Guzy wilmsa
Leczenie

Guzy Wilmsa (nephroblastoma) stanowią najczęstszy nowotwór nerki u dzieci, z wysokim wskaźnikiem wyleczalności około 90% przy zastosowaniu wielomodalnego leczenia obejmującego chirurgię, chemioterapię i radioterapię. Leczenie jest indywidualizowane w zależności od stadium zaawansowania, histologii, wieku pacjenta oraz cech molekularnych guza, takich jak utrata heterozygotyczności 1p i 16q. Standardowo stosuje się radykalną nefrektomię lub częściową nefrektomię w przypadku guzów obustronnych, a chemioterapia opiera się na lekach takich jak winkrystyna, daktynomycyna, doksorubicyna, cyklofosfamid, etopozyd, karboplatyna i irynotekan. Radioterapia jest wskazana w stadium III i IV, przy niekorzystnej histologii lub gdy guz nie jest całkowicie resekcyjny, z dawkami około 1080 cGy dla jamy brzusznej i 1200 cGy dla płuc. Strategie leczenia różnią się geograficznie – protokoły NWTS/COG preferują najpierw chirurgię, a SIOP – chemioterapię neoadjuwantową.

Leczenie Guzów Wilmsa

Guzy Wilmsa (nephroblastoma) to najczęstszy typ nowotworu nerki występujący u dzieci. Leczenie tego schorzenia jest jednym z największych sukcesów współczesnej onkologii dziecięcej, a wskaźniki wyleczalności sięgają około 90% przy zastosowaniu odpowiedniego, wielomodalnego podejścia terapeutycznego12. Strategia leczenia jest indywidualizowana i zależy od wielu czynników, w tym stadium zaawansowania nowotworu, histologii guza, wieku dziecka oraz obecności zmian chromosomalnych3.

Główne strategie leczenia

Istnieją dwie główne strategie leczenia guzów Wilmsa, które różnią się podejściem początkowym. W Ameryce Północnej stosuje się zazwyczaj protokoły National Wilms Tumor Study (NWTS) i Children’s Oncology Group (COG), które rekomendują najpierw zabieg chirurgiczny, a następnie chemioterapię. Natomiast w Europie i innych częściach świata preferuje się protokoły International Society of Pediatric Oncology (SIOP), które zalecają najpierw chemioterapię przedoperacyjną, a następnie zabieg chirurgiczny45.

Główne metody leczenia guzów Wilmsa obejmują:

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Leczenie chirurgiczne

Chirurgia jest podstawową metodą leczenia guzów Wilmsa. Głównym celem zabiegu jest usunięcie guza pierwotnego, nawet jeśli nowotwór rozprzestrzenił się do innych części ciała8. Najczęściej wykonuje się następujące zabiegi chirurgiczne:

  • Radykalna nefrektomia – całkowite usunięcie zajętej nerki wraz z otaczającymi tkankami i węzłami chłonnymi. Jest to standardowe podejście w przypadku jednostronnych guzów Wilmsa910.
  • Częściowa nefrektomia (oszczędzająca nerki) – usunięcie jedynie guza i marginesu zdrowej tkanki, z zachowaniem jak największej ilości funkcjonalnej tkanki nerki. Stosowana głównie w przypadkach guzów obustronnych (stadium V), guzów w jedynej nerce lub u pacjentów z zespołami predysponującymi do rozwoju wieloogniskowych guzów1112.

Podczas operacji chirurdzy dokładają wszelkich starań, aby usunąć guz w całości bez jego pęknięcia, gdyż pęknięcie guza zwiększa sześciokrotnie ryzyko wznowy miejscowej13. Jednocześnie pobierane są próbki okolicznych węzłów chłonnych w celu oceny zaawansowania choroby i dostosowania dalszego leczenia14.

Chemioterapia

Niemal wszystkie dzieci z guzem Wilmsa otrzymują chemioterapię jako część leczenia15. Chemioterapia może być stosowana:

  • Przed operacją (neoadjuwantowa) – aby zmniejszyć guz i ułatwić jego usunięcie, szczególnie w przypadku dużych guzów, guzów obustronnych lub guzów naciekających sąsiednie struktury1617.
  • Po operacji (adjuwantowa) – aby zniszczyć pozostałe komórki nowotworowe i zmniejszyć ryzyko nawrotu18.
  • W leczeniu rozsianej choroby – gdy nowotwór rozprzestrzenił się do innych organów19.

Najczęściej stosowane schematy chemioterapii w leczeniu guzów Wilmsa obejmują następujące leki2021:

  • Winkrystyna (Oncovin) – podstawowy lek w większości schematów leczenia
  • Daktynomycyna (Cosmegen, aktynomycyna-D) – powszechnie stosowana w skojarzeniu z winkrystyną w przypadkach niskiego i standardowego ryzyka
  • Doksorubicyna (Adriamycyna) – dodawana do schematów leczenia w przypadkach wyższego ryzyka
  • Cyklofosfamid (Procytox) – stosowany w zaawansowanych przypadkach
  • Etopozyd (Vepesid, VP-16) – stosowany w zaawansowanych przypadkach
  • Karboplatyna (Paraplatin) – stosowana w zaawansowanych przypadkach
  • Irynotekan (Camptosar) – stosowany w przypadkach nawrotowych lub opornych na leczenie

Dobór leków i długość terapii zależą od stadium i histologii guza. Typowe schematy chemioterapii obejmują2223:

  • Dla stadium I i II z korzystną histologią – winkrystyna i daktynomycyna (schemat EE-4A)
  • Dla stadium I i II z niekorzystnymi czynnikami biologicznymi (LOH 1p i 16q) oraz stadium III bez LOH 1p i 16q – winkrystyna, daktynomycyna i doksorubicyna (schemat DD-4A)
  • Dla stadium III i IV z LOH 1p i 16q – pięciolekowy schemat M (winkrystyna, daktynomycyna, doksorubicyna, cyklofosfamid i etopozyd)

Radioterapia

Radioterapia wykorzystuje wysokoenergetyczne promieniowanie do niszczenia komórek nowotworowych. Nie jest konieczna u wszystkich pacjentów z guzem Wilmsa, ale jest zalecana w określonych przypadkach24:

  • W stadium III – napromienianie jamy brzusznej po operacji2526
  • W stadium IV – napromienianie miejsc przerzutów, np. płuc27
  • W przypadkach z niekorzystną histologią (anaplastyczną)28
  • Gdy nowotwór nie może być całkowicie usunięty chirurgicznie29

Radioterapię pooperacyjną rozpoczyna się zwykle w ciągu 14 dni od nefrektomii. Opóźnienie rozpoczęcia radioterapii zwiększa ryzyko nawrotu30. Standardowa dawka promieniowania dla guzów Wilmsa o korzystnej histologii wynosi około 1080 cGy dla jamy brzusznej i 1200 cGy dla płuc31.

Leczenie w zależności od stadium zaawansowania

Strategia leczenia guzów Wilmsa jest ściśle uzależniona od stadium zaawansowania choroby3233:

Stadium I

Guzy w stadium I są ograniczone do nerki i mogą być całkowicie usunięte chirurgicznie. Leczenie obejmuje3435:

  • Nefrektomię z usunięciem węzłów chłonnych
  • Chemioterapię pooperacyjną – najczęściej winkrystyna i daktynomycyna przez 4-18 tygodni, w zależności od grupy ryzyka
  • W niektórych przypadkach dzieci poniżej 2 roku życia z małymi guzami (poniżej 550g) i korzystną histologią mogą być leczone samą operacją bez chemioterapii36

Stadium II

Nowotwór rozprzestrzenił się poza nerkę do otaczających tkanek, ale może być całkowicie usunięty chirurgicznie. Leczenie obejmuje3738:

  • Nefrektomię z usunięciem węzłów chłonnych
  • Chemioterapię pooperacyjną – dla guzów z korzystną histologią winkrystyna i daktynomycyna przez 18-27 tygodni
  • W przypadku niekorzystnej histologii lub czynników ryzyka (LOH 1p i 16q) – dodatkowy lek (doksorubicyna) oraz dłuższa chemioterapia (do 34 tygodni)

Stadium III

Guz nie może być całkowicie usunięty chirurgicznie lub rozprzestrzenił się do regionalnych węzłów chłonnych. Leczenie obejmuje3940:

  • Nefrektomię z usunięciem węzłów chłonnych
  • Radioterapię pooperacyjną jamy brzusznej
  • Intensywną chemioterapię – dla guzów z korzystną histologią winkrystyna i daktynomycyna przez 27 tygodni
  • W przypadku wysokiego ryzyka lub niekorzystnej histologii – chemioterapia wielolekowa (etopozyd, karboplatyna, cyklofosfamid, doksorubicyna) przez 34 tygodnie

Stadium IV

Nowotwór rozprzestrzenił się do odległych narządów, najczęściej płuc, wątroby, kości lub mózgu. Leczenie obejmuje4142:

  • Nefrektomię z usunięciem węzłów chłonnych
  • Radioterapię jamy brzusznej i miejsc przerzutów (np. płuc)
  • Intensywną chemioterapię – winkrystyna, daktynomycyna i doksorubicyna przez 6 tygodni, a następnie ocena odpowiedzi
  • W przypadku niepełnej odpowiedzi na leczenie po 6 tygodniach – zmiana na schemat 5-lekowy (schemat M) plus napromienianie płuc43

Stadium V (obustronne guzy)

Guzy występują w obu nerkach. Leczenie jest szczególnie złożone i wymaga indywidualnego podejścia4445:

  • Chemioterapia przedoperacyjna – winkrystyna i daktynomycyna (czasem z doksorubicyną) przez 6-12 tygodni w celu zmniejszenia guzów
  • Chirurgia oszczędzająca nerki (częściowa nefrektomia) mająca na celu usunięcie jak największej części guza z zachowaniem jak największej ilości funkcjonalnej tkanki nerkowej46
  • Dalsza chemioterapia pooperacyjna
  • Radioterapia w wybranych przypadkach

W przypadku stadium V, głównym celem jest zachowanie wystarczającej funkcji nerek, aby uniknąć niewydolności nerek, dializy i przeszczepu nerki4748.

Leczenie guzów Wilmsa z niekorzystną histologią

Guzy Wilmsa z histologią anaplastyczną (niekorzystną) stanowią około 5-10% wszystkich przypadków i wymagają bardziej intensywnego leczenia ze względu na gorsze rokowanie49:

  • Ogniskowa anaplazja (stadium I-III) i rozlana anaplazja (stadium I) – nefrektomia, następnie winkrystyna, daktynomycyna i doksorubicyna oraz lokalna radioterapia50
  • Rozlana anaplazja (stadium II-IV) – bardziej agresywne leczenie: nefrektomia, a następnie intensywna chemioterapia wielolekowa (irynotekan, winkrystyna, aktynomycyna-D, doksorubicyna, cyklofosfamid, karboplatyna, etopozyd) oraz radioterapia51

Leczenie nawrotu choroby

Nawrót guza Wilmsa występuje u około 10-15% pacjentów52. Leczenie choroby nawrotowej zależy od wcześniejszego leczenia, czasu, jaki upłynął od pierwszego leczenia, oraz miejsca nawrotu5354:

  • Chirurgia – usunięcie nawrotowego guza, jeśli to możliwe
  • Chemioterapia – często z użyciem leków innych niż w pierwotnym leczeniu
  • Radioterapia – jeśli nie była wcześniej stosowana w danym obszarze
  • W trudnych przypadkach – wysokodawkowa chemioterapia z przeszczepieniem komórek macierzystych55

COG klasyfikuje pacjentów z nawrotowym guzem Wilmsa do trzech grup ryzyka: standardowego, wysokiego i bardzo wysokiego, co determinuje intensywność leczenia56.

