Ewing sarcoma grows in bone, or in a minority of children in the soft tissue beside it, in children, teenagers and young adults, with a peak between ten and twenty. The pelvis, the thigh bone, the shin bone and the ribs carry most tumors. Pain that wakes the child at night and a swelling that does not go down bring families to the doctor, and in a third of cases a fever makes the picture look like an infection at first.
Every Ewing tumor carries a gene fusion, EWSR1-FLI1 in 85 percent of them, which confirms the diagnosis on the biopsy.
Surgery, radiotherapy or both?
Ewing sarcoma responds to radiotherapy, unlike most bone cancers, so the tumor board chooses between three ways of treating the original site, surgery alone, radiotherapy alone, or both. Five things decide it.
- Whether the surgeon can take the tumor out with a margin of healthy tissue all round.
- What the operation would cost in function, a joint, a nerve, the ability to walk.
- Location, since a tumor of the femur comes out far more easily than one deep in the sacrum or spine.
- Age and growth.
- How much the tumor shrank with chemotherapy.
The Children's Oncology Group compared the three routes in 465 children with localized Ewing sarcoma of bone treated on the same chemotherapy, and radiotherapy alone carried more than twice the risk of the tumor returning at its site, with no clear difference in overall survival once tumor location and size were taken into account.
Surgery wins on local control.
These data support surgical resection when appropriate, whereas radiotherapy remains a reasonable alternative in selected patients.
Children's Oncology Group, 465 patients, Cancer, 2015
When radiotherapy alone is the better choice
A tumor in the sacrum, the base of the skull or the spine, where removal would leave paralysis or loss of bladder and bowel control, receives radiotherapy alone. So does a tumor that has shrunk well but could only come out with a margin too close to be safe. Radiotherapy in a growing child slows growth of the treated bone and raises the risk of a second cancer years later, which tips the balance toward surgery wherever an operation keeps function.
Inside Ewing sarcoma surgery
Wide resection and the margin
The surgeon removes the tumor in one piece with a cuff of normal tissue around it, together with the biopsy track, and the pathologist inks every surface to show whether the margin is clear. A wide margin with a good response to chemotherapy needs no radiotherapy afterward. A close or positive margin brings radiotherapy to the tumor bed, and a tumor expected to have close margins can receive radiotherapy before the operation. The plan comes from the MRI taken after induction chemotherapy, which shows how far the tumor has pulled back from the joint, the nerves and the main vessels, and the surgeon marks the planned bone cuts on it a few centimeters beyond the original edge of the tumor, measured on the scan from before chemotherapy, because shrinkage on the scan does not mean the cells at the old edge have died. Navigation systems and custom cutting guides, printed from the child's own scans, help in the pelvis and around joints.
Patients with resectable tumors after initial chemotherapy had a low local failure rate.
CESS 81, CESS 86 and EICESS 92 trials, 1,058 patients, International Journal of Radiation Oncology Biology Physics, 2003
Local control figures from the CESS trials
The tumor returned at its original site in 7.5 percent after surgery with or without radiotherapy afterward, 5.3 percent when radiotherapy came before surgery, and 26.3 percent after radiotherapy alone, in a group that included the tumors hardest to reach.
Why the biopsy track comes out too
Tumor cells can seed along the path of the biopsy needle. The surgeon plans the incision so that the skin and muscle of that path come out with the tumor. The biopsy therefore belongs with the team that will later operate.
Once the bone section is out, the surgeon rebuilds the gap. The options for a limb, in the order the team weighs them, run as follows.
- A bone graft from a donor bank (allograft), or the child's own fibula with its blood supply, for shaft defects in younger children.
- A metal endoprosthesis replacing the joint, with an expandable version in growing children that lengthens without open surgery.
- Rotationplasty.
- Amputation, when no reconstruction would leave a working limb.
Tumors of the pelvis, ribs and spine
A quarter of Ewing tumors start in the pelvis.
These operations take six to ten hours, need a surgeon who knows the nerves and vessels of the pelvis well, and combine with radiotherapy in many cases because the margin around the hip socket or sacrum stays tight. Rib tumors come out with the neighboring ribs and part of the chest wall, rebuilt with a mesh, and the child breathes freely afterward.
Rebuilding the pelvis
When the hip socket is removed the surgeon can leave the gap to scar (a flail hip, which still lets the child walk with a limp), fuse the remaining bones, or fit a custom pelvic prosthesis. Each has its trade-off in walking, infection risk and later surgery, and the team sets these out for your child before the operation.
