Pediatric Surgery
Most children with a bone cancer today keep their arm or leg. Chemotherapy, then surgery that removes the tumor with a margin of healthy bone, then a reconstruction that can grow with the child.
Osteosarcoma and Ewing sarcoma are the two main bone cancers of childhood and adolescence, mainly around the knee, in the upper arm or in the pelvis. Treatment combines chemotherapy before and after surgery with an operation that removes the tumor completely. Limb-sparing surgery has replaced amputation for most children, without lowering survival, and expandable implants lengthen as the child grows. In the international EURAMOS-1 study of 2,260 patients with osteosarcoma, 71 percent were alive five years after diagnosis. Send us the scans, biopsy report and chemotherapy plan, and an orthopedic oncology surgeon working with pediatric oncologists will tell you what surgery and reconstruction would involve.
71%
Five-year survival in 2,260 patients with osteosarcoma, EURAMOS-1
73%
Five-year event-free survival in localized Ewing sarcoma, two-weekly chemotherapy
2 to 3 weeks
In Istanbul for the operation
Free
Orthopedic oncology review of your child's file
Free consultation
Most bone lumps in children are benign, such as osteochondromas, bone cysts and fibrous lesions, and many need only watching or a small operation. Two cancers matter most. Osteosarcoma grows in the fast-growing ends of long bones, near the knee in most children, during the adolescent growth spurt. Ewing sarcoma affects slightly younger children and appears in the pelvis, ribs and the shafts of long bones as well as the limbs. Both are rare, and both are best treated in centers that see them regularly.
How are bone tumors treated in children?
Osteosarcoma
Survival depends on spread and response.
EURAMOS-1 registered 2,260 patients aged 40 or younger from 17 countries, the largest osteosarcoma cohort ever followed. Three years after biopsy, 79 percent were alive, and five years after, 71 percent. Lung metastases at diagnosis more than doubled the risk of an event, and tumors in the pelvis or spine did worse than those in the limbs. For patients whose tumor was completely removed, a poor response to chemotherapy, meaning ten percent or more of the tumor still alive under the microscope, doubled the risk of relapse.
Further efforts are required to drive improvements for patients who can be identified to be at higher risk of adverse outcome.
EURAMOS-1 cohort of 2,260 patients, European Journal of Cancer, 2019
Signs that need a doctor
Bone pain that wakes a child at night, pain that lasts beyond a few weeks without a clear injury, a swelling over a bone, a limp, or a fracture after minor trauma need an X-ray. Growing pains do not cause swelling or persistent pain in one spot.
Chemotherapy around surgery
More drugs did not help poor responders. The randomized part of EURAMOS-1 gave 618 patients whose tumors had responded poorly either the standard three drugs after surgery or the same three plus ifosfamide and etoposide, and event-free survival was the same, with severe toxicity twice as common in the intensified group, 24 against 12 percent.
The results define standard of care for this population.
EURAMOS-1 randomized trial, Lancet Oncology, 2016
- Ten weeks of chemotherapy with methotrexate, doxorubicin and cisplatin, known as MAP, before surgery.
- Surgery, with the removed tumor examined to see how much chemotherapy killed.
- Around eighteen more weeks of MAP after surgery.
- Regular checks of hearing, kidneys and the heart during and after treatment.
Ewing sarcoma
Ewing sarcoma responds to timing.
In a Children's Oncology Group trial, 587 patients with localized Ewing sarcoma were randomized to the same chemotherapy given every three weeks or every two, with growth-factor injections between cycles. Event-free survival five years later was 73 percent with the two-weekly schedule against 65 percent. Side effects were similar. The faster schedule became the North American standard.
For localized Ewing sarcoma, chemotherapy administered every 2 weeks is more effective than chemotherapy administered every 3 weeks, with no increase in toxicity.
Children's Oncology Group trial of 587 patients, Journal of Clinical Oncology, 2012
- Surgery alone, for removable tumors.
- Radiotherapy alone, for tumors in places where surgery would cause major loss of function, such as parts of the spine or pelvis, accepting a slightly higher risk of local recurrence in exchange for keeping the child walking and continent.
- Surgery followed by radiotherapy, when margins are close or the response to chemotherapy is poor.
Biopsy where the surgery will be done
The biopsy needle or cut must be placed so that the surgeon can remove its track with the tumor later. A poorly placed biopsy can contaminate healthy tissue and make limb-sparing surgery harder. Ideally, the team that will operate also performs or plans the biopsy.
Limb salvage
The use of a limb-salvage procedure for osteosarcoma of the distal end of the femur did not shorten the disease-free interval or compromise long-term survival.
Multi-institutional study of 227 patients, Journal of Bone and Joint Surgery, 1986
Reconstruction in growing children
Three paths.
1
Expandable endoprosthesis
A metal implant replaces the removed bone and joint and can be lengthened as the child grows, by a small operation or, with newer designs, by a magnet outside the leg without surgery.
2
Biological reconstruction
Bone from a donor bank or from the child's own fibula fills the gap, and in time can join and remodel like living bone.
3
Rotationplasty or amputation
In rotationplasty, the lower leg is turned and attached to the thigh so the ankle works as a knee in a prosthesis, an option that lets very young children run and play sport.
What the Florida series showed
A Tampa center reviewed 38 children. All had lower-limb reconstructions with an expandable endoprosthesis, and among the 26 survivors function scores were good, the hip and knee moved well and scores for emotional acceptance and happiness were high, which matters as much to a teenager as the range of movement. The implants were lengthened by 4.5 centimeters on average by skeletal maturity, leaving a leg-length difference of only 0.7 centimeters.