Nowe kierunki w leczeniu

Trwają badania nad nowymi metodami leczenia guzów Wilmsa, szczególnie w przypadkach wysokiego ryzyka, opornych na standardowe leczenie lub nawrotowych5758:

  • Nowe leki chemioterapeutyczne – takie jak topotekan i irynotekan59
  • Terapie celowane – ukierunkowane na specyficzne szlaki molekularne zaangażowane w rozwój guza60
  • Immunoterapia – w tym szczepionki peptydowe ukierunkowane na WT1 i inhibitory punktów kontrolnych układu immunologicznego6162
  • Przeznerkowa chemoembolizacja tętnicza (TACE) – technika, w której dostawa chemioterapeutyków i blokada dopływu krwi do guza poprawiają wyniki resekcji63
  • Radioterapia z modulacją intensywności wiązki (IMRT) – technika, w której intensywność wiązki fotonowej jest modulowana w zależności od układu tkanki docelowej i otaczających narządów64

Skuteczność leczenia i rokowanie

Dzięki postępom w leczeniu wielomodalnym, rokowanie w guzach Wilmsa jest bardzo dobre6566:

  • Ogólny wskaźnik wyleczalności wynosi około 85-90%67
  • Dla stadium I z korzystną histologią – 5-letnie przeżycie wynosi ponad 95%68
  • Dla stadium II z korzystną histologią – 5-letnie przeżycie wynosi około 90-94%69
  • Dla stadium III z korzystną histologią – 5-letnie przeżycie wynosi około 85-88%70
  • Dla stadium IV z korzystną histologią – 5-letnie przeżycie wynosi około 75-80%71
  • Dla stadium V – 5-letnie przeżycie wynosi około 75%72

Czynniki prognostyczne wpływające na rokowanie obejmują73:

  • Histopatologiczne cechy guza (korzystna vs anaplastyczna histologia)
  • Stadium zaawansowania w momencie rozpoznania
  • Cechy molekularne guza, takie jak obecność dodatkowego materiału genetycznego w chromosomie 1q oraz utrata heterozygotyczności 1p i 16q
  • Odpowiedź na wstępną chemioterapię

Opieka po leczeniu i efekty późne

Po zakończeniu leczenia dzieci z guzem Wilmsa wymagają długoterminowej obserwacji w celu monitorowania potencjalnych późnych efektów leczenia7475:

  • Zmniejszona funkcja nerek – regularna ocena funkcji nerek jest szczególnie ważna u pacjentów po nefrektomii lub radioterapii jamy brzusznej76
  • Problemy kardiologiczne – doksorubicyna może powodować kardiotoksyczność, dlatego pacjenci otrzymujący ten lek wymagają regularnej oceny kardiologicznej77
  • Opóźnienie wzrostu i rozwoju – szczególnie po radioterapii78
  • Zmiany w rozwoju płciowym – radioterapia może wpływać na funkcje gonad79
  • Zwiększone ryzyko rozwoju wtórnych nowotworów – zwłaszcza po radioterapii80

Indywidualizowany plan obserwacji określa specyficzne długoterminowe badania kontrolne i bieżące badania, których dziecko będzie wymagało81. Opieka wielodyscyplinarna po zakończeniu leczenia, obejmująca nie tylko onkologów, ale także nefrologów, kardiologów, endokrynologów i psychologów, jest kluczowa dla zapewnienia optymalnej jakości życia pacjentów wyleczonych z guza Wilmsa82.

Podsumowanie

Leczenie guzów Wilmsa jest jednym z największych sukcesów onkologii dziecięcej, z wysokimi wskaźnikami wyleczalności, szczególnie w przypadkach wczesnego wykrycia i odpowiedniego leczenia. Standardowe podejście obejmuje kombinację chirurgii, chemioterapii i w wybranych przypadkach radioterapii, dostosowanych do stadium i histologii guza oraz indywidualnych cech pacjenta8384.

Obecne badania koncentrują się na optymalizacji leczenia w celu maksymalizacji wyleczeń przy jednoczesnej minimalizacji toksyczności terapii i długotrwałych efektów ubocznych. Nowe metody leczenia, w tym terapie celowane i immunoterapia, stwarzają nadzieję na poprawę wyników u pacjentów z wysokim ryzykiem nawrotu lub opornych na standardowe leczenie8586.

Najlepsze wyniki leczenia osiąga się w specjalistycznych ośrodkach onkologii dziecięcej, gdzie multidyscyplinarny zespół składający się z chirurgów dziecięcych, onkologów, radioterapeutów, radiologów, patologów i pielęgniarek współpracuje w celu zapewnienia kompleksowej opieki nad dzieckiem z guzem Wilmsa8788.

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  1. 09.04.2026
  2. www.leksykon.com.pl