After the operation
The pathologist counts how many tumor cells survived chemotherapy, and that number changes the rest of treatment.
Pain control and physiotherapy
An epidural or nerve catheter controls pain for the first two to three days. Physiotherapists start in the first day or two, and a child with a knee or hip replacement stands with support within the first week. Walking aids stay for six to twelve weeks.
BuMel improved EFS and OS when given after vincristine, ifosfamide, doxorubicin, and etoposide induction in localized ES with predefined high-risk factors.
Euro-EWING 99 and Ewing-2008, 240 patients, Journal of Clinical Oncology, 2018
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What the pathology after surgery means
| Finding |
What it means |
What follows |
| Fewer than 10 percent living tumor cells |
Good response |
Standard consolidation chemotherapy |
| 10 percent or more living tumor cells |
Poor response |
Intensified treatment, which in European trials included high-dose busulfan and melphalan with stem cell rescue |
| Tumor at or close to the margin |
Risk of local return |
Radiotherapy to the tumor bed |
1
Days one to three
Intensive care or a high-dependency bed after pelvic surgery, a regular ward bed after limb surgery, drains and pain catheter in place.
2
Days four to ten
Drains out, standing and first steps, wound checks, and the pathology result with the oncologist.
3
Weeks two to three
Chemotherapy restarts once the wound has healed, here or at home with a written handover.
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Hospital stay and flying home by operation
| Operation |
Nights in hospital |
Cleared to fly |
| Limb resection with endoprosthesis |
Seven to ten |
Two to three weeks after surgery, with a clot prevention plan |
| Limb resection with bone graft |
Seven to ten |
Two to three weeks |
| Pelvic resection |
Ten to twenty |
Three to four weeks |
| Chest wall resection |
Five to seven |
Two weeks, after a chest X-ray |
Your companion, your language, your food
A parent sleeps in the room on the companion bed every night of the admission. The international patients office books the hotel nights either side, arranges the airport pickup and daily transport, and sends the visa invitation letter ten days before travel.
Your coordinator works in English, Arabic, French, Russian, Serbian, Romanian or Spanish and books interpreters for others. The kitchen cooks halal, vegetarian and diabetic meals, the hospital has a prayer room, and a request for a female doctor is met wherever the rota allows.
Call the team the same day
A temperature of 38 degrees or more during chemotherapy, redness, swelling or discharge at the wound, a calf that swells and hurts, sudden breathlessness, or new numbness or weakness in the operated limb. Fever after chemotherapy needs antibiotics within the hour.
What moves the cost
The reconstruction weighs most. An expandable endoprosthesis, a custom pelvic implant, a donor bone graft and a vascularized fibula differ widely, and a pelvic resection adds intensive care nights and a longer stay. Chemotherapy cycles given in Istanbul, radiotherapy, high-dose treatment and the physiotherapy program change the figure next. Published packages for pediatric bone tumor surgery in Turkey include the operation, anesthesia, the implant named in the plan, the ward nights, physiotherapy before discharge, the interpreter and transfers, and leave out flights, insurance and the treatment of complications. A written estimate, free after the team reads the scans, names the operation, the implant and the nights it assumes.
Chemotherapy before and after, in one place
Chemotherapy, radiotherapy and surgery all run at Biruni Hospital, so a child who starts induction here keeps the same pediatric oncology team through the operation and consolidation, without a referral or a transfer of records. The file never changes hands. Families who prefer home leave with a written handover after any phase.
Dose-intensive chemotherapy with vincristine, doxorubicin, cyclophosphamide, ifosfamide, and etoposide is more effective, less toxic, and shorter in duration for all stages of newly diagnosed Ewing sarcoma than vincristine, ifosfamide, doxorubicin, and etoposide induction.
EURO EWING 2012 trial, 640 patients, The Lancet, 2022
Follow-up after you return home
Once you are back home, the coordinator stays on the same WhatsApp number, and the surgeon reviews X-rays and MRI scans of the operated site sent from your local team.
Most relapses appear in the first two years. Chest CT and an X-ray or MRI of the tumor site follow every three months in the first two years, every six months up to year five, then once a year.
Long-term checks
Children treated with doxorubicin need heart scans, ifosfamide calls for kidney tests, and the drugs together affect fertility, so teenagers are offered sperm or tissue storage before treatment. An expandable prosthesis needs lengthening visits until growth stops. Follow-up lasts for life.