The price was a high complication rate. Forty-two percent had complications, ten needed the implant revised and two of those eventually needed an amputation. The authors concluded that no single reconstruction is best for every child, and that amputation and rotationplasty remain reasonable choices when families accept them.
Current technology does not offer a single best reconstruction option for children.
Series of 38 expandable endoprostheses, Journal of Bone and Joint Surgery, 2012
How families choose
Age, the bone involved, the expected remaining growth, the child's activities and the family's wishes all shape the decision. A ten-year-old with a tumor at the knee and years of growth ahead faces a different choice from a fifteen-year-old who has nearly finished growing. The surgeon explains each option with its function, durability and expected further operations, and families meet a physiotherapist and, where helpful, a child who has had the same reconstruction.
The operation and recovery
Surgery and the weeks after
Surgeons remove the tumor in one piece with a cuff of normal tissue, guided by MRI taken after chemotherapy, then reconstruct the bone and joint. It takes four to eight hours. Children stay in hospital for one to two weeks, start physiotherapy on the first days, and walk with crutches before leaving. Chemotherapy resumes two to three weeks after surgery once the wound has healed.
Infection, wound problems, loosening or breakage of the implant and nerve or vessel injury are the main risks. Infection is the most serious. Chemotherapy weakens defenses, and an infected implant sometimes has to come out. Long-term follow-up continues to adulthood, both for cancer and for the reconstruction, which will need further surgery as the child grows.
What should we send, and how long is the stay?
Start with the scans and the biopsy.
- MRI of the whole bone involved, and a chest CT to look for lung spread, taken at diagnosis and again after chemotherapy if available.
- The biopsy report.
- The chemotherapy plan and how many cycles have been given.
Plan two to three weeks in Istanbul for the operation. The team coordinates chemotherapy with your home oncologists, so that much of it can continue closer to home, and the family returns for reviews and lengthening procedures. Children fly home once the wound has healed and they can manage crutches safely, with a letter for the airline about the implant.
Cost depends on the type of reconstruction, the implant, the operation, the hospital stay and physiotherapy. Your own figure follows the free review of your file.
One coordinator plans the stay with the family, from the visa invitation letter sent ten days ahead to the transfer and an apartment near the hospital with step-free access. A parent stays with the child throughout, physiotherapists teach the family the exercises before discharge, and the team works in seven languages with halal meals and a prayer room available. Back home, the surgeon reviews X-rays and photographs sent by your local doctors and stays reachable on the same WhatsApp number, and for children with an expandable implant the team plans each lengthening around the growth measured on those X-rays, so that the two legs stay close in length through the growth spurt and the family knows months in advance when the next short visit to Istanbul will be needed and what it will involve.
What are the results?
Side by side, the main studies show the progress of current treatment and its limits.
This table scrolls sideways on a narrow screen. Swipe or drag to see every column.
Results from the main studies
| Study |
Patients |
Finding |
| EURAMOS-1 cohort, osteosarcoma |
2,260 |
Overall survival 79 percent at 3 years, 71 percent at 5 years |
| EURAMOS-1 randomized trial, poor responders |
618 |
Adding ifosfamide and etoposide after surgery did not improve event-free survival and added toxicity |
| Children's Oncology Group, localized Ewing sarcoma |
568 eligible |
Event-free survival at 5 years 73 percent with two-weekly chemotherapy, 65 percent with three-weekly |
| Limb salvage against amputation, distal femur |
227 |
No difference in disease-free or overall survival |
| Expandable endoprosthesis |
38 |
Good function, complications in 42 percent |
Pediatric bone tumor surgery FAQ
Can my child keep the leg or arm?
In most children, yes. Limb-sparing surgery did not lower survival compared with amputation, and implants that lengthen with growth keep the limb working.
What is the survival rate for osteosarcoma?
In the EURAMOS-1 cohort of 2,260 patients, 71 percent were alive five years after diagnosis. Survival is lower when the cancer has spread at diagnosis.
How does an expandable implant work?
It contains a mechanism that lengthens the implant as the child grows, adjusted by a small operation or, in newer designs, by a magnet outside the leg.
What is rotationplasty?
An operation that turns the lower leg so the ankle works as a knee inside a prosthesis. It gives very active children good function and avoids repeated implant surgery.
How long will we stay in Istanbul?
Two to three weeks for the operation, with chemotherapy coordinated with your home team.
Can a parent stay with the child?
Yes. A parent stays with the child throughout the hospital stay.
References
- Smeland S, Bielack SS, Whelan J, et al. Survival and prognosis with osteosarcoma: outcomes in more than 2000 patients in the EURAMOS-1 (European and American Osteosarcoma Study) cohort. European Journal of Cancer. 2019;109:36-50.
- Marina NM, Smeland S, Bielack SS, et al. Comparison of MAPIE versus MAP in patients with a poor response to preoperative chemotherapy for newly diagnosed high-grade osteosarcoma (EURAMOS-1): an open-label, international, randomised controlled trial. Lancet Oncology. 2016;17(10):1396-1408.
- Womer RB, West DC, Krailo MD, et al. Randomized controlled trial of interval-compressed chemotherapy for the treatment of localized Ewing sarcoma: a report from the Children's Oncology Group. Journal of Clinical Oncology. 2012;30(33):4148-4154.
- Simon MA, Aschliman MA, Thomas N, Mankin HJ. Limb-salvage treatment versus amputation for osteosarcoma of the distal end of the femur. Journal of Bone and Joint Surgery, American Volume. 1986;68(9):1331-1337.
- Henderson ER, Pepper AM, Marulanda G, et al. Outcome of lower-limb preservation with an expandable endoprosthesis after bone tumor resection in children. Journal of Bone and Joint Surgery, American Volume. 2012;94(6):537-547.