Materiały źródłowe

  • #1 Wilms Tumor Treatment | American Cancer Society
    https://www.cancer.org/cancer/types/wilms-tumor/treating.html
    If your child has been diagnosed with a Wilms tumor, the treatment team will discuss your options with you. Its important to weigh the benefits of each treatment option against the possible risks and side effects. […] Overall, about 9 of 10 children with Wilms tumors are cured. A great deal of progress has been made in treating this cancer in recent decades. Much of this progress is the result of children with Wilms tumors taking part in clinical trials of new treatments. […] Today, most children with this cancer are treated in a clinical trial to try to improve on what doctors believe is the best treatment. The goal of these studies is to find ways to cure as many children as possible, while limiting side effects by giving as little treatment as needed. […] Most children with Wilms tumors will get more than one type of treatment. The main types of treatment are: Surgery for Wilms Tumors, Chemotherapy for Wilms Tumors, Radiation Therapy for Wilms Tumors.
  • #2 Management of Wilms’ tumor: NWTS vs SIOP
    https://pmc.ncbi.nlm.nih.gov/articles/PMC2809467/
    Progress has occurred from the times when this tumor was universally fatal to this era when more than 85% of the patients can be completely cured with localized disease and over 70% for metastatic disease. […] The two major groups which have tremendous contributions in the management of Wilms’ tumor are National Wilms’ Tumor Study (NWTS) and the Societe Internationale D’oncologie Pediatrique (SIOP). […] The essential difference between the NWTS and SIOP treatment protocols is that the latter routinely gives preoperative chemotherapy. […] The recommended algorithm for management of WT as per NWTSG is Clinical staging Surgical Staging (Histological Diagnosis); details are given in Table 2. […] The algorithm for management of Wilms’ tumor as per SIOP 93-01 protocol is as shown in Table 3.
  • #3 Wilms Tumor Treatment | American Cancer Society
    https://www.cancer.org/cancer/types/wilms-tumor/treating.html
    Treatment for Wilms tumors can often be effective, but it can also cause serious side effects. Its important to discuss all treatment options and their possible side effects with your childs cancer care team so you can make an informed decision. […] Treatment options depend on several factors, including: The type, stage, and histology of the Wilms tumor, The childs age, If the tumor cells have certain chromosome changes, Possible side effects, The child and familys preferences. […] Take time to learn about all of your child’s treatment options. Be sure to ask questions about anything that is unclear. Also, talk to your childs cancer care team about the goals of each treatment and what you and your child can expect during treatment. […] The treatment team will also help you manage side effects. They can help you work closely with nutritionists, psychologists, social workers, and other professionals to understand and deal with medical problems, stress, and other issues related to treatment.
  • #4 Management of Wilms’ tumor: NWTS vs SIOP
    https://pmc.ncbi.nlm.nih.gov/articles/PMC2809467/
    With the availability of several protocols in the management of Wilms’ tumor, there is dilemma in the minds of the treating oncologists or pediatric onco-surgeons as to whether the child should receive upfront chemotherapy or should be operated upon primarily. […] It is necessary for us to understand why do we follow either of the protocols, NWTS which follows the upfront surgery principle or the SIOP which follows the upfront chemotherapy principle in all stages of the disease. […] While deciding which protocol to follow, it is imperative to know the pros and cons of the treatment strategies and also to study the outcome patterns in both the treatment regimes which is what this article highlights. […] The treatment of Wilms’ tumor can be considered as the paradigm for multimodal treatment of malignant solid tumors in childhood.
  • #5 Current treatment for Wilms tumor: COG and SIOP standards | World Journal of Pediatric Surgery
    https://wjps.bmj.com/content/2/3/e000038
    Most cases with WT are sporadic. The current survival in patients with WT is high (90%). Involvement of mutidisciplinary collaborative groups in the diagnosis and treatment of WT. National Wilms Tumor Study Group (NWTSG)/Childrens Oncology Group (COG) and The International Society of Paediatric Oncology (SIOP) are two major guidelines used for the current management of WT worldwide. The major difference exists in the two guidelines is the timing of surgery: SIOP recommends using preoperative chemotherapy, and NWTSG/COG prefers using primary surgery before any adjuvant treatments. […] Surgery, chemotherapy, and radiotherapy comprise the treatment modalities for WT. NWTSG/COG and SIOP guidelines provide two different strategies for the initial treatment of WT in children. NWTSG/COG recommends patients undergo surgery before chemotherapy. North America commonly adopts NWTSG/COG guideline. However, most children in European countries are treated with preoperative chemotherapy based on the SIOP guideline. Different treatment strategies are based on different staging systems.
  • #6 Wilms Tumor and Other Childhood Kidney Tumors Treatment – NCI
    https://www.cancer.gov/types/kidney/patient/wilms-treatment-pdq
    Childhood kidney tumors are diseases in which malignant (cancer) cells form in the tissues of the kidney. […] Treatment for Wilms tumor and other childhood kidney tumors may include genetic counseling. […] Different types of treatment are available for children with Wilms and other childhood kidney tumors. Some treatments are standard (the currently used treatment), and some are being tested in clinical trials. […] Children with Wilms tumor or other childhood kidney tumors should have their treatment planned by a team of health care providers who are experts in treating cancer in children. […] Six types of treatment are used: Surgery, Radiation therapy, Chemotherapy, Immunotherapy, High-dose chemotherapy with stem cell rescue, Targeted therapy. […] Nephrectomy: Wilms tumor and other childhood kidney tumors are usually treated with nephrectomy (surgery to remove the whole kidney).
  • #7 Wilms Tumor Treatment | American Cancer Society
    https://www.cancer.org/cancer/types/wilms-tumor/treating.html
    If your child has been diagnosed with a Wilms tumor, the treatment team will discuss your options with you. Its important to weigh the benefits of each treatment option against the possible risks and side effects. […] Overall, about 9 of 10 children with Wilms tumors are cured. A great deal of progress has been made in treating this cancer in recent decades. Much of this progress is the result of children with Wilms tumors taking part in clinical trials of new treatments. […] Today, most children with this cancer are treated in a clinical trial to try to improve on what doctors believe is the best treatment. The goal of these studies is to find ways to cure as many children as possible, while limiting side effects by giving as little treatment as needed. […] Most children with Wilms tumors will get more than one type of treatment. The main types of treatment are: Surgery for Wilms Tumors, Chemotherapy for Wilms Tumors, Radiation Therapy for Wilms Tumors.
  • #8 Wilms Tumor Treatment | American Cancer Society
    https://www.cancer.org/cancer/types/wilms-tumor/treating.html
    In the United States and Canada, surgery is the first treatment for most Wilms tumors. In Europe and some other parts of the world, doctors often prefer to give a short course of chemotherapy before the surgery. There seems to be no difference in the results from these approaches. […] The first goal of treatment is to remove the main tumor in the kidney, even if the cancer has spread to other parts of the body. […] Sometimes the tumor might be hard to remove because it is very large, it has grown into nearby blood vessels or other vital structures, or its in both kidneys. For children with these tumors, doctors might use chemotherapy, radiation therapy, or a combination of these to try to shrink the tumor(s) before surgery. […] If any cancer is left after surgery, radiation therapy or more surgery may be needed.
  • #9 4 Innovative Wilms Tumor Treatment Options | MD Anderson Cancer Center
    https://www.mdanderson.org/cancer-types/wilms-tumor/wilms-tumor-treatment.html
    Your child’s doctor will design a course of treatment especially for your child and discuss suggestions for treatment. The age and health of your child and how advanced the cancer is will help determine the type of treatment. […] Wilms’ tumor is almost always treated with more than one type of treatment usually surgery and chemotherapy. Sometimes chemotherapy or radiation therapy may be used to make the tumor smaller before surgery. If cancer cells remain after surgery, radiation therapy may be used. […] The main goal of surgery to treat Wilms’ tumor is to remove as much of the main tumor as possible. […] Two types of nephrectomy are used to treat Wilms’ tumor: […] The most common surgery to treat Wilms’ tumor. The entire kidney, along with surrounding tissue, is removed. […] When Wilms’ tumors are in both kidneys, this surgery may be done to save as much healthy tissue as possible. The cancer and some of the surrounding healthy tissue are removed.
  • #10 Current treatment for Wilms tumor: COG and SIOP standards | World Journal of Pediatric Surgery
    https://wjps.bmj.com/content/2/3/e000038
    The COG recommends primary surgery before chemotherapy. For resectable tumors, preoperative biopsy or intraoperative biopsy is also not performed. Radical nephrectomy and lymph node sampling are done through a transabdominal incision. En bloc resection can be done to avoid tumor spill. Resection of the primary renal tumor should be considered even if in a stage IV disease (usually pulmonary metastases); renal-sparing surgery is not recommended by COG guideline, except when children having a solitary kidney, with predisposition to bilateral tumors, horseshoe kidney or in infants with Denys-Drash or Frasier syndrome (to delay the need for dialysis). […] The SIOP recommends radical tumor nephrectomy performed after preoperative chemotherapy. Lymph node sampling is important for staging, and sampling seven locoregional lymph nodes is necessary for accurate staging. Nephron-sparing surgery is used for non-syndromic unilateral WTs under following conditions such as with small tumor volume (300mL) and the expectation of a substantial remnant kidney function in patients who never had lymph node involvement.
  • #11 Wilms Tumor (Kidney Tumor) | Children’s Hospital of Philadelphia
    https://www.chop.edu/conditions-diseases/wilms-tumor-kidney-tumor
    Most children with Wilms tumor will require surgery as part of their treatment plan. At CHOP, your child will be treated by a multidisciplinary team including surgeons, oncologists, radiation oncologists and nurse practitioners, who work together to create a treatment plan specific to your child. You and your child will meet with the treatment team to ensure your family has an opportunity to build a trusting relationship with them. […] The affected kidney, either in whole or sometimes in part, may be surgically removed. If the whole kidney is removed, this is called a nephrectomy. In certain situations, CHOP surgeons can perform nephron-sparing surgery (NSS), also known as partial nephrectomy, meaning that only part of the kidney is removed. NSS has been advocated in cases of bilateral Wilms tumor, for which the only alternative is bilateral nephrectomy, in cases of multifocal unilateral disease, or with Wilms tumor predisposition syndromes. NSS can be performed with a minimal complication rate and with no increase in the incidence of tumor recurrence and should be considered the standard modern approach to the management of selected cases of Wilms tumor.
  • #12 Standard of Care in the Management of Wilms Tumors in North America – American Urological Association
    https://auanews.net/issues/articles/2022/september-2022/standard-of-care-in-the-management-of-wilms-tumors-in-north-america
    Patients with bilateral tumors, unilateral tumors in the setting of a predisposition syndrome, and tumors in a solitary kidney should be treated with neoadjuvant chemotherapy with assessment of response at 6 and 12 weeks followed by nephron-sparing surgery (NSS) if feasible. Continuation of chemotherapy for longer than 12 weeks is discouraged in such cases. […] The most commonly used agents in the treatment of FHWT are vincristine, dactinomycin, and doxorubicin. Reducing or omitting exposure to doxorubicin and its cardiotoxic side effects is an important goal of cooperative trials in the WT literature. […] Patients with stage I FHWT stratified as very low risk (younger than 2 years of age and tumor weighing less than 550 gm) who had nodes sampled, which were negative, are candidates for surgical treatment alone, with excellent results (event-free survival of 89.7% and overall survival 100%).
  • #13 Wilms’ tumour: biology, diagnosis and treatment – Szychot – Translational Pediatrics
    https://tp.amegroups.org/article/view/3228/html
    Nevertheless, both trial groups agree that specific patient groups seem to benefit from pre-operative chemotherapy. […] Surgery maintains an important role in the treatment of Wilms tumor despite the fact that the improved outcome for this malignancy during the last century is assigned mainly to advances in chemotherapy. […] Careful removal of the tumor without rupture or spill is imperative because these patients have a six-fold increased risk of local abdominal relapse. […] The absence of node sampling may result in understaging and undertreatment of the tumor as reported by the NWTS group in 2005. […] Partial nephrectomy or wedge excision of the tumor is advocated for suitable cases of children with synchronous disease in both kidneys. […] Management of a child with bilateral Wilms tumor is very challenging and requires planning according to individualised patient needs, careful monitoring of response to chemotherapy, together with an understanding of the underlying histology and biology.
  • #14 Treatment of Wilms Tumor by Type and Stage | American Cancer Society
    https://www.cancer.org/cancer/types/wilms-tumor/treating/by-stage.html
    Treatment for Wilms tumor is based mainly on the cancers stage and its histology, as described in detail below. […] In the United States and Canada, doctors prefer to use surgery as the first treatment for Wilms tumor in most cases. Chemotherapy (and possibly radiation therapy) are then given afterward. […] Most often, the stage and histology of the cancer are actually determined when surgery is done to remove the cancer. This is because the true extent of the tumor often cant be determined by imaging tests alone. The doctors use what they learn during surgery to guide further treatment. […] For children older than 2 and for those of any age who have larger tumors, surgery is usually followed by chemo for several months, with the drugs actinomycin D and vincristine. […] For children of any age who have tumors with anaplastic histology, surgery is usually followed by radiation therapy to the area of the tumor, along with chemo with actinomycin D, vincristine, and possibly doxorubicin (Adriamycin) for several months.
