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Wilms Tumor Surgery
Pediatric Surgery

Wilms Tumor Surgery

About This Department

 
Pediatric Surgery

Nine in ten children with Wilms tumor are cured. A careful operation keeps it that way.

In a Children's Oncology Group study of 116 children under two years old with a small stage I Wilms tumor, removal of the kidney with no chemotherapy afterward left every child alive four years later. Send the CT or MRI, the pathology report if surgery has already taken place, and any chemotherapy record, and our pediatric surgery team will tell you which operation fits your child and when.

100%
Alive at four years after kidney removal alone, 116 very low-risk children
94.9%
Alive at four years with tumors in both kidneys, 189 children
39%
Of those 189 kept working tissue in both kidneys
Free
Review of your child's scans and reports
Free consultation

Wilms tumor, or nephroblastoma, grows in the kidney of a young child, at its peak between three and four years of age. Parents find it most of the time themselves, as a firm swelling in the belly noticed at bath time, in a child who otherwise looks well.

Few childhood cancers respond as well.

Surgery and chemotherapy together cure close to nine children in ten, and the operation carries more weight in that result than its few hours suggest, because the way the surgeon removes the kidney, samples the lymph nodes and keeps the tumor intact sets the stage, and the stage decides how much chemotherapy and radiotherapy the child needs afterward.

What to send for a free review

The CT or MRI of the abdomen and chest as image files, with the radiologist's report. An ultrasound of the kidney veins if one was done.

If the kidney has already been removed, the operation note and the pathology report with stage and histology. If chemotherapy has started, the drugs, doses and dates.

Blood count, kidney function and urine test results. A pediatric surgeon and a pediatric oncologist read the file together, and the coordinator sends their written opinion back in your language.

Surgery first or chemotherapy first?

Two international schools treat Wilms tumor, and both cure the same share of children. North American centers following the Children's Oncology Group remove the kidney at diagnosis and stage the tumor from what the surgeon and pathologist find. European and many other centers follow the International Society of Pediatric Oncology (SIOP), whose current UMBRELLA protocol gives four weeks of chemotherapy first, six when the tumor has spread, and then operates on a tumor that has shrunk and firmed up. The SIOP order runs as follows.

  1. Ultrasound, CT or MRI of the abdomen and a chest CT. No biopsy in a typical case.
  2. Four weeks of vincristine and dactinomycin, weekly, as a day patient.
  3. Nephrectomy.
  4. Further chemotherapy, and radiotherapy for some, chosen by the stage and by how much living tumor the pathologist finds after the first four weeks.

Why the first approach avoids a needle biopsy
A needle passed into a Wilms tumor upstages it under the SIOP rules, because tumor cells can track along the needle path. A child aged six months to six years with a typical kidney mass on imaging starts chemotherapy without one. A biopsy comes into play when the picture looks unusual, in a baby under six months, in an older child, or when calcification or a very large lymph node suggests another tumor type.

What does a good Wilms tumor operation look like?

The surgeon opens the abdomen through a transverse cut above the navel, ties the kidney's artery and vein early, and lifts the kidney out with the tumor whole inside its capsule. The other kidney gets a look and a feel, the main vein is checked for tumor growing inside it, and lymph nodes along the aorta and vena cava go to the pathologist even when they look normal. Every one of these steps changes the stage.

Some tumors need more. Wilms tumor grows into the renal vein and up the inferior vena cava in a small share of children, sometimes as far as the heart, and the scan before surgery shows how far, because a thrombus that reaches above the liver needs a cardiac surgeon and heart-lung bypass in the same operation while one confined to the vena cava below the liver comes out through a cut in the vein wall after the surgeon controls the vessel above and below it. After SIOP chemotherapy a small tumor that sits away from the kidney's center, with no thrombus and no node involvement, qualifies in some European centers for laparoscopic removal through keyhole incisions, under strict criteria and with the same rules on spill and node sampling as an open operation. The surgeon who cannot meet those rules through keyhole cuts opens the abdomen, and parents hear in advance that this switch can happen during the operation.


This study has demonstrated that surgical rupture of the tumor must be prevented by the surgeon, because spills produce an increased risk of local relapse.
National Wilms Tumor Study 4, 2,482 children, Annals of Surgery, 1999

Removing only part of the kidney

Partial nephrectomy, removal of the tumor with a rim of healthy kidney, keeps working tissue for children at higher risk of kidney failure later. That covers tumors in both kidneys, a tumor in a single kidney, and syndromes that predispose to Wilms tumor, among them WAGR, Denys-Drash and Beckwith-Wiedemann syndrome. A child with one normal kidney on the other side and a large central tumor gains little from partial removal and runs a higher risk of leaving tumor behind, so whole-kidney removal stays the standard there.

