FOR IMMEDIATE RELEASEMay 2, 2001
Contact: Cynthia Atwood215-590-4092[email protected]
Saving Life and Limb in Children with Bone, Muscle and Tissue Cancer At The Children's Hospital of Philadelphia
Philadelphia, Pa.--Pennsylvania's snowy mountains are crowded with careening snowboarders with nerves of steel and legs of iron. At age 10, Mark "Bubba" Gernerd of Gynnedd Valley, Pa., was one of them until a sharp hip pain and subsequent x-ray led to a diagnosis of Ewing's sarcoma, one of the most insidious forms of malignant bone cancers.
If Bubba had received this diagnosis a decade earlier, he might have faced the same fate as Edward Kennedy Jr. whose bone cancer led to amputation of his lower leg at age 12, more than a quarter century ago. Thanks to recent technological advances, Bubba's surgical and medical teams at The Children's Hospital of Philadelphia were able to save both his life and his leg.
Limb-sparing procedures are a growing option for children with malignant tumors of the extremities because of several advances including magnetic resonance imaging (MRI) that improve the surgeon's ability to visualize and access tumors preoperatively. Removing only the tumor while sparing the limb is also easier with improved chemotherapy regimens. The drugs shrink tumors so more tissue, bone and muscle can be spared and better mobility can be restored.
In Bubba's case, a pelvic tumor was situated dangerously close to his hip joint, spinal cord, and bladder. It took 13 weeks of chemotherapy before his doctor, John P. Dormans, M.D., chief of the Hospital's Division of Orthopaedic Surgery, could remove the tumor. In a pioneering operation called an interval hemipelvectomy with "A-frame" free vascular fibula reconstruction, Dr. Dormans and his musculoskeletal team (composed of team members from orthopedic surgery and plastic surgery), removed Bubba's tumor as well as a surrounding cuff of normal pelvic tissue. Bubba's pelvis was rebuilt using the fibula bone from his leg--one of two bones in the lower leg--and the blood vessels attached to it.
After his rebuilt pelvis healed and Bubba underwent reconstructive surgery, he was able to resume activities. In fact, today, at age 17, he not only frequents the slopes but the swimming pool and golf links. A slight limp is all that hints of the tumor that was eating away his pelvis and threatening his life years ago.
Most Kids with Bone Cancer are Now Spared Life and Limb
Bubba is a shining example of how treatment technology has beaten limb- and life-threatening cancer in children. Today, 85 percent of the 880 kids diagnosed annually with bone cancer (osteosarcoma and Ewing's sarcoma) and soft tissue cancer are cured--and up to 90 percent without limb loss. "A little more than two decades ago, 85 percent of children and teens lost their lives. Limb removal was the standard treatment," Dr. Dormans says.
Studies have shown that there is no survival disadvantage for patients treated with limb-sparing surgery compared to those treated with amputation as long as surgery involves removing the entire tumor with wide margins (a cuff of normal tissue surrounding the entire tumor).
As tumor removal procedures evolve, there are more customized limb-sparing options. "Kids with bone cancer are like snowflakes," says Dr. Dormans. "Each tumor is different and requires a different approach."
There are 206 bones in the body that may involve cancer, and deciding which option is best depends upon the location of the tumor, the size of the tumor and whether it has spread to other areas of the body, such as other bones and the lungs.
Depending on these factors, once the cancerous bone or soft tissue tumor is removed, replacement options may involve one of the following:
--Allograft. A real human bone is obtained from a surgical patient or cadaver to graft onto the unaffected part of the bone.--Endoprothesis. A metal plastic bone and joint device is implanted inside the limb.--Allograft-prosthetic composites. Involves both bone grafts and implantation of an artificial device.--Vascularized autograft reconstruction. Involves rebuilding the damaged bone as well as the blood vessels attached to it.
In each case, soft tissue and muscle are transferred to cover and close the site and restore motor power. Chemotherapy often follows surgery. Physical therapy helps retrain muscles and nerves. While all this rehabilitation and healing is going on, the extremity appears normal with barely a trace of what occurred inside.