  • #15 Chemotherapy for Wilms tumour | Canadian Cancer Society
    https://cancer.ca/en/cancer-information/cancer-types/wilms-tumour/treatment/chemotherapy
    Chemotherapy uses anticancer (cytotoxic) drugs to destroy cancer cells. Most children with Wilms tumour have chemotherapy. The healthcare team will consider your childs personal needs to plan the drugs, doses and schedules of chemotherapy. Your child may also receive other treatments. […] Chemotherapy is given for different reasons. Your child may have chemotherapy to: […] shrink a tumour before surgery (called neoadjuvant chemotherapy) […] destroy cancer cells left behind after surgery and reduce the risk that the cancer will come back (recur) (called adjuvant chemotherapy) […] destroy cancer cells in the body, relieve pain or control the symptoms of advanced Wilms tumour. […] Chemotherapy is usually a systemic therapy. This means that the drugs travel through the bloodstream to reach and destroy cancer cells all over the body, including those that may have broken away from the primary tumour in the kidney.
  • #16 Chemotherapy for Wilms tumour | Canadian Cancer Society
    https://cancer.ca/en/cancer-information/cancer-types/wilms-tumour/treatment/chemotherapy
    Chemotherapy uses anticancer (cytotoxic) drugs to destroy cancer cells. Most children with Wilms tumour have chemotherapy. The healthcare team will consider your childs personal needs to plan the drugs, doses and schedules of chemotherapy. Your child may also receive other treatments. […] Chemotherapy is given for different reasons. Your child may have chemotherapy to: […] shrink a tumour before surgery (called neoadjuvant chemotherapy) […] destroy cancer cells left behind after surgery and reduce the risk that the cancer will come back (recur) (called adjuvant chemotherapy) […] destroy cancer cells in the body, relieve pain or control the symptoms of advanced Wilms tumour. […] Chemotherapy is usually a systemic therapy. This means that the drugs travel through the bloodstream to reach and destroy cancer cells all over the body, including those that may have broken away from the primary tumour in the kidney.
  • #17 Current treatment for Wilms tumor: COG and SIOP standards | World Journal of Pediatric Surgery
    https://wjps.bmj.com/content/2/3/e000038
    The COG guideline recommends surgery as the initial therapy before chemotherapy. Preoperative chemotherapy is only indicated under the following conditions: with inoperable WT; with a solitary kidney; with synchronous bilateral WT; tumor thrombus in the inferior vena cava extending above the level of the hepatic veins; tumor involving contiguous structures whereby removing the kidney tumor requiring removal of the other organs, such as spleen, pancreas, or colon and with extensive pulmonary metastases. […] The SIOP guideline recommends preoperative chemotherapy for all patients after diagnosis. For patients with unilateral localized tumor, 4-week pretreatment with vincristine (weekly) and dactinomycin (biweekly) is given; for patients with bilateral tumors, vincristine-dactinomycin for no longer than 9-12 weeks is recommended (doxorubicin is added for reinforcement in some patients); for patients with metastasis, a regimen including 6 weeks of vincristine-dactinomycin and doxorubicin on weeks 1 and 5 is given.
  • #18 Chemotherapy for Wilms tumour | Canadian Cancer Society
    https://cancer.ca/en/cancer-information/cancer-types/wilms-tumour/treatment/chemotherapy
    Chemotherapy uses anticancer (cytotoxic) drugs to destroy cancer cells. Most children with Wilms tumour have chemotherapy. The healthcare team will consider your childs personal needs to plan the drugs, doses and schedules of chemotherapy. Your child may also receive other treatments. […] Chemotherapy is given for different reasons. Your child may have chemotherapy to: […] shrink a tumour before surgery (called neoadjuvant chemotherapy) […] destroy cancer cells left behind after surgery and reduce the risk that the cancer will come back (recur) (called adjuvant chemotherapy) […] destroy cancer cells in the body, relieve pain or control the symptoms of advanced Wilms tumour. […] Chemotherapy is usually a systemic therapy. This means that the drugs travel through the bloodstream to reach and destroy cancer cells all over the body, including those that may have broken away from the primary tumour in the kidney.
  • #19 Wilms Tumor in Children – In Treatment | CureSearch
    https://curesearch.org/Wilms-Tumor-In-Treatment/
    Chemotherapy is given to all children with Wilms tumor. It may be given: To shrink a tumor too large to remove surgically; After surgery to destroy any tumor cells that might be circulating in the body (called adjuvant therapy); To treat cancer that has spread to organs beyond the kidney. […] Radiation therapy uses x-ray light to kill cancer cells. External beam radiation therapy focuses energy onto the cancer using a radiation source outside the body. This type of radiation therapy is often used along with surgery in more advanced cases of Wilms tumor (stages III, IV and V) that have spread beyond the kidney or are not able to be completely removed at surgery.
  • #20 Chemotherapy for Wilms tumour | Canadian Cancer Society
    https://cancer.ca/en/cancer-information/cancer-types/wilms-tumour/treatment/chemotherapy
    Chemotherapy drugs used to treat Wilms tumour are: […] vincristine (Oncovin) […] dactinomycin (Cosmegen, actinomycin-D) […] doxorubicin (Adriamycin) […] cyclophosphamide (Procytox) […] cisplatin […] carboplatin (Paraplatin, Paraplatin AQ) […] etoposide (Vepesid, VP-16) […] irinotecan (Camptosar). […] The most common chemotherapy drug combinations used to treat Wilms tumour are: […] vincristine and dactinomycin […] vincristine, dactinomycin and doxorubicin […] vincristine, doxorubicin, etoposide and cyclophosphamide (with or without dactinomycin or carboplatin). […] If Wilms tumour does not respond to drugs used in earlier treatments or if it comes back, the following drug combinations may be used: […] vincristine, doxorubicin and cyclophosphamide […] ifosfamide (Ifex), carboplatin and etoposide […] cyclophosphamide and etoposide alternating with carboplatin and etoposide. […] Cyclophosphamide can irritate the bladder. When this chemotherapy drug is used, mesna (Uromitexan) may also be given to protect the bladder.
  • #21 Wilms Tumor Treatment & Management: Approach Considerations, Considerations in von Willebrand Disease, Long-Term Monitoring
    https://emedicine.medscape.com/article/989398-treatment
    The usual approach in most patients is nephrectomy followed by chemotherapy, with or without postoperative radiotherapy. […] Children found to have loss of heterozygosity at 1p and 16q receive more aggressive chemotherapy because they have a worse prognosis than do children without this heterozygosity loss. […] Some evidence suggests that certain children with stage I disease and favorable histology do well with nephrectomy alone. […] Currently, patients enrolled in the COG AREN0321 protocol for high risk Wilms tumor are treated as follows: Focal anaplastic stage I-III Wilms tumors and diffuse anaplastic stage I Wilms tumors – Nephrectomy followed by vincristine, actinomycin-D, and doxorubicin in addition to local radiotherapy. […] Stage IV diffuse anaplastic Wilms tumors – More aggressive treatment is delivered; nephrectomy is followed by initial irinotecan and vincristine administration, which in turn is followed by actinomycin-D, doxorubicin, cyclophosphamide, carboplatin, etoposide, and radiotherapy.
  • #22 Wilms Tumor Treatment & Management: Approach Considerations, Considerations in von Willebrand Disease, Long-Term Monitoring
    https://emedicine.medscape.com/article/989398-treatment
    The usual approach in most patients is nephrectomy followed by chemotherapy, with or without postoperative radiotherapy. […] Children found to have loss of heterozygosity at 1p and 16q receive more aggressive chemotherapy because they have a worse prognosis than do children without this heterozygosity loss. […] Some evidence suggests that certain children with stage I disease and favorable histology do well with nephrectomy alone. […] Currently, patients enrolled in the COG AREN0321 protocol for high risk Wilms tumor are treated as follows: Focal anaplastic stage I-III Wilms tumors and diffuse anaplastic stage I Wilms tumors – Nephrectomy followed by vincristine, actinomycin-D, and doxorubicin in addition to local radiotherapy. […] Stage IV diffuse anaplastic Wilms tumors – More aggressive treatment is delivered; nephrectomy is followed by initial irinotecan and vincristine administration, which in turn is followed by actinomycin-D, doxorubicin, cyclophosphamide, carboplatin, etoposide, and radiotherapy.
  • #23 Standard of Care in the Management of Wilms Tumors in North America – American Urological Association
    https://auanews.net/issues/articles/2022/september-2022/standard-of-care-in-the-management-of-wilms-tumors-in-north-america
    Patients with stage I and II standard risk disease (no LOH 1p and 16q) are treated with the 2-drug chemotherapy regimen known as EE-4A (vincristine and dactinomycin). In cases of stage I or II disease with unfavorable biology (LOH 1p and 16q) and stage III disease without LOH 1p and 16q, adjuvant treatment includes 3 drugs and is known as DD-4A (vincristine, dactinomycin, and doxorubicin). […] Stage III and IV patients with LOH 1p and 16q are treated with a 5-drug regimen known as regimen M, which consists of vincristine, dactinomycin, doxorubicin, cyclophosphamide, and etoposide. Of note, all patients with local stage III disease also receive abdominal radiation. […] FHWT Stage IV patients with lung metastases only are a good example of the paradigm of maximizing oncological outcomes while minimizing treatment-related morbidity. If there is no evidence of adverse biology features (LOH 1p and 16q), they are treated with 3-drug chemotherapy (DD-4A). If complete response to chemotherapy is observed in the lungs at week 6 of DD-4A, they remain on this regimen. If the lung response is incomplete, they are switched to 5-drug chemotherapy (regimen M), as well as whole lung irradiation. […] The next generation of COG studies will continue to focus on the dichotomy of improving outcomes for patients at increased risk of relapse and decreasing therapy for patients expected to experience excellent results based on risk stratification.
  • #24 4 Innovative Wilms Tumor Treatment Options | MD Anderson Cancer Center
    https://www.mdanderson.org/cancer-types/wilms-tumor/wilms-tumor-treatment.html
    Most children who are treated for Wilms’ tumor will receive chemotherapy. If a tumor is large or difficult to reach, or if there are tumors in both kidneys, chemotherapy may be given before surgery to shrink the tumors and make them easier to remove. […] Radiation therapy (also called radiotherapy) uses high-energy beams to destroy cancer cells. […] Because of our association with one of the world’s premier cancer centers, Children’s Cancer Hospital participates in many clinical trials (research studies) for Wilms’ tumors. […] Our highly specialized experts personalize care to deliver leading-edge therapies with the least impact on your child’s body now and in the future. […] Surgical skill is of the utmost importance when treating Wilms’ tumors. […] Children’s Cancer Hospital surgeons rank among the most skilled in the world. They have a high level of experience in Wilms’ tumor surgeries, using the least-invasive and most-advanced techniques. […] Childhood Wilms’ Tumor is treated in our Children’s Cancer Hospital. […] MD Anderson patients have access to clinical trials offering promising new treatments that cannot be found anywhere else.
  • #25 Wilms Tumor and Other Childhood Kidney Tumors Treatment – NCI
    https://www.cancer.gov/types/kidney/patient/wilms-treatment-pdq
    Treatment of stage I Wilms tumor with favorable histology may include: Nephrectomy with removal of lymph nodes, followed by combination chemotherapy. […] Treatment of stage II Wilms tumor with favorable histology may include: Nephrectomy with removal of lymph nodes, followed by combination chemotherapy. […] Treatment of stage III Wilms tumor with favorable histology may include: Nephrectomy with removal of lymph nodes, followed by radiation therapy to the abdomen and combination chemotherapy. […] Treatment of stage IV Wilms tumor with favorable histology may include: Nephrectomy with removal of lymph nodes, followed by radiation therapy to the abdomen and combination chemotherapy. […] Treatment of stage V Wilms tumor (bilateral) may be different for each patient and may include: Combination chemotherapy to shrink the tumor, followed by repeat imaging at 4 to 8 weeks to decide on further therapy.
  • #26 Wilms Tumor Treatment & Management: Approach Considerations, Considerations in von Willebrand Disease, Long-Term Monitoring
    https://emedicine.medscape.com/article/989398-treatment
    The usual approach in most patients is nephrectomy followed by chemotherapy, with or without postoperative radiotherapy. […] Children found to have loss of heterozygosity at 1p and 16q receive more aggressive chemotherapy because they have a worse prognosis than do children without this heterozygosity loss. […] Some evidence suggests that certain children with stage I disease and favorable histology do well with nephrectomy alone. […] Currently, patients enrolled in the COG AREN0321 protocol for high risk Wilms tumor are treated as follows: Focal anaplastic stage I-III Wilms tumors and diffuse anaplastic stage I Wilms tumors – Nephrectomy followed by vincristine, actinomycin-D, and doxorubicin in addition to local radiotherapy. […] Stage IV diffuse anaplastic Wilms tumors – More aggressive treatment is delivered; nephrectomy is followed by initial irinotecan and vincristine administration, which in turn is followed by actinomycin-D, doxorubicin, cyclophosphamide, carboplatin, etoposide, and radiotherapy.
  • #27 Current treatment for Wilms tumor: COG and SIOP standards | World Journal of Pediatric Surgery
    https://wjps.bmj.com/content/2/3/e000038
    The COG recommends postoperative chemotherapy routinely used in all patients with WT except those at a very low risk: younger than 2 years at diagnosis with stage I favorable histology tumor weighing 550g was sampled and confirmed negative lymph nodes. […] The SIOP recommends postoperative chemotherapy in all patients with WT except those with stage I low-risk tumor. […] The COG recommends postoperative radiation used to the tumor bed for all patients with tumor stage III. […] The SIOP recommends whole-abdominal radiotherapy for patients with intermediate-risk or high-risk histology tumors with major preoperative or intraoperative tumor rupture, or macroscopic peritoneal deposits; pulmonary radiotherapy is indicated for lung metastases lacking complete response until postoperative week 10.
  • #28 Treatment of Wilms Tumor by Type and Stage | American Cancer Society
    https://www.cancer.org/cancer/types/wilms-tumor/treating/by-stage.html