When both kidneys are affected

Five to seven children in a hundred with Wilms tumor have it in both kidneys. The Children's Oncology Group AREN0534 study treated 189 of them with three drugs for six or twelve weeks, then surgery within twelve weeks of diagnosis, aiming to save as much kidney as possible. Four-year survival reached 94.9 percent, against far poorer results in earlier studies, and 39 percent kept part of both kidneys.

Surgical resection within 12 weeks of diagnosis and response and histology-based postoperative therapy improved EFS and OS and preservation of renal parenchyma compared with historical outcomes for children with BWT.
AREN0534, bilateral Wilms tumor, Annals of Surgery, 2017

From admission to discharge

The operation sets the length of the hospital stay.

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Hospital stay and the flight home by operation
Operation Nights in hospital Cleared to fly
Open radical nephrectomy Five to seven Ten to fourteen days after surgery
Laparoscopic nephrectomy, selected small tumors after chemotherapy Three to four Seven to ten days
Partial nephrectomy, one or both sides Six to ten Two to three weeks, after a urine leak is ruled out
Nephrectomy with removal of tumor from the vena cava Seven to twelve, with intensive care Three weeks or more
Two days before
Blood tests, blood group, a fresh scan if the last one is more than two weeks old, and a meeting with the anesthesia team.
The operation
Three to five hours under general anesthesia, with an epidural or a pain infusion placed at the start.

The first days after surgery

  1. Day one. The child sits up and drinks small sips.
  2. Days two and three. The bladder catheter comes out, food restarts, and the child walks in the corridor with a parent.
  3. Days four to seven. Pain medicine moves to syrup, the pathology report arrives with the final stage and histology, and the oncologist sets the chemotherapy plan from it.
Discharge
The wound gets checked in clinic three to five days after the child leaves the ward, and the stitches dissolve on their own.
Pain control in a small child
Children under five cannot always say where it hurts. Nurses score pain from the face, the cry and how the child moves, every few hours, and the epidural or infusion runs for two to three days before paracetamol and ibuprofen by mouth take over.

When chemotherapy follows

Chemotherapy after surgery starts within two to three weeks. Here it continues on the same site with the same pediatric oncology team, along with radiotherapy for the children whose stage calls for it, so the oncologist who planned the drugs before the operation reads the pathology report and gives the next dose without a referral, a new set of tests or a transfer of records between hospitals. Families who prefer home leave with a written handover.

Risks and how they are handled

The National Wilms Tumor Study Group reviewed 534 children from its fourth study and found surgical complications in 12.7 percent, down from 19.8 percent in the study before.

Each has a standard fix.

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Complications after nephrectomy in NWTS-4, 534 children
Complication How often What the team does
Bowel obstruction from adhesions 5.1 percent Rest for the bowel and a stomach tube, and an operation to free the adhesion when that fails
Heavy bleeding 1.9 percent Transfusion during the operation, with blood ready in theater
Wound infection 1.9 percent Antibiotics and dressings
Injury to a blood vessel 1.5 percent Repair at the time by the operating surgeon

What raises the risk

Three things in that review made complications likelier, a tumor growing into the vena cava or the heart, which raised the odds almost fourfold, a tumor of ten centimeters or more, and a cut in the flank instead of across the abdomen, which gives the surgeon a narrower view of the great vessels and no view at all of the other kidney. The surgeon's training mattered as well. Nephrectomy by a general surgeon carried nine times the risk of the same operation by a pediatric surgeon or pediatric urologist.

Tumor spill

Spill means the tumor's capsule breaking during the operation and cells escaping into the abdomen. Spill changes the stage in a single moment.

In NWTS-4 spill raised the risk of the cancer returning in the abdomen, and children whose cancer returned there had a two-year survival of 43 percent. Spill also moves a child to a higher stage and adds radiotherapy to the whole abdomen, with its own long-term effects on growth of the spine and on the bowel, which makes careful handling of the tumor, from the first cut to the moment the kidney leaves the body, the single most important thing the surgeon does in this operation.

Call the team the same day
Vomiting green fluid, a swollen hard belly, no stool or wind for a day, a temperature of 38 degrees or more, blood in the urine, or redness and discharge from the wound. Fever during chemotherapy needs antibiotics within the hour.
Bowel obstruction months later
Adhesions, bands of scar inside the abdomen, can block the bowel weeks or years after the operation. Colicky pain, vomiting and a swollen belly need a hospital visit the same day, wherever the child is, and the discharge letter tells the local team about the operation.

