Saving Life and Limb in Children With Bone, Muscle and Tissue Cancer at The Children's Hospital of Philadelphia

Apr 30, 2001, 01:00 ET from The Children's Hospital of Philadelphia

    PHILADELPHIA, April 30 /PRNewswire/ -- Pennsylvania's snowy mountains are
 crowded with careening snowboarders with nerves of steel and legs of iron.  At
 age 10, Mark "Bubba" Gernerd of Gwynedd Valley, Pennsylvania 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 (MRIs) 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 Orthopedic
 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 healing his rebuilt pelvis and reconstruction, Bubba 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 (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 kids 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 prothesis to replace the missing
 lower leg while retaining full knee mobility.
     To help kids make the best decision, doctors at 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 endoprothesis 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 Argly 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."
 
                                     FACTS
                              LIMB-SPARING SURGERY
 
     -- 8,000 children are diagnosed with pediatric cancer a year. Bone and
 soft-tissue sarcomas make up 11% 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
 8-12 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.
 
     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.
 
     CONTACT:  Cynthia Atwood of The Children's Hospital of Philadelphia,
 215-590-4092, or atwood@email.chop.edu.
 
                     MAKE YOUR OPINION COUNT -  Click Here
                http://tbutton.prnewswire.com/prn/11690X83484979
 
 

SOURCE The Children's Hospital of Philadelphia
    PHILADELPHIA, April 30 /PRNewswire/ -- Pennsylvania's snowy mountains are
 crowded with careening snowboarders with nerves of steel and legs of iron.  At
 age 10, Mark "Bubba" Gernerd of Gwynedd Valley, Pennsylvania 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 (MRIs) 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 Orthopedic
 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 healing his rebuilt pelvis and reconstruction, Bubba 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 (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 kids 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 prothesis to replace the missing
 lower leg while retaining full knee mobility.
     To help kids make the best decision, doctors at 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 endoprothesis 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 Argly 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."
 
                                     FACTS
                              LIMB-SPARING SURGERY
 
     -- 8,000 children are diagnosed with pediatric cancer a year. Bone and
 soft-tissue sarcomas make up 11% 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
 8-12 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.
 
     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.
 
     CONTACT:  Cynthia Atwood of The Children's Hospital of Philadelphia,
 215-590-4092, or atwood@email.chop.edu.
 
                     MAKE YOUR OPINION COUNT -  Click Here
                http://tbutton.prnewswire.com/prn/11690X83484979
 
 SOURCE  The Children's Hospital of Philadelphia