    Treatment for Wilms tumor is based mainly on the cancers stage and its histology, as described in detail below. […] In the United States and Canada, doctors prefer to use surgery as the first treatment for Wilms tumor in most cases. Chemotherapy (and possibly radiation therapy) are then given afterward. […] Most often, the stage and histology of the cancer are actually determined when surgery is done to remove the cancer. This is because the true extent of the tumor often cant be determined by imaging tests alone. The doctors use what they learn during surgery to guide further treatment. […] For children older than 2 and for those of any age who have larger tumors, surgery is usually followed by chemo for several months, with the drugs actinomycin D and vincristine. […] For children of any age who have tumors with anaplastic histology, surgery is usually followed by radiation therapy to the area of the tumor, along with chemo with actinomycin D, vincristine, and possibly doxorubicin (Adriamycin) for several months.
  • #29 Azthena logo with the word Azthena
    https://www.news-medical.net/health/Wilms-Tumor-Treatments.aspx
    Nearly every patient with Wilms tumor will require some form of adjuvant chemotherapy as a part of their treatment plan. The only main exception to this treatment protocol is patients with a stage 1 tumor with favorable histology. […] As Wilms tumor is usually localized to the abdominal area, radiotherapy can offer localized treatment that is able to specifically target the tumor cells. Radiotherapy is a common option in the treatment of Wilms disease and is typically initiated following surgical nephrectomy and chemotherapy. […] The stage of cancer is an important factor in the decision of which type of treatment should be administered. For a tumor with favorable histology, such as one without anaplasia, the standard treatment is: […] Children with Wilms tumor with anaplasia generally have a poorer prognosis as compared to those with favorable histology. As a result, the treatment protocol for these patients usually differs considerably.
  • #30 Wilms Tumor Medication: Antineoplastic agents
    https://emedicine.medscape.com/article/989398-medication
    The current dose for radiation therapy for favorable histology Wilms tumor is approximately 1080 cGy for the abdomen and 1200 cGy for the lung. […] Postoperative radiotherapy is started within 14 days of nephrectomy. […] The guidelines note an increased risk for recurrence in patients with a later start of radiation therapy. […] Patients with stage IV favorable histology Wilms tumor and lung metastases whose pulmonary lesions do not disappear after 6 weeks of chemotherapy receive whole-lung radiation therapy. […] Some evidence suggests that certain children with stage I disease and favorable histology do well with nephrectomy alone.
  • #31 Wilms Tumor Medication: Antineoplastic agents
    https://emedicine.medscape.com/article/989398-medication
    The current dose for radiation therapy for favorable histology Wilms tumor is approximately 1080 cGy for the abdomen and 1200 cGy for the lung. […] Postoperative radiotherapy is started within 14 days of nephrectomy. […] The guidelines note an increased risk for recurrence in patients with a later start of radiation therapy. […] Patients with stage IV favorable histology Wilms tumor and lung metastases whose pulmonary lesions do not disappear after 6 weeks of chemotherapy receive whole-lung radiation therapy. […] Some evidence suggests that certain children with stage I disease and favorable histology do well with nephrectomy alone.
  • #32 Wilms Tumor and Other Childhood Kidney Tumors Treatment – NCI
    https://www.cancer.gov/types/kidney/patient/wilms-treatment-pdq
    Treatment of stage I Wilms tumor with favorable histology may include: Nephrectomy with removal of lymph nodes, followed by combination chemotherapy. […] Treatment of stage II Wilms tumor with favorable histology may include: Nephrectomy with removal of lymph nodes, followed by combination chemotherapy. […] Treatment of stage III Wilms tumor with favorable histology may include: Nephrectomy with removal of lymph nodes, followed by radiation therapy to the abdomen and combination chemotherapy. […] Treatment of stage IV Wilms tumor with favorable histology may include: Nephrectomy with removal of lymph nodes, followed by radiation therapy to the abdomen and combination chemotherapy. […] Treatment of stage V Wilms tumor (bilateral) may be different for each patient and may include: Combination chemotherapy to shrink the tumor, followed by repeat imaging at 4 to 8 weeks to decide on further therapy.
  • #33
    https://more.navigatingcare.com/library/wilms/learn/treatment-options-for-wilms-tumor
    Treatment of stage I Wilms tumor with favorable histology may include: Nephrectomy with removal of lymph nodes, followed by combination chemotherapy. […] Treatment of stage I anaplastic Wilms tumor may include: Nephrectomy with removal of lymph nodes, followed by combination chemotherapy and radiation therapy to the flank area (either side of the body between the ribs and hipbone). […] Treatment of stage II Wilms tumor with favorable histology may include: Nephrectomy with removal of lymph nodes, followed by combination chemotherapy. […] Treatment of stage II anaplastic Wilms tumor may include: Nephrectomy with removal of lymph nodes, followed by radiation therapy to the abdomen and combination chemotherapy. […] Treatment of stage III Wilms tumor with favorable histology may include: Nephrectomy with removal of lymph nodes, followed by radiation therapy to the abdomen and combination chemotherapy.
  • #34 Treatment of Wilms Tumor by Type and Stage | American Cancer Society
    https://www.cancer.org/cancer/types/wilms-tumor/treating/by-stage.html
    Treatment for Wilms tumor is based mainly on the cancers stage and its histology, as described in detail below. […] In the United States and Canada, doctors prefer to use surgery as the first treatment for Wilms tumor in most cases. Chemotherapy (and possibly radiation therapy) are then given afterward. […] Most often, the stage and histology of the cancer are actually determined when surgery is done to remove the cancer. This is because the true extent of the tumor often cant be determined by imaging tests alone. The doctors use what they learn during surgery to guide further treatment. […] For children older than 2 and for those of any age who have larger tumors, surgery is usually followed by chemo for several months, with the drugs actinomycin D and vincristine. […] For children of any age who have tumors with anaplastic histology, surgery is usually followed by radiation therapy to the area of the tumor, along with chemo with actinomycin D, vincristine, and possibly doxorubicin (Adriamycin) for several months.
  • #35 Staging, risk groups and treatment plans for Wilms tumour | Children’s cancers | Cancer Research UK
    https://www.cancerresearchuk.org/about-cancer/childrens-cancer/wilms-tumour/staging-risk-group-treatment-plan
    Most children have 4 weeks of chemotherapy before surgery unless there are special circumstances. […] Treatment for stage 1 Wilms tumour is usually chemotherapy for 4 weeks. The chemotherapy drugs used are usually dactinomycin (also known as actinomycin D) and vincristine. This is called the AV regime. […] If your child has a medium risk tumour, they will have a further 4 weeks of chemotherapy. […] For a high risk Wilms tumour your child has 27 weeks of chemotherapy. High risk stage 1 tumours are usually treated with AV chemotherapy and another chemotherapy drug called doxorubicin. […] Treatment for stage 2 Wilms tumour is usually chemotherapy for 4 weeks. Then surgery to remove the kidney. […] If your child is low risk they usually have chemotherapy for 27 weeks. Using dactinomycin and vincristine (AV regimen).
  • #36 Standard of Care in the Management of Wilms Tumors in North America – American Urological Association
    https://auanews.net/issues/articles/2022/september-2022/standard-of-care-in-the-management-of-wilms-tumors-in-north-america
    Patients with bilateral tumors, unilateral tumors in the setting of a predisposition syndrome, and tumors in a solitary kidney should be treated with neoadjuvant chemotherapy with assessment of response at 6 and 12 weeks followed by nephron-sparing surgery (NSS) if feasible. Continuation of chemotherapy for longer than 12 weeks is discouraged in such cases. […] The most commonly used agents in the treatment of FHWT are vincristine, dactinomycin, and doxorubicin. Reducing or omitting exposure to doxorubicin and its cardiotoxic side effects is an important goal of cooperative trials in the WT literature. […] Patients with stage I FHWT stratified as very low risk (younger than 2 years of age and tumor weighing less than 550 gm) who had nodes sampled, which were negative, are candidates for surgical treatment alone, with excellent results (event-free survival of 89.7% and overall survival 100%).
  • #37 Wilms Tumor and Other Childhood Kidney Tumors Treatment – NCI
    https://www.cancer.gov/types/kidney/patient/wilms-treatment-pdq
    Treatment of stage I Wilms tumor with favorable histology may include: Nephrectomy with removal of lymph nodes, followed by combination chemotherapy. […] Treatment of stage II Wilms tumor with favorable histology may include: Nephrectomy with removal of lymph nodes, followed by combination chemotherapy. […] Treatment of stage III Wilms tumor with favorable histology may include: Nephrectomy with removal of lymph nodes, followed by radiation therapy to the abdomen and combination chemotherapy. […] Treatment of stage IV Wilms tumor with favorable histology may include: Nephrectomy with removal of lymph nodes, followed by radiation therapy to the abdomen and combination chemotherapy. […] Treatment of stage V Wilms tumor (bilateral) may be different for each patient and may include: Combination chemotherapy to shrink the tumor, followed by repeat imaging at 4 to 8 weeks to decide on further therapy.
  • #38 Staging, risk groups and treatment plans for Wilms tumour | Children’s cancers | Cancer Research UK
    https://www.cancerresearchuk.org/about-cancer/childrens-cancer/wilms-tumour/staging-risk-group-treatment-plan
    Most children have 4 weeks of chemotherapy before surgery unless there are special circumstances. […] Treatment for stage 1 Wilms tumour is usually chemotherapy for 4 weeks. The chemotherapy drugs used are usually dactinomycin (also known as actinomycin D) and vincristine. This is called the AV regime. […] If your child has a medium risk tumour, they will have a further 4 weeks of chemotherapy. […] For a high risk Wilms tumour your child has 27 weeks of chemotherapy. High risk stage 1 tumours are usually treated with AV chemotherapy and another chemotherapy drug called doxorubicin. […] Treatment for stage 2 Wilms tumour is usually chemotherapy for 4 weeks. Then surgery to remove the kidney. […] If your child is low risk they usually have chemotherapy for 27 weeks. Using dactinomycin and vincristine (AV regimen).
  • #39 Wilms Tumor and Other Childhood Kidney Tumors Treatment – NCI
    https://www.cancer.gov/types/kidney/patient/wilms-treatment-pdq
    Treatment of stage I Wilms tumor with favorable histology may include: Nephrectomy with removal of lymph nodes, followed by combination chemotherapy. […] Treatment of stage II Wilms tumor with favorable histology may include: Nephrectomy with removal of lymph nodes, followed by combination chemotherapy. […] Treatment of stage III Wilms tumor with favorable histology may include: Nephrectomy with removal of lymph nodes, followed by radiation therapy to the abdomen and combination chemotherapy. […] Treatment of stage IV Wilms tumor with favorable histology may include: Nephrectomy with removal of lymph nodes, followed by radiation therapy to the abdomen and combination chemotherapy. […] Treatment of stage V Wilms tumor (bilateral) may be different for each patient and may include: Combination chemotherapy to shrink the tumor, followed by repeat imaging at 4 to 8 weeks to decide on further therapy.
  • #40 Staging, risk groups and treatment plans for Wilms tumour | Children’s cancers | Cancer Research UK
    https://www.cancerresearchuk.org/about-cancer/childrens-cancer/wilms-tumour/staging-risk-group-treatment-plan
    Treatment for high risk stage 2 Wilms tumours after surgery is usually more intensive chemotherapy for 34 weeks. […] Treatment for stage 3 Wilms tumor is usually chemotherapy for 4 weeks. Then surgery to remove the kidney. […] For low risk stage 3 tumours they have 27 weeks of chemotherapy with dactinomycin and vincristine. […] For high risk stage 3 Wilms tumour your child usually has the following chemotherapy drugs for 34 weeks: etoposide, carboplatin, cyclophosphamide, doxorubicin. […] Treatment for stage 4 Wilms tumour is usually chemotherapy for 6 weeks. Using dactinomycin, vincristine, and doxorubicin. Then surgery to remove the kidney. […] Treatment is usually chemotherapy first. This may be up to 12 weeks. Then surgery to remove as much of the cancer as possible from each kidney. […] In most situations, children 6 months and older usually have chemotherapy before their surgery. Children younger than 6 months usually have surgery first then have chemotherapy. […] Chemotherapy and surgery are the main treatments for Wilms tumour.
  • #41 Wilms Tumor and Other Childhood Kidney Tumors Treatment – NCI
    https://www.cancer.gov/types/kidney/patient/wilms-treatment-pdq
    Treatment of stage I Wilms tumor with favorable histology may include: Nephrectomy with removal of lymph nodes, followed by combination chemotherapy. […] Treatment of stage II Wilms tumor with favorable histology may include: Nephrectomy with removal of lymph nodes, followed by combination chemotherapy. […] Treatment of stage III Wilms tumor with favorable histology may include: Nephrectomy with removal of lymph nodes, followed by radiation therapy to the abdomen and combination chemotherapy. […] Treatment of stage IV Wilms tumor with favorable histology may include: Nephrectomy with removal of lymph nodes, followed by radiation therapy to the abdomen and combination chemotherapy. […] Treatment of stage V Wilms tumor (bilateral) may be different for each patient and may include: Combination chemotherapy to shrink the tumor, followed by repeat imaging at 4 to 8 weeks to decide on further therapy.
  • #42 Staging, risk groups and treatment plans for Wilms tumour | Children’s cancers | Cancer Research UK
    https://www.cancerresearchuk.org/about-cancer/childrens-cancer/wilms-tumour/staging-risk-group-treatment-plan
    Treatment for high risk stage 2 Wilms tumours after surgery is usually more intensive chemotherapy for 34 weeks. […] Treatment for stage 3 Wilms tumor is usually chemotherapy for 4 weeks. Then surgery to remove the kidney. […] For low risk stage 3 tumours they have 27 weeks of chemotherapy with dactinomycin and vincristine. […] For high risk stage 3 Wilms tumour your child usually has the following chemotherapy drugs for 34 weeks: etoposide, carboplatin, cyclophosphamide, doxorubicin. […] Treatment for stage 4 Wilms tumour is usually chemotherapy for 6 weeks. Using dactinomycin, vincristine, and doxorubicin. Then surgery to remove the kidney. […] Treatment is usually chemotherapy first. This may be up to 12 weeks. Then surgery to remove as much of the cancer as possible from each kidney. […] In most situations, children 6 months and older usually have chemotherapy before their surgery. Children younger than 6 months usually have surgery first then have chemotherapy. […] Chemotherapy and surgery are the main treatments for Wilms tumour.
  • #43 Standard of Care in the Management of Wilms Tumors in North America – American Urological Association
    https://auanews.net/issues/articles/2022/september-2022/standard-of-care-in-the-management-of-wilms-tumors-in-north-america
    Patients with stage I and II standard risk disease (no LOH 1p and 16q) are treated with the 2-drug chemotherapy regimen known as EE-4A (vincristine and dactinomycin). In cases of stage I or II disease with unfavorable biology (LOH 1p and 16q) and stage III disease without LOH 1p and 16q, adjuvant treatment includes 3 drugs and is known as DD-4A (vincristine, dactinomycin, and doxorubicin). […] Stage III and IV patients with LOH 1p and 16q are treated with a 5-drug regimen known as regimen M, which consists of vincristine, dactinomycin, doxorubicin, cyclophosphamide, and etoposide. Of note, all patients with local stage III disease also receive abdominal radiation. […] FHWT Stage IV patients with lung metastases only are a good example of the paradigm of maximizing oncological outcomes while minimizing treatment-related morbidity. If there is no evidence of adverse biology features (LOH 1p and 16q), they are treated with 3-drug chemotherapy (DD-4A). If complete response to chemotherapy is observed in the lungs at week 6 of DD-4A, they remain on this regimen. If the lung response is incomplete, they are switched to 5-drug chemotherapy (regimen M), as well as whole lung irradiation. […] The next generation of COG studies will continue to focus on the dichotomy of improving outcomes for patients at increased risk of relapse and decreasing therapy for patients expected to experience excellent results based on risk stratification.
  • #44 Wilms Tumor and Other Childhood Kidney Tumors Treatment – NCI
    https://www.cancer.gov/types/kidney/patient/wilms-treatment-pdq
    Treatment of stage I Wilms tumor with favorable histology may include: Nephrectomy with removal of lymph nodes, followed by combination chemotherapy. […] Treatment of stage II Wilms tumor with favorable histology may include: Nephrectomy with removal of lymph nodes, followed by combination chemotherapy. […] Treatment of stage III Wilms tumor with favorable histology may include: Nephrectomy with removal of lymph nodes, followed by radiation therapy to the abdomen and combination chemotherapy. […] Treatment of stage IV Wilms tumor with favorable histology may include: Nephrectomy with removal of lymph nodes, followed by radiation therapy to the abdomen and combination chemotherapy. […] Treatment of stage V Wilms tumor (bilateral) may be different for each patient and may include: Combination chemotherapy to shrink the tumor, followed by repeat imaging at 4 to 8 weeks to decide on further therapy.
  • #45 Current treatment for Wilms tumor: COG and SIOP standards | World Journal of Pediatric Surgery
    https://wjps.bmj.com/content/2/3/e000038
    Both the COG and SIOP recommends preoperative chemotherapy and resection for bilateral WT. […] The SIOP recommends primary nephrectomy for infants younger than 6 months unless tumors are judged not suitable to immediate nephrectomy. […] The COG guideline has categorized the patients with recurrent WT into three risk groups: standard risk, high risk and very high risk. For standard-risk relapsed WTs, surgery is given when feasible; radiation therapy and chemotherapy are given. For patients with high risk and very high risk relapsed WTs, chemotherapy, surgery, and/or radiation therapy and hematopoietic stem cell transplantation are recommended. […] The SIOP classifies the patients with recurrent WT into group AA, group BB and group CC. For patients in group AA, only vincristine and/or dactinomycin (no radiotherapy) is adopted as the first-line treatment.
  • #46 Wilms Tumor Treatment | St. Jude Care & Treatment
    https://www.stjude.org/care-treatment/treatment/childhood-cancer/solid-tumors/wilms-tumor.html
    Wilms tumor treatment will depend on your childs age and how much the disease has spread. […] Treatments can include: Surgery, Chemotherapy Treatment, Radiation therapy. […] St. Jude has extensive experience treating children with Wilms tumors in 1 or both kidneys. […] Our surgeons pioneered a way to remove the malignant tumor when the Wilms tumor is in both kidneys. This approach, called nephron-sparing surgery, helps surgeons save healthy kidney tissue. Survival rates are excellent. The surgery also helps children avoid kidney failure, dialysis, and kidney transplant. […] Our doctors and researchers take part in many cooperative trials through the Childrens Oncology Group and other organizations. These trials are focused on improving Wilms tumor treatments and therapies. […] Overall outcomes of Wilms tumor treatments and therapies at St. Jude are quite good. Each has improved significantly in the last 50 years.
  • #47 Wilms Tumor Treatment | St. Jude Care & Treatment
    https://www.stjude.org/care-treatment/treatment/childhood-cancer/solid-tumors/wilms-tumor.html
    Wilms tumor treatment will depend on your childs age and how much the disease has spread. […] Treatments can include: Surgery, Chemotherapy Treatment, Radiation therapy. […] St. Jude has extensive experience treating children with Wilms tumors in 1 or both kidneys. […] Our surgeons pioneered a way to remove the malignant tumor when the Wilms tumor is in both kidneys. This approach, called nephron-sparing surgery, helps surgeons save healthy kidney tissue. Survival rates are excellent. The surgery also helps children avoid kidney failure, dialysis, and kidney transplant. […] Our doctors and researchers take part in many cooperative trials through the Childrens Oncology Group and other organizations. These trials are focused on improving Wilms tumor treatments and therapies. […] Overall outcomes of Wilms tumor treatments and therapies at St. Jude are quite good. Each has improved significantly in the last 50 years.
  • #48 Standard of Care in the Management of Wilms Tumors in North America – American Urological Association
    https://auanews.net/issues/articles/2022/september-2022/standard-of-care-in-the-management-of-wilms-tumors-in-north-america
    Patients with bilateral tumors, unilateral tumors in the setting of a predisposition syndrome, and tumors in a solitary kidney should be treated with neoadjuvant chemotherapy with assessment of response at 6 and 12 weeks followed by nephron-sparing surgery (NSS) if feasible. Continuation of chemotherapy for longer than 12 weeks is discouraged in such cases. […] The most commonly used agents in the treatment of FHWT are vincristine, dactinomycin, and doxorubicin. Reducing or omitting exposure to doxorubicin and its cardiotoxic side effects is an important goal of cooperative trials in the WT literature. […] Patients with stage I FHWT stratified as very low risk (younger than 2 years of age and tumor weighing less than 550 gm) who had nodes sampled, which were negative, are candidates for surgical treatment alone, with excellent results (event-free survival of 89.7% and overall survival 100%).
  • #49 Wilms Tumor Treatment & Management: Approach Considerations, Considerations in von Willebrand Disease, Long-Term Monitoring
    https://emedicine.medscape.com/article/989398-treatment
    The usual approach in most patients is nephrectomy followed by chemotherapy, with or without postoperative radiotherapy. […] Children found to have loss of heterozygosity at 1p and 16q receive more aggressive chemotherapy because they have a worse prognosis than do children without this heterozygosity loss. […] Some evidence suggests that certain children with stage I disease and favorable histology do well with nephrectomy alone. […] Currently, patients enrolled in the COG AREN0321 protocol for high risk Wilms tumor are treated as follows: Focal anaplastic stage I-III Wilms tumors and diffuse anaplastic stage I Wilms tumors – Nephrectomy followed by vincristine, actinomycin-D, and doxorubicin in addition to local radiotherapy. […] Stage IV diffuse anaplastic Wilms tumors – More aggressive treatment is delivered; nephrectomy is followed by initial irinotecan and vincristine administration, which in turn is followed by actinomycin-D, doxorubicin, cyclophosphamide, carboplatin, etoposide, and radiotherapy.
  • #50 Wilms Tumor Treatment & Management: Approach Considerations, Considerations in von Willebrand Disease, Long-Term Monitoring
    https://emedicine.medscape.com/article/989398-treatment
    The usual approach in most patients is nephrectomy followed by chemotherapy, with or without postoperative radiotherapy. […] Children found to have loss of heterozygosity at 1p and 16q receive more aggressive chemotherapy because they have a worse prognosis than do children without this heterozygosity loss. […] Some evidence suggests that certain children with stage I disease and favorable histology do well with nephrectomy alone. […] Currently, patients enrolled in the COG AREN0321 protocol for high risk Wilms tumor are treated as follows: Focal anaplastic stage I-III Wilms tumors and diffuse anaplastic stage I Wilms tumors – Nephrectomy followed by vincristine, actinomycin-D, and doxorubicin in addition to local radiotherapy. […] Stage IV diffuse anaplastic Wilms tumors – More aggressive treatment is delivered; nephrectomy is followed by initial irinotecan and vincristine administration, which in turn is followed by actinomycin-D, doxorubicin, cyclophosphamide, carboplatin, etoposide, and radiotherapy.
  • #51 Wilms Tumor Treatment & Management: Approach Considerations, Considerations in von Willebrand Disease, Long-Term Monitoring
    https://emedicine.medscape.com/article/989398-treatment
    The usual approach in most patients is nephrectomy followed by chemotherapy, with or without postoperative radiotherapy. […] Children found to have loss of heterozygosity at 1p and 16q receive more aggressive chemotherapy because they have a worse prognosis than do children without this heterozygosity loss. […] Some evidence suggests that certain children with stage I disease and favorable histology do well with nephrectomy alone. […] Currently, patients enrolled in the COG AREN0321 protocol for high risk Wilms tumor are treated as follows: Focal anaplastic stage I-III Wilms tumors and diffuse anaplastic stage I Wilms tumors – Nephrectomy followed by vincristine, actinomycin-D, and doxorubicin in addition to local radiotherapy. […] Stage IV diffuse anaplastic Wilms tumors – More aggressive treatment is delivered; nephrectomy is followed by initial irinotecan and vincristine administration, which in turn is followed by actinomycin-D, doxorubicin, cyclophosphamide, carboplatin, etoposide, and radiotherapy.
  • #52 Wilms tumor | Alex’s Lemonade Stand Foundation for Childhood Cancer
    https://www.alexslemonade.org/childhood-cancer/guides/childhood-cancer/chapter-7-kidney-tumors/wilms-tumor
    Following is a discussion of treatment for tumors in only one kidney. […] A pediatric surgeon with experience operating on children with kidney cancer should do all biopsies and surgeries. In North America, children diagnosed with Wilms tumor usually have surgery to remove the kidney, called a nephrectomy, before any other therapy is given. […] The vast majority of children diagnosed with Wilms tumor receive chemotherapy (drugs that kill cancer cells) as part of the standard treatment. Several chemotherapy drugs are effective against this type of cancer. The use of dactinomycin and vincristine has dramatically increased survival rates. […] The decision to use radiation therapy to treat a child with Wilms tumor is based largely on the stage and histology of the tumor. […] The goal of treatment for stage V Wilms tumor is to try to destroy the tumors while preserving as much healthy kidney tissue as possible. Current treatments use chemotherapy (vincristine, dactinomycin, and doxorubicin) to reduce the size of the tumors before surgery. […] After treatment, there is a chance that the Wilms tumor will return (called a relapse or recurrence). The likelihood of relapse depends on the initial stage and histology, but the average relapse rate is only 10 percent.
  • #53 Treatment of Wilms Tumor by Type and Stage | American Cancer Society
    https://www.cancer.org/cancer/types/wilms-tumor/treating/by-stage.html
    After surgery, standard treatment is chemo with actinomycin D and vincristine. If the tumor cells have certain chromosome changes, the drug doxorubicin (Adriamycin) may be given as well. The chemo is given for several months. […] Treatment is usually surgery if it can be done, followed by radiation therapy over several days. This is followed by chemo with 3 drugs (actinomycin D, vincristine, and doxorubicin). […] Treatment might start with surgery if it can be done. This is often followed by chemo with the drugs vincristine, doxorubicin, etoposide, cyclophosphamide, and carboplatin, along with mesna given for about 6 months. […] The usual treatment for these children is surgery to remove the recurrent cancer (if possible), radiation therapy (if not already given to the area), and chemo, often with drugs different from those used during first treatment.
  • #54 Current treatment for Wilms tumor: COG and SIOP standards | World Journal of Pediatric Surgery
    https://wjps.bmj.com/content/2/3/e000038
    Both the COG and SIOP recommends preoperative chemotherapy and resection for bilateral WT. […] The SIOP recommends primary nephrectomy for infants younger than 6 months unless tumors are judged not suitable to immediate nephrectomy. […] The COG guideline has categorized the patients with recurrent WT into three risk groups: standard risk, high risk and very high risk. For standard-risk relapsed WTs, surgery is given when feasible; radiation therapy and chemotherapy are given. For patients with high risk and very high risk relapsed WTs, chemotherapy, surgery, and/or radiation therapy and hematopoietic stem cell transplantation are recommended. […] The SIOP classifies the patients with recurrent WT into group AA, group BB and group CC. For patients in group AA, only vincristine and/or dactinomycin (no radiotherapy) is adopted as the first-line treatment.
  • #55 Wilms Tumor | Boston Children’s Hospital
    https://www.childrenshospital.org/conditions/wilms-tumor
    Surgery is usually followed by chemotherapy and sometimes radiation therapy. The type of chemotherapy and need for radiation are determined by tumor stage (extent of spread of the tumor) and tumor type. Radiation therapy may be indicated for some tumors, including any that have spread to the lymph nodes in the abdomen or the lungs. […] The goal of treatment is both to cure the cancer and minimize the long-term effects of therapy. […] With prompt, aggressive treatment, most children with Wilms tumor are successfully treated and experience relatively few side effects. More aggressive tumors that have spread significantly may be harder to cure. Should a tumor come back after treatment, your doctor may recommend additional surgery, chemotherapy, and/or radiation therapy. […] In very rare cases, a stem cell transplant, involving a transplant of your child’s own stem cells, may be used. This allows your child’s physician to give high-intensity chemotherapy in difficult-to-treat cases of Wilms tumor.
  • #56 Current treatment for Wilms tumor: COG and SIOP standards | World Journal of Pediatric Surgery
    https://wjps.bmj.com/content/2/3/e000038
    Both the COG and SIOP recommends preoperative chemotherapy and resection for bilateral WT. […] The SIOP recommends primary nephrectomy for infants younger than 6 months unless tumors are judged not suitable to immediate nephrectomy. […] The COG guideline has categorized the patients with recurrent WT into three risk groups: standard risk, high risk and very high risk. For standard-risk relapsed WTs, surgery is given when feasible; radiation therapy and chemotherapy are given. For patients with high risk and very high risk relapsed WTs, chemotherapy, surgery, and/or radiation therapy and hematopoietic stem cell transplantation are recommended. […] The SIOP classifies the patients with recurrent WT into group AA, group BB and group CC. For patients in group AA, only vincristine and/or dactinomycin (no radiotherapy) is adopted as the first-line treatment.
  • #57 Wilms Tumor – StatPearls – NCBI Bookshelf
    https://www.ncbi.nlm.nih.gov/books/NBK442004/
    Patients who relapse after initial combined therapy tend to have a worse prognosis than newly discovered Wilms patients. […] The hepatic veno-occlusive disease can occur in patients receiving therapy for Wilms. It is characterized by right upper quadrant pain associated with jaundice, ascites, weight gain, and/or hepatomegaly. Treatment for hepatic veno-occlusive disease is mainly supportive. […] New chemotherapy drugs appear promising, such as topotecan and irinotecan, while stem cell transplants and targeted therapies represent promising new approaches to Wilms tumor treatment.
  • #58 The future of Wilms tumor therapies – Children’s National
    https://innovationdistrict.childrensnational.org/the-future-of-wilms-tumor-therapies/
    Dr. Domes mission is to come up with other therapies to treat Wilms tumor. […] Our mission is to come up with other therapies that work differently. […] Therefore, we must think about other forms of therapy for Wilms tumor, such as immunotherapy. […] I think the future of Wilms tumor therapy will be combining the classic chemotherapy and radiation therapy that weve used for many years but also adding another component for the patients with the highest risk of relapse, such as immunotherapy. […] Up until now, the studies that weve conducted using immunotherapy have introduced this treatment very late as a last resort after everything else has failed. […] I believe the prospects for Wilms tumor are also exciting.
  • #59 Wilms Tumor – StatPearls – NCBI Bookshelf
    https://www.ncbi.nlm.nih.gov/books/NBK442004/
    Patients who relapse after initial combined therapy tend to have a worse prognosis than newly discovered Wilms patients. […] The hepatic veno-occlusive disease can occur in patients receiving therapy for Wilms. It is characterized by right upper quadrant pain associated with jaundice, ascites, weight gain, and/or hepatomegaly. Treatment for hepatic veno-occlusive disease is mainly supportive. […] New chemotherapy drugs appear promising, such as topotecan and irinotecan, while stem cell transplants and targeted therapies represent promising new approaches to Wilms tumor treatment.
  • #60 Current Recommendations, Controversies, and Potential Novel Approaches in the Treatment of Wilms Tumor | Published in Georgetown Medical Review
    https://gmr.scholasticahq.com/article/18059-current-recommendations-controversies-and-potential-novel-approaches-in-the-treatment-of-wilms-tumor
    These results may be inaccurate due to inconsistent reporting and small sample sizes of partial nephrectomy cases at most institutions. Nonetheless, there have been reported benefits of partial nephrectomies over radical nephrectomies in WT, such as a reduced risk for developing severe chronic kidney disease. […] Radiotherapy significantly improves the overall survival of patients with WT lung metastases, as shown by the NWTS-3 and 4 which found that the overall survival rate was 91% after whole lung irradiation compared to 85% with just chemotherapy alone. […] Intensity-modulated radiation therapy (IMRT) is a technique where photon beam intensity is modulated based on the arrangement of the target tissue and surrounding organs. […] The prospective use of Wnt/ -catenin pathway inhibitors, miRNA, M6620, and stem cell therapy in treating this disease is particularly intriguing.
  • #61 Wilms Tumor: Symptoms, Causes, Stages, Diagnosis and Treatment – OncoDaily
    https://oncodaily.com/oncolibrary/cancer-types/234450
    Recent advancements in Wilms tumor treatment have focused on targeted therapies and immunotherapies to improve outcomes, especially in high-risk cases. According to a 2024 study by Choochuen et al. published in Cancers, exome sequencing of 43 Wilms tumor samples identified mutations in genes such as ALPK2, C16orf96, PRKDC, and SVIL that correlate with chemotherapy resistance. These findings suggest that targeting these mutations with specific agents could overcome resistance and improve treatment efficacy. […] A 2024 narrative review in Cancer Immunology, Immunotherapy highlighted the development of WT1-targeted peptide vaccines. Phase I/II clinical trials have demonstrated their safety and efficacy across various cancers, including Wilms tumor. The combination of these vaccines with immune checkpoint inhibitors has shown synergistic effects, enhancing anti-tumor responses.
  • #62 The future of Wilms tumor therapies – Children’s National
    https://innovationdistrict.childrensnational.org/the-future-of-wilms-tumor-therapies/
    Dr. Domes mission is to come up with other therapies to treat Wilms tumor. […] Our mission is to come up with other therapies that work differently. […] Therefore, we must think about other forms of therapy for Wilms tumor, such as immunotherapy. […] I think the future of Wilms tumor therapy will be combining the classic chemotherapy and radiation therapy that weve used for many years but also adding another component for the patients with the highest risk of relapse, such as immunotherapy. […] Up until now, the studies that weve conducted using immunotherapy have introduced this treatment very late as a last resort after everything else has failed. […] I believe the prospects for Wilms tumor are also exciting.
  • #63 Current Recommendations, Controversies, and Potential Novel Approaches in the Treatment of Wilms Tumor | Published in Georgetown Medical Review
    https://gmr.scholasticahq.com/article/18059-current-recommendations-controversies-and-potential-novel-approaches-in-the-treatment-of-wilms-tumor
    Chemotherapeutic treatment regimens for WT include baseline medications of vincristine and dactinomycin for the majority of patients with additional agents added depending on the risk of recurrence and severity of disease stage. […] The main argument in favor of preoperative chemotherapy is that it significantly decreases tumor size prior to surgical resection which reduces the risk of tumor rupture during surgery. […] Renal transcatheter arterial chemoembolization (TACE), a technique where blood supply to a tumor is blocked using chemotherapeutic agents, has recently been used as a newer ancillary treatment approach to improve WT resection outcomes. […] Currently, the United States follows the recommendations laid out by the COG guidelines, so upfront resection prior to chemotherapy remains the cornerstone treatment for WT.
  • #64 Current Recommendations, Controversies, and Potential Novel Approaches in the Treatment of Wilms Tumor | Published in Georgetown Medical Review
    https://gmr.scholasticahq.com/article/18059-current-recommendations-controversies-and-potential-novel-approaches-in-the-treatment-of-wilms-tumor
    These results may be inaccurate due to inconsistent reporting and small sample sizes of partial nephrectomy cases at most institutions. Nonetheless, there have been reported benefits of partial nephrectomies over radical nephrectomies in WT, such as a reduced risk for developing severe chronic kidney disease. […] Radiotherapy significantly improves the overall survival of patients with WT lung metastases, as shown by the NWTS-3 and 4 which found that the overall survival rate was 91% after whole lung irradiation compared to 85% with just chemotherapy alone. […] Intensity-modulated radiation therapy (IMRT) is a technique where photon beam intensity is modulated based on the arrangement of the target tissue and surrounding organs. […] The prospective use of Wnt/ -catenin pathway inhibitors, miRNA, M6620, and stem cell therapy in treating this disease is particularly intriguing.
  • #65 Wilms Tumor Treatment | American Cancer Society
    https://www.cancer.org/cancer/types/wilms-tumor/treating.html
    If your child has been diagnosed with a Wilms tumor, the treatment team will discuss your options with you. Its important to weigh the benefits of each treatment option against the possible risks and side effects. […] Overall, about 9 of 10 children with Wilms tumors are cured. A great deal of progress has been made in treating this cancer in recent decades. Much of this progress is the result of children with Wilms tumors taking part in clinical trials of new treatments. […] Today, most children with this cancer are treated in a clinical trial to try to improve on what doctors believe is the best treatment. The goal of these studies is to find ways to cure as many children as possible, while limiting side effects by giving as little treatment as needed. […] Most children with Wilms tumors will get more than one type of treatment. The main types of treatment are: Surgery for Wilms Tumors, Chemotherapy for Wilms Tumors, Radiation Therapy for Wilms Tumors.
  • #66 Management of Wilms’ tumor: NWTS vs SIOP
    https://pmc.ncbi.nlm.nih.gov/articles/PMC2809467/
    Progress has occurred from the times when this tumor was universally fatal to this era when more than 85% of the patients can be completely cured with localized disease and over 70% for metastatic disease. […] The two major groups which have tremendous contributions in the management of Wilms’ tumor are National Wilms’ Tumor Study (NWTS) and the Societe Internationale D’oncologie Pediatrique (SIOP). […] The essential difference between the NWTS and SIOP treatment protocols is that the latter routinely gives preoperative chemotherapy. […] The recommended algorithm for management of WT as per NWTSG is Clinical staging Surgical Staging (Histological Diagnosis); details are given in Table 2. […] The algorithm for management of Wilms’ tumor as per SIOP 93-01 protocol is as shown in Table 3.
  • #67 Wilms Tumor (Kidney Tumor) | Children’s Hospital of Philadelphia
    https://www.chop.edu/conditions-diseases/wilms-tumor-kidney-tumor
    At CHOP, your child will be treated by a multidisciplinary team including surgeons, oncologists, urologists, radiologists, radiation oncologists, pathologists and nurse practitioners, who work together to create a treatment plan specific to your child. You and your child will meet with the treatment team to ensure your family has an opportunity to build a trusting relationship with them. […] Proton therapy, a more targeted form of radiation therapy, is offered at Children’s Hospital of Philadelphia as a treatment option for Wilms tumor. This therapy is provided in partnership with Penn Medicine at the Roberts Proton Therapy Center. […] The overall cure rate for Wilms tumor is 85 percent. As with any cancer, however, prognosis and long-term survival can vary greatly from child to child. Prompt medical attention and aggressive therapy are important for the best prognosis. Continuous follow-up care is essential for a child diagnosed with Wilms tumor because side effects of radiation and chemotherapy may occur as well as second malignancy.
  • #68
    https://link.springer.com/article/10.1007/BF00183143
    Between June 1980 and June 1988, 373 patients with Wilms’ tumors were diagnosed and treated (34.5%, stage I; 34.5%, stage II; 14.8%, stage III; 10.7%, stage IV; and 5.4%, stage V). […] Aside from radical tumor nephrectomy, treatment encompassed chemotherapy in all cases and radiotherapy in half of the patients according to the study protocol. […] Overall, 81.8% (305/373) of the patients were disease-free according to the probability of disease-free survival after 6 years as calculated using the Kaplan-Meier method, with 90% of the protocol group (196/218) being free of disease. […] After primary surgery and subsequent chemotherapy, 97.3% of the children with standard-type Wilms’ tumors of stage I were cured and 94% of those with stage II tumors survived disease-free, as did 88% of those in stage III, 46% of those in stage IV, and 75% of those in stage V (Kaplan-Meier method).
  • #69
    https://link.springer.com/article/10.1007/BF00183143
    Between June 1980 and June 1988, 373 patients with Wilms’ tumors were diagnosed and treated (34.5%, stage I; 34.5%, stage II; 14.8%, stage III; 10.7%, stage IV; and 5.4%, stage V). […] Aside from radical tumor nephrectomy, treatment encompassed chemotherapy in all cases and radiotherapy in half of the patients according to the study protocol. […] Overall, 81.8% (305/373) of the patients were disease-free according to the probability of disease-free survival after 6 years as calculated using the Kaplan-Meier method, with 90% of the protocol group (196/218) being free of disease. […] After primary surgery and subsequent chemotherapy, 97.3% of the children with standard-type Wilms’ tumors of stage I were cured and 94% of those with stage II tumors survived disease-free, as did 88% of those in stage III, 46% of those in stage IV, and 75% of those in stage V (Kaplan-Meier method).
  • #70
    https://link.springer.com/article/10.1007/BF00183143
    Between June 1980 and June 1988, 373 patients with Wilms’ tumors were diagnosed and treated (34.5%, stage I; 34.5%, stage II; 14.8%, stage III; 10.7%, stage IV; and 5.4%, stage V). […] Aside from radical tumor nephrectomy, treatment encompassed chemotherapy in all cases and radiotherapy in half of the patients according to the study protocol. […] Overall, 81.8% (305/373) of the patients were disease-free according to the probability of disease-free survival after 6 years as calculated using the Kaplan-Meier method, with 90% of the protocol group (196/218) being free of disease. […] After primary surgery and subsequent chemotherapy, 97.3% of the children with standard-type Wilms’ tumors of stage I were cured and 94% of those with stage II tumors survived disease-free, as did 88% of those in stage III, 46% of those in stage IV, and 75% of those in stage V (Kaplan-Meier method).
  • #71
    https://link.springer.com/article/10.1007/BF00183143
    Between June 1980 and June 1988, 373 patients with Wilms’ tumors were diagnosed and treated (34.5%, stage I; 34.5%, stage II; 14.8%, stage III; 10.7%, stage IV; and 5.4%, stage V). […] Aside from radical tumor nephrectomy, treatment encompassed chemotherapy in all cases and radiotherapy in half of the patients according to the study protocol. […] Overall, 81.8% (305/373) of the patients were disease-free according to the probability of disease-free survival after 6 years as calculated using the Kaplan-Meier method, with 90% of the protocol group (196/218) being free of disease. […] After primary surgery and subsequent chemotherapy, 97.3% of the children with standard-type Wilms’ tumors of stage I were cured and 94% of those with stage II tumors survived disease-free, as did 88% of those in stage III, 46% of those in stage IV, and 75% of those in stage V (Kaplan-Meier method).
  • #72
    https://link.springer.com/article/10.1007/BF00183143
    Between June 1980 and June 1988, 373 patients with Wilms’ tumors were diagnosed and treated (34.5%, stage I; 34.5%, stage II; 14.8%, stage III; 10.7%, stage IV; and 5.4%, stage V). […] Aside from radical tumor nephrectomy, treatment encompassed chemotherapy in all cases and radiotherapy in half of the patients according to the study protocol. […] Overall, 81.8% (305/373) of the patients were disease-free according to the probability of disease-free survival after 6 years as calculated using the Kaplan-Meier method, with 90% of the protocol group (196/218) being free of disease. […] After primary surgery and subsequent chemotherapy, 97.3% of the children with standard-type Wilms’ tumors of stage I were cured and 94% of those with stage II tumors survived disease-free, as did 88% of those in stage III, 46% of those in stage IV, and 75% of those in stage V (Kaplan-Meier method).
  • #73 Wilms Tumor and Other Childhood Kidney Tumors Treatment (PDQ®) – NCI
    https://www.cancer.gov/types/kidney/hp/wilms-treatment-pdq
    The prognosis for patients with Wilms tumor depends on the histopathological features of the tumor, stage of disease at diagnosis, and molecular features of the tumor such as 1q gain and loss of heterozygosity of 1p and 16q. […] Treatment of adults with Wilms tumor includes surgery, chemotherapy, and radiation therapy. […] The current treatment approach is extrapolated from the experience of treating Ewing sarcoma at other sites with multimodal therapy consisting of multiagent chemotherapy, surgery, and/or radiation therapy for local control. […] There is no standard treatment option for anaplastic sarcoma of the kidney. […] The standard treatment options for stages I and II congenital mesoblastic nephroma include nephrectomy only and adjuvant chemotherapy for stage III cellular subtype.
  • #74 Diagnosis & Treatment | Wilms Foundation
    https://www.wilmsfoundation.org/diagnosisandtreatment
    Radiotherapy may also be given, depending on the stage of the tumor. This involves targeting the tumour with high-energy rays. […] After your treatment is complete your child will still need to be seen regularly at an outpatient clinic. […] Once your childs risk of relapse has reduced, the focus of on-going follow up changes to looking out for potential long term side effects of treatment. […] A personalized surveillance plan is usually created that outlines the specific long term follow up and on-going investigations that your child will require.
  • #75 Content – Health Encyclopedia – University of Rochester Medical Center
    https://www.urmc.rochester.edu/encyclopedia/content.aspx?contenttypeid=90&contentid=P02747
    Radiation therapy. These are high-energy X-rays or other types of radiation. Radiation is used to kill cancer cells or stop them from growing. It may be used to treat cancer that has spread or grows back. […] Supportive care. Cancer treatment can cause side effects. Medicines and other treatments can be used to help ease side effects, such as pain, fever, infection, and nausea and vomiting. […] Clinical trials. Ask your child’s healthcare provider if there are any new treatments being tested that may work well for your child. […] Your child will need follow-up care during and after treatment to: Check on your child’s response to the treatment, Manage the side effects of treatment, Look for returning or spreading cancer. […] Some treatments may be hard on your child, but they increase the chance of your child living a long time. Discuss the side effects of treatment with your child’s provider.
  • #76 Wilms Tumor: Treatment, Procedure, Cost, Recovery, Side Effects And More
    https://www.lybrate.com/topic/wilms-tumor
    Are there any side effects? As with other cancer treatments that involves chemotherapy and radiation therapy, there are side various side effects of these procedures. In chemotherapy which involves using cytotoxic medications to prevent cancer cells from dividing and growing, there are many side effects like hair loss, loss of appetite, low white blood cells, nausea and vomiting. This usually go way after chemotherapy is stopped. For radiation therapy where high-energy x-rays or particles are used to destroy cancer cells, side effects may include diarrhea, fatigue, nausea and skin irritation as areas hit by radiation may become red and sore. […] Long-term and late complication and side effects may include reduced kidney function, heart or lung problems, slowed or delayed growth and development, changes in sexual development and increased risk of cancer recurrence. These symptoms usually appear in teenage or adulthood years.
  • #77 Wilms Tumor Medication: Antineoplastic agents
    https://emedicine.medscape.com/article/989398-medication
    As previously stated, several cytotoxic agents may cause liver damage in patients treated for Wilms tumor. […] A study by Pritchard-Jones et al on 583 children from 251 hospitals in 26 countries aimed to assess whether doxorubicin can be omitted safely from chemotherapy for stage II-III, histologic intermediate-risk Wilms’ tumor in order to avoid doxorubicin-related cardiotoxicity effects. The study concluded that doxorubicin does not need to be included in treatment of stage II-III intermediate risk Wilms tumor when the histologic response to preoperative chemotherapy is incorporated into the risk stratification. […] These chemotherapeutic agents used to treat patients with Wilms tumor depend on the stage and histology of disease. Commonly used agents include dactinomycin, vincristine, doxorubicin, cyclophosphamide, etoposide, and carboplatin. The dosage depends on the particular stage of the disease and on the child.
  • #78 Wilms Tumor: Treatment, Procedure, Cost, Recovery, Side Effects And More
    https://www.lybrate.com/topic/wilms-tumor
    Are there any side effects? As with other cancer treatments that involves chemotherapy and radiation therapy, there are side various side effects of these procedures. In chemotherapy which involves using cytotoxic medications to prevent cancer cells from dividing and growing, there are many side effects like hair loss, loss of appetite, low white blood cells, nausea and vomiting. This usually go way after chemotherapy is stopped. For radiation therapy where high-energy x-rays or particles are used to destroy cancer cells, side effects may include diarrhea, fatigue, nausea and skin irritation as areas hit by radiation may become red and sore. […] Long-term and late complication and side effects may include reduced kidney function, heart or lung problems, slowed or delayed growth and development, changes in sexual development and increased risk of cancer recurrence. These symptoms usually appear in teenage or adulthood years.
  • #79 Radiation Therapy for Wilms Tumor | NYU Langone Health
    https://nyulangone.org/conditions/wilms-tumor-in-children/treatments/radiation-therapy-for-wilms-tumor
    At NYU Langone, our doctors sometimes recommend low-dose radiation therapy for children who have a Wilms tumor. This may be used after surgery or to treat cancer that has spread to other organs. Radiation uses energy beams that penetrate the skin, destroying cancer cells in the body. When it is directed at the area where the tumor was located, it can help reduce the risk of a recurrence. […] Radiation treatment typically occurs daily, five days a week, for up to seven weeks. […] The most common side effects of radiation therapy include mild, sunburn-like redness and burning of the skin, as well as diarrhea, fatigue, and nausea. Long-term side effects include reduced kidney function, delayed growth, and damage to the ovaries in girls, which can lead to problems with menstruation and fertility. […] Your childs NYU Langone pediatric oncologist and radiation oncologist work with nurses, social workers, and child psychologists to provide support to help your child with the physical and emotional side effects of radiation therapy.
  • #80 Wilms Tumor: Treatment, Procedure, Cost, Recovery, Side Effects And More
    https://www.lybrate.com/topic/wilms-tumor
    Are there any side effects? As with other cancer treatments that involves chemotherapy and radiation therapy, there are side various side effects of these procedures. In chemotherapy which involves using cytotoxic medications to prevent cancer cells from dividing and growing, there are many side effects like hair loss, loss of appetite, low white blood cells, nausea and vomiting. This usually go way after chemotherapy is stopped. For radiation therapy where high-energy x-rays or particles are used to destroy cancer cells, side effects may include diarrhea, fatigue, nausea and skin irritation as areas hit by radiation may become red and sore. […] Long-term and late complication and side effects may include reduced kidney function, heart or lung problems, slowed or delayed growth and development, changes in sexual development and increased risk of cancer recurrence. These symptoms usually appear in teenage or adulthood years.
  • #81 Diagnosis & Treatment | Wilms Foundation
    https://www.wilmsfoundation.org/diagnosisandtreatment
    Radiotherapy may also be given, depending on the stage of the tumor. This involves targeting the tumour with high-energy rays. […] After your treatment is complete your child will still need to be seen regularly at an outpatient clinic. […] Once your childs risk of relapse has reduced, the focus of on-going follow up changes to looking out for potential long term side effects of treatment. […] A personalized surveillance plan is usually created that outlines the specific long term follow up and on-going investigations that your child will require.
  • #82 Wilms Tumor | Mays Cancer Center
    https://cancer.uthscsa.edu/cancer-care/types-and-treatments/wilms-tumor
    Throughout treatment, child psychologists and child-life professionals are on hand to help meet your childs emotional needs. Our long-term survivorship care ensures we monitor your childs health, including possible side effects, long after treatment ends. […] Our pediatric oncologists participate in ongoing research to advance Wilms tumor care. Our renowned academic research efforts help expand your care options, including promising new treatments and cancer detection tests.
  • #83 Wilms Tumor Treatment | American Cancer Society
    https://www.cancer.org/cancer/types/wilms-tumor/treating.html
    If your child has been diagnosed with a Wilms tumor, the treatment team will discuss your options with you. Its important to weigh the benefits of each treatment option against the possible risks and side effects. […] Overall, about 9 of 10 children with Wilms tumors are cured. A great deal of progress has been made in treating this cancer in recent decades. Much of this progress is the result of children with Wilms tumors taking part in clinical trials of new treatments. […] Today, most children with this cancer are treated in a clinical trial to try to improve on what doctors believe is the best treatment. The goal of these studies is to find ways to cure as many children as possible, while limiting side effects by giving as little treatment as needed. […] Most children with Wilms tumors will get more than one type of treatment. The main types of treatment are: Surgery for Wilms Tumors, Chemotherapy for Wilms Tumors, Radiation Therapy for Wilms Tumors.
  • #84 Wilms’ tumour: biology, diagnosis and treatment – Szychot – Translational Pediatrics
    https://tp.amegroups.org/article/view/3228/html
    Wilms tumor is the commonest renal tumor of childhood affecting one in 10,000 children. […] Successful management of Wilms tumor necessitates meticulous attention to correct staging of the tumor and a collaborative effort between paediatric oncologists, specialist surgeons, radiologists, pathologists, and radiation oncologists. […] The usual treatment approach in most patients is a combination of surgery and chemotherapy, with the addition of radiotherapy in high risk patients. […] Substantial progress in the treatment of Wilms tumor over the past few decades has been made by refining risk stratification and by the use of existing chemotherapy schedules. […] The two largest collaborative groups that have studied the optimal management of Wilms tumor are the Childrens Oncology Group (COG) and the International Society of Paediatric Oncology (SIOP).
  • #85 The future of Wilms tumor therapies – Children’s National
    https://innovationdistrict.childrensnational.org/the-future-of-wilms-tumor-therapies/
    Dr. Domes mission is to come up with other therapies to treat Wilms tumor. […] Our mission is to come up with other therapies that work differently. […] Therefore, we must think about other forms of therapy for Wilms tumor, such as immunotherapy. […] I think the future of Wilms tumor therapy will be combining the classic chemotherapy and radiation therapy that weve used for many years but also adding another component for the patients with the highest risk of relapse, such as immunotherapy. […] Up until now, the studies that weve conducted using immunotherapy have introduced this treatment very late as a last resort after everything else has failed. […] I believe the prospects for Wilms tumor are also exciting.
  • #86 Wilms’ tumour: biology, diagnosis and treatment – Szychot – Translational Pediatrics
    https://tp.amegroups.org/article/view/3228/html
    Over the last few decades the treatment of Wilms tumor has undergone incremental improvement in survival rates despite a general trend to reduced therapy for the majority. […] The increased risk of late effects are directly associated with the aggressiveness of treatment for high stage Wilms tumor.
  • #87 Wilms Tumor (Kidney Tumor) | Children’s Hospital of Philadelphia
    https://www.chop.edu/conditions-diseases/wilms-tumor-kidney-tumor
    At CHOP, your child will be treated by a multidisciplinary team including surgeons, oncologists, urologists, radiologists, radiation oncologists, pathologists and nurse practitioners, who work together to create a treatment plan specific to your child. You and your child will meet with the treatment team to ensure your family has an opportunity to build a trusting relationship with them. […] Proton therapy, a more targeted form of radiation therapy, is offered at Children’s Hospital of Philadelphia as a treatment option for Wilms tumor. This therapy is provided in partnership with Penn Medicine at the Roberts Proton Therapy Center. […] The overall cure rate for Wilms tumor is 85 percent. As with any cancer, however, prognosis and long-term survival can vary greatly from child to child. Prompt medical attention and aggressive therapy are important for the best prognosis. Continuous follow-up care is essential for a child diagnosed with Wilms tumor because side effects of radiation and chemotherapy may occur as well as second malignancy.
  • #88 Wilms Tumor in Children – In Treatment | CureSearch
    https://curesearch.org/Wilms-Tumor-In-Treatment/
    A great deal of progress has been made in treating Wilms tumor over the last 25 years. Nine out of ten children are successfully treated with surgery, chemotherapy and sometimes radiation therapy. […] Wilms tumors are relatively rare, therefore it is important to seek care at an experienced children’s cancer center. A team approach that includes the child’s pediatrician as well as specialists at the children’s cancer center where the treatment will be provided is recommended. Once a Wilms tumor is discovered, children should begin treatment quickly. […] The first goal is to remove the tumor from the involved kidney or major site, even if the cancer has spread (metastasized) to other parts of the body. […] Surgery is the main treatment for Wilms tumor. The most common operation for Wilms tumor is called a radical nephrectomy.