Life with one kidney

One kidney is enough
The remaining kidney grows to take on the work of two, and children play sport, grow and live a full adult life with it. Blood pressure and a urine protein test once a year protect it, and children with a predisposing syndrome or tumors in both kidneys need closer kidney follow-up.

Staying in Istanbul and going home

Your companion, your language, your food

A parent sleeps on the companion bed in the child's room every night of the admission. Hotel nights before and after, the airport pickup, the daily transport to the hospital and the visa invitation letter, sent ten days before the flight, come from the international patients office.

The coordinator writes and speaks with you in English, Arabic, French, Russian, Serbian, Romanian or Spanish, and books an interpreter for other languages. The hospital kitchen cooks halal, vegetarian and diabetic meals, and the hospital has a prayer room. A request for a female doctor, made in the first message, goes to the department and is met wherever the rota allows.

Follow-up after you return home

Once you are back home, the discharge letter in English, the pathology report and the scan images go to your local pediatric oncologist, and the coordinator stays on the same WhatsApp number.

The surgeon reviews later scans sent from home and replies in writing.

This table scrolls sideways on a narrow screen. Swipe or drag to see every column.

Scans after Wilms tumor treatment
Period Abdomen Chest
First two years Ultrasound every three months X-ray or CT every three months
Years three to five Ultrasound every six months X-ray every six months
After five years Yearly blood pressure and urine test No routine imaging
What moves the cost

The operation itself weighs most. Whole-kidney removal, partial removal of one or both kidneys and an operation that clears tumor from the vena cava differ in hours in theater, nights in intensive care and nights on the ward.

Chemotherapy given in Istanbul before or after the operation, radiotherapy, repeat scans and a longer hotel stay add to the figure. Published packages for pediatric kidney surgery in Turkey include the operation, anesthesia, the planned ward nights, the interpreter and transfers, and leave out flights, insurance and the treatment of a complication. A written estimate names the operation, the nights and the tests it assumes, and comes free after the team has read your child's scans.


Wilms tumor surgery FAQ

Can Wilms tumor be cured?
Yes. Surgery and chemotherapy cure close to nine children in ten, and some children under two with a small stage I tumor need surgery alone.
Will my child need chemotherapy before the operation?
Under the European SIOP approach, yes. Under the North American approach the kidney comes out first. Both cure the same share of children, and the team explains which suits your child's scan.
Can the kidney be saved?
Part of it, when tumors affect both kidneys, when the child has one kidney, or with a predisposing syndrome. Otherwise the whole kidney comes out.
How long will we stay in Istanbul?
Two to three weeks for a standard nephrectomy. Longer if chemotherapy or radiotherapy continue here.
Can I stay with my child?
Yes. A parent sleeps in the room every night on the companion bed.
What if something goes wrong after we fly home?
Your coordinator stays reachable on WhatsApp, the discharge letter tells your local doctors about the operation, and the surgeon here reviews scans and answers their questions.

References

  1. Fernandez CV, Perlman EJ, Mullen EA, et al. Clinical Outcome and Biological Predictors of Relapse After Nephrectomy Only for Very Low-risk Wilms Tumor: A Report From Children's Oncology Group AREN0532. Annals of Surgery. 2017;265(4):835-840.
  2. Ehrlich P, Chi YY, Chintagumpala MM, et al. Results of the First Prospective Multi-institutional Treatment Study in Children With Bilateral Wilms Tumor (AREN0534): A Report From the Children's Oncology Group. Annals of Surgery. 2017;266(3):470-478.
  3. Shamberger RC, Guthrie KA, Ritchey ML, et al. Surgery-related factors and local recurrence of Wilms tumor in National Wilms Tumor Study 4. Annals of Surgery. 1999;229(2):292-297.
  4. Ritchey ML, Shamberger RC, Haase G, Horwitz J, Bergemann T, Breslow NE. Surgical complications after primary nephrectomy for Wilms' tumor: report from the National Wilms' Tumor Study Group. Journal of the American College of Surgeons. 2001;192(1):63-68.
  5. van den Heuvel-Eibrink MM, Hol JA, Pritchard-Jones K, et al. Position paper: Rationale for the treatment of Wilms tumour in the UMBRELLA SIOP-RTSG 2016 protocol. Nature Reviews Urology. 2017;14(12):743-752.

Editor's note

Written by the Biruni Hospital medical editorial team. Reviewed by Assistant Professor Kamil Değer DEVECİOĞLU, Pediatric Surgery.

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