Limb-Sparing Options are Customized to Each Child
"Many kids opt to have an endoprothesis because it's invisible and function and movement are good, but limb-sparing is not always the best option for every child," says Dr. Dormans. For one thing, growth in young children--particularly the growth of the legs--presents a major challenge. While an expandable endoprothesis can be lengthened in small increments to allow for the growth of the child, the healthy leg may grow at a faster pace in very young children. This can result in a significant difference in leg lengths. One way to remedy this problem is to halt the growth plates in the healthy leg.
A child's activity level is another consideration. "Kids who wish to partake in high impact sports such as running or contact sports such as football learn that an endoprothesis cannot bear the brunt of these activities," says Dr. Dormans. For this reason, some children with difficult tumors in difficult sites choose other reconstructive options.
For very young children with cancer above the knee, one alternative is rotationplasty. This procedure removes the diseased bone and replaces the knee joint with the ankle joint. "By moving up the ankle and reattaching it to where the knee joint was, we can preserve more mobility," says Dr. Dormans. The patient is fitted with an artificial prosthesis to replace the missing lower leg while retaining full knee mobility.
To help children make the best decision, doctors at the Children's Hospital musculoskeletal tumor program encourage them to meet other young people who have had various surgical options including limb-sparing surgery.
In Hillary Hunter's case, she knew that choosing an endoprosthesis after surgery meant no more field hockey, the sport she adored up until last fall, when an x-ray revealed a tumor "the size of an orange" at the base of her femur. "I wanted to keep my leg," she says. While the physical therapy to strengthen the muscles and nerves in hr leg is arduous she says, "I've got great flexibility and the scar is barely there." Hillary, who is now 17 and a senior at Pen Argyl High School in Pennsylvania, looks forward to the non-impact activities, such as swimming and riding, that she enjoyed before surgery.
While Hillary is among the majority of youngsters with extremity sarcoma who are cured with limb-sparing surgery, "the war is not yet over," says Dr. Dormans. "The next frontier is prevention. Our team is not just focusing on removing tumors but in studying how they form in bone, tissue and muscle in the first place."
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FACTSLIMB-SPARING SURGERY
--8,000 children are diagnosed with pediatric cancer a year. Bone and soft-tissue sarcomas make up 11 percent of this total.
--Primary bone tumors are the sixth most frequent type of cancer in children. In adolescents and young adults, they are the third most frequent type of cancer. Pain is the most common presenting symptom.
--Osteosarcoma is the most common malignant bone tumor of childhood and is followed by Ewing's sarcoma. Both generally affect children between the ages of 10 and 20 years, but they may occur at any age.
--Rhabdomyosarcoma, a malignant tumor in the muscle, is the most common soft-tissue sarcoma of childhood, accounting for 5 percent of all childhood cancer. These lesions may be present anywhere in the body.
--Over the past five years, more than a thousand children with musculoskeletal tumors or tumor-like conditions have been treated at Children's Hospital. Of these, 50 have been diagnosed with primary musculoskeletal sarcomas or cancer of the musculoskeletal system (ranging from 8 to 12 patients per year). Approximately 80 percent of these children have been treated using limb-sparing techniques.
--Neither surgery nor chemotherapy alone is sufficient to successfully treat children with bone cancer. The combination of both together is required and is usually very successful.
References:Himelstein, Bruce B., M.D., and Dormans, John P., M.D., "Malignant Bone Tumors of Childhood," Common Orthopedic Problems, 1, Pediatric Clinics of North America, 1996, vol. 1; 43, number 4.
Rougraff BT, et al. "Limb Salvage Compared with Amputation for Osteosarcoma of the Distal End of the Femur: A long-term oncological, functional, and quality-of-life study," Journal of Bone Joint Surgery, 1994; 76A; 649-656.
Dormans, John P., M.D., "Limb-salvage Surgery vs. Amputation for Children with Extremity Sarcoma," Chapter 25, pp. 289-303, in The Child with a Limb Deficiency, Herring, JA & Birch, JG, editors, The American Academy of Orthopaedic Surgeons, 1998.
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