The Musculoskeletal Tumor Program at Children's Hospital Colorado
Care and Treatment of Childhood Bone and Soft Tissue Tumors
Shelley Dell'Orfano, RN, MSN, CPNP, ND, Musculoskeletal Tumor Program, Children's Hospital Colorado
Department of Orthopedics, University of Colorado Denver
Travis Heare, MD, Medical Director, Muscoluoskeletal Tumor Program, Children's Hospital Colorado
Associate Professor, Department of Orthopedics, University of Colorado Denver
The Musculoskeletal Tumor Program (MSTP) at Children's Hospital Colorado specializes in diagnosing and treating benign and malignant bone and soft tissue tumors. As a subspecialty in the Department of Orthopedics, the MSTP treats children and young adults from birth to 30 years of age. The MSTP also manages other oncology-related issues, including avascular necrosis, infection and trauma. It is especially known for its success in limb-sparing surgeries. