  • #63 Wilms Tumor: Symptoms, Causes, Stages, Diagnosis and Treatment – OncoDaily
    https://oncodaily.com/oncolibrary/cancer-types/234450
    Nearly 90% of children with this syndrome develop Wilms tumor, making it one of the highest-risk genetic conditions and highlighting the importance of proactive monitoring. […] According to data from the Childrens Oncology Group and the American Cancer Society, favorable histology Wilms tumor is the most common type, accounting for approximately 90% of all Wilms tumor cases. […] Survival rates for favorable histology Wilms tumor are highly encouraging. For localized cases, the 5-year survival rate exceeds 90%. […] According to data from the Childrens Oncology Group, the 5-year survival rate for children with localized anaplastic tumors is approximately 70-75% when treated with surgery, chemotherapy, and radiation therapy. […] The prognosis of Wilms tumor depends on tumor stage, histology, and patient health. Early-stage tumors (Stages I and II) have a 5-year survival rate exceeding 90%, while Stage IV cases have a 4-year survival rate of approximately 86%.
  • #64 Wilms Tumor – Diagnosis & Disease Information
    https://www.cancertherapyadvisor.com/ddi/wilms-tumor/
    The prognosis of Wilms tumor has dramatically improved over the past decades, with current treatment approaches achieving excellent outcomes for most patients. The overall survival rate for Wilms tumor is high approximately 90% of patients survive at least 5 years after diagnosis. This can be attributed to effective multimodal therapies, including surgery, chemotherapy, and radiation therapy. […] Wilms tumor prognosis varies by stage at diagnosis, and histology of the tumor. Children diagnosed with early-stage Wilms tumor with a favorable histology have a 4-year survival rate of 95% to 100%. In contrast, patients with advanced disease and/or unfavorable Wilms tumor histology (anaplastic, with poorly differentiated cells) have slightly lower survival rates, but generally favorable outcomes compared with many other childhood cancers. The survival rates by stage are 95% in patients in stages I and II, 75% to 80% in stage III, and 65% to 75% in stage IV. […] In addition to advanced-stage disease and anaplastic histology, older age at diagnosis and incomplete response to initial treatment also are associated with a worse prognosis. Patients with these factors may require more intensive therapies and close monitoring to improve outcomes.
  • #65 Wilms Tumor: Symptoms, Causes, Stages, Diagnosis and Treatment – OncoDaily
    https://oncodaily.com/oncolibrary/cancer-types/234450
    Nearly 90% of children with this syndrome develop Wilms tumor, making it one of the highest-risk genetic conditions and highlighting the importance of proactive monitoring. […] According to data from the Childrens Oncology Group and the American Cancer Society, favorable histology Wilms tumor is the most common type, accounting for approximately 90% of all Wilms tumor cases. […] Survival rates for favorable histology Wilms tumor are highly encouraging. For localized cases, the 5-year survival rate exceeds 90%. […] According to data from the Childrens Oncology Group, the 5-year survival rate for children with localized anaplastic tumors is approximately 70-75% when treated with surgery, chemotherapy, and radiation therapy. […] The prognosis of Wilms tumor depends on tumor stage, histology, and patient health. Early-stage tumors (Stages I and II) have a 5-year survival rate exceeding 90%, while Stage IV cases have a 4-year survival rate of approximately 86%.
  • #66 Wilms Tumor: Updates about Pathogenesis and New Possible Clinical Treatments of the Most Frequent Pediatric Urogenital Cancer: A Narrative Review
    https://www.mdpi.com/2673-4095/4/4/64
    Wilms tumor is the most common renal tumor in childhood, and the prognosis is mainly related to the histologic appearance of the neoplasm, where the presence of anaplastic (undifferentiated) cells suggests a poorer prognosis. The prognosis of Wilms tumor also depends on the stage at diagnosis and the patient’s age (advanced age is associated with a worse prognosis). Cure rates for low-stage disease (localized to the kidneys) range from 85 to 95 percent; children with the more advanced disease also have a good prognosis: cure rates range from 60 percent (unfavorable histology) to 90 percent (favorable histology). The problem of tumor recurrence, especially in the case of bilaterality, is a negative index that warrants closer and more prolonged monitoring over time.
  • #67 Wilms Tumor – Diagnosis & Disease Information
    https://www.cancertherapyadvisor.com/ddi/wilms-tumor/
    The prognosis of Wilms tumor has dramatically improved over the past decades, with current treatment approaches achieving excellent outcomes for most patients. The overall survival rate for Wilms tumor is high approximately 90% of patients survive at least 5 years after diagnosis. This can be attributed to effective multimodal therapies, including surgery, chemotherapy, and radiation therapy. […] Wilms tumor prognosis varies by stage at diagnosis, and histology of the tumor. Children diagnosed with early-stage Wilms tumor with a favorable histology have a 4-year survival rate of 95% to 100%. In contrast, patients with advanced disease and/or unfavorable Wilms tumor histology (anaplastic, with poorly differentiated cells) have slightly lower survival rates, but generally favorable outcomes compared with many other childhood cancers. The survival rates by stage are 95% in patients in stages I and II, 75% to 80% in stage III, and 65% to 75% in stage IV. […] In addition to advanced-stage disease and anaplastic histology, older age at diagnosis and incomplete response to initial treatment also are associated with a worse prognosis. Patients with these factors may require more intensive therapies and close monitoring to improve outcomes.
  • #68 Survival characteristics of Wilms Tumor, a reference developed from a longitudinal cohort study | Italian Journal of Pediatrics | Full Text
    https://ijponline.biomedcentral.com/articles/10.1186/s13052-024-01698-7
    Wilms tumor (WT) survival has been affected by the evolution in clinical and biological prognostic factors. Significant differences in survival rates indicate the need for further efforts to reduce these disparities. […] The prognosis for children diagnosed with WT can vary considerably based on various factors, such as age, sex, race, chemotherapy status, laterality, and tumor. […] The survival for patients with WT is strongly influenced by both their age and the stage of cancer at diagnosis, with survival rates decreasing significantly as the disease advances to higher stages. […] Moreover, significant differences in survival rates persist among different regions and nations, indicating the need for further efforts to reduce these disparities. […] The overall survival (OS) rates for the entire cohort at the 5-year and 10-year marks were 93.6% and 92.5%, respectively.
  • #69 Survival characteristics of Wilms Tumor, a reference developed from a longitudinal cohort study | Italian Journal of Pediatrics | Full Text
    https://ijponline.biomedcentral.com/articles/10.1186/s13052-024-01698-7
    Patients aged 15 years and older exhibited a 10-year CSS of 73.4%, in contrast to patients aged 0-3 years who demonstrated a CSS of 95.5%. […] However, a notable decline in CSS was observed with the increasing spread of tumors. […] Patients with distant spread had a markedly worse CSS, as did those with regional spread and individuals with an unknown stage when compared to patients with localized tumors. […] Among patients diagnosed with WT, the foremost cause of death was attributed to renal cancers, followed by non-renal cancers and non-cancer causes. […] The one-year, five-year, and ten-year relative survival for WT patients were 98.10%, 92.80%, and 91.3% respectively. […] The five-year conditional survival after one year, five years and ten years of survival for WT were 94.0%, 98.4%, and 99.0%, respectively. […] Management and follow-up should be tailored to the specific needs for each WT patient.
  • #70 Epidemiology and Clinical Outcomes of Childhood Wilms Tumor in Korea
    https://www.cpho.or.kr/journal/view.html?spage=164&volume=11&number=2
    Wilms tumor is the most common malignant renal tumor in children. We investigated the epidemiology, clinical features and treatment outcome of the children with Wilms tumor in Korea during the recent 10 years. […] The annual incidence rate per 1,000,000 population varied from 1.9 to 2.1. […] The 10 years overall survival rate according to sex, clinical stage, pathologic type and relapse were as follows: 88.6% in male, 90.9% in female, 100% in stage I, 94.7% in stage II, 92.1% in stage III, 63.4% in stage IV, 85.7% in stage V, 95.3% in favorable histology, 64.1% in unfavorable histology, 94.8% in non-relapse, and 40.9% in relapse. […] The relapse rate was 12%. […] The 10 years overall survival rate of 246 patients were 89.1%. […] Our results could provide the most recent and important clinical information on Wilms tumor of children in Korea.
  • #71 Survival characteristics of Wilms Tumor, a reference developed from a longitudinal cohort study | Italian Journal of Pediatrics | Full Text
    https://ijponline.biomedcentral.com/articles/10.1186/s13052-024-01698-7
    Patients aged 15 years and older exhibited a 10-year CSS of 73.4%, in contrast to patients aged 0-3 years who demonstrated a CSS of 95.5%. […] However, a notable decline in CSS was observed with the increasing spread of tumors. […] Patients with distant spread had a markedly worse CSS, as did those with regional spread and individuals with an unknown stage when compared to patients with localized tumors. […] Among patients diagnosed with WT, the foremost cause of death was attributed to renal cancers, followed by non-renal cancers and non-cancer causes. […] The one-year, five-year, and ten-year relative survival for WT patients were 98.10%, 92.80%, and 91.3% respectively. […] The five-year conditional survival after one year, five years and ten years of survival for WT were 94.0%, 98.4%, and 99.0%, respectively. […] Management and follow-up should be tailored to the specific needs for each WT patient.
  • #72 Survival characteristics of Wilms Tumor, a reference developed from a longitudinal cohort study | Italian Journal of Pediatrics | Full Text
    https://ijponline.biomedcentral.com/articles/10.1186/s13052-024-01698-7
    Wilms tumor (WT) survival has been affected by the evolution in clinical and biological prognostic factors. Significant differences in survival rates indicate the need for further efforts to reduce these disparities. […] The prognosis for children diagnosed with WT can vary considerably based on various factors, such as age, sex, race, chemotherapy status, laterality, and tumor. […] The survival for patients with WT is strongly influenced by both their age and the stage of cancer at diagnosis, with survival rates decreasing significantly as the disease advances to higher stages. […] Moreover, significant differences in survival rates persist among different regions and nations, indicating the need for further efforts to reduce these disparities. […] The overall survival (OS) rates for the entire cohort at the 5-year and 10-year marks were 93.6% and 92.5%, respectively.
  • #73 Survival characteristics of Wilms Tumor, a reference developed from a longitudinal cohort study | Italian Journal of Pediatrics | Full Text
    https://ijponline.biomedcentral.com/articles/10.1186/s13052-024-01698-7
    Patients aged 15 years and older exhibited a 10-year CSS of 73.4%, in contrast to patients aged 0-3 years who demonstrated a CSS of 95.5%. […] However, a notable decline in CSS was observed with the increasing spread of tumors. […] Patients with distant spread had a markedly worse CSS, as did those with regional spread and individuals with an unknown stage when compared to patients with localized tumors. […] Among patients diagnosed with WT, the foremost cause of death was attributed to renal cancers, followed by non-renal cancers and non-cancer causes. […] The one-year, five-year, and ten-year relative survival for WT patients were 98.10%, 92.80%, and 91.3% respectively. […] The five-year conditional survival after one year, five years and ten years of survival for WT were 94.0%, 98.4%, and 99.0%, respectively. […] Management and follow-up should be tailored to the specific needs for each WT patient.
  • #74 Wilms Tumor (Nephroblastoma), Version 2.2021, NCCN Clinical Practice Guidelines in Oncology in: Journal of the National Comprehensive Cancer Network Volume 19 Issue 8 (2021)
    https://jnccn.org/abstract/journals/jnccn/19/8/article-p945.xml
    Wilms tumor (WT, also known as nephroblastoma) is the most common primary renal tumor in children. In the United States, approximately 650 children are diagnosed with WT each year. WT accounts for more than 90% of primary renal tumors in patients younger than 20 years and for 5% of all childhood cancers. Most children (75%) present with WT between 1 and 5 years of age, most commonly at 3 years. The incidence of WT is highest among African American children, followed by Caucasian children, and then Asian children. Five-year survival is more than 90% for children with all stages of favorable histology WT (FHWT) who receive appropriate treatment. However, survival remains poor for children with higher-stage diffuse anaplastic WT. Most children present with resectable disease in one kidney, and upfront unilateral nephrectomy is recommended for most children. These NCCN Guidelines for Wilms Tumor (Nephroblastoma) were first published in 2021 and only address FHWT at this time. These NCCN Guidelines will be updated at least once a year by the NCCN Wilms Tumor Panel.
  • #75 Multi-institutional study of Wilms tumor facilitates enrollment, optimizes treatment – VUMC News
    https://news.vumc.org/2024/02/27/multi-institutional-study-of-wilms-tumor-facilitates-enrollment-optimizes-treatment/
    About 650 cases of Wilms tumor are diagnosed in the United States each year, and the five-year survival rate is 93%, according to the National Cancer Institute. […] Enrollment by race group differed from U.S. census data as expected, with Black children overrepresented reflecting higher incidence of Wilms tumor and Asian/Pacific Islander children underrepresented reflecting lower incidence. […] Overall, the study found no statistically significant differences between race and ethnic groups regarding risk-assigned therapeutic study enrollment, disease stage, histology, biologic factors, or event-free and overall survival, other than the following: Black children were older and had larger tumors at enrollment. […] The study confirmed previous findings that although Asian/Pacific Islander children have lower incidence of Wilms tumor, they are diagnosed at younger ages and have potentially poorer survival, suggesting that Wilms tumor impacts this racial group differently, the researchers noted.
  • #76 Multi-institutional study of Wilms tumor facilitates enrollment, optimizes treatment – VUMC News
    https://news.vumc.org/2024/02/27/multi-institutional-study-of-wilms-tumor-facilitates-enrollment-optimizes-treatment/
    Overall, the excellent and largely equivalent survival observed between race and ethnic groups in each of the therapeutic groups highlights the capacity of cooperative trials. […] Future studies should explore whether the burden of late toxic effects of treatment are equally distributed across race and ethnicity, the researchers noted.
  • #77 Surveillance Recommendations for Children with Overgrowth Syndromes and Predisposition to Wilms Tumors and Hepatoblastoma
    https://www.repository.cam.ac.uk/items/4bf7136b-63c3-4094-81ed-4d1d11d3e045
    A number of genetic syndromes have been linked to increased risk for Wilms tumor (WT), hepatoblastoma (HB), and other embryonal tumors. […] Specifically, for syndromes with increased risk for WT, we recommend renal ultrasounds every 3 months from birth (or the time of diagnosis) through the seventh birthday. […] Further study is expected to lead to modifications of these recommendations.
  • #78 Multi-institutional study of Wilms tumor facilitates enrollment, optimizes treatment – VUMC News
    https://news.vumc.org/2024/02/27/multi-institutional-study-of-wilms-tumor-facilitates-enrollment-optimizes-treatment/
    Overall, the excellent and largely equivalent survival observed between race and ethnic groups in each of the therapeutic groups highlights the capacity of cooperative trials. […] Future studies should explore whether the burden of late toxic effects of treatment are equally distributed across race and ethnicity, the researchers noted.
  • #79 Wilms Tumor: Symptoms, Causes, Stages, Diagnosis and Treatment – OncoDaily
    https://oncodaily.com/oncolibrary/cancer-types/234450
    Recent advancements in treatment, including targeted therapies and refined chemotherapy protocols, have further improved survival, though certain subgroups, such as anaplastic histology cases, still require better strategies. […] Experts recommend routine abdominal ultrasounds every 3-6 months for early detection in at-risk children.
  • #80 Multi-institutional study of Wilms tumor facilitates enrollment, optimizes treatment – VUMC News
    https://news.vumc.org/2024/02/27/multi-institutional-study-of-wilms-tumor-facilitates-enrollment-optimizes-treatment/
    Enrollment and outcomes were similar across racial and ethnic groups for children participating in therapeutic trials for Wilms tumor the most common pediatric kidney cancer worldwide, according to a recent report in the Journal of the American College of Surgeons. […] The findings also confirmed previously observed variations in Wilms tumor incidence across diverse race groups. […] While the racial variations in incidence for Wilms tumor remain poorly explained, it is encouraging that trial enrollment and outcomes both in the overall study and in different therapeutic groups remained largely consistent between race groups. […] This study emphasizes the tremendous benefits of multi-institutional cooperative studies to facilitate enrollment and optimize treatment for a pediatric cancer that has global variations in incidence and survival according to race and ethnicity.
  • #81 Multi-institutional study of Wilms tumor facilitates enrollment, optimizes treatment – VUMC News
    https://news.vumc.org/2024/02/27/multi-institutional-study-of-wilms-tumor-facilitates-enrollment-optimizes-treatment/
    Overall, the excellent and largely equivalent survival observed between race and ethnic groups in each of the therapeutic groups highlights the capacity of cooperative trials. […] Future studies should explore whether the burden of late toxic effects of treatment are equally distributed across race and ethnicity, the researchers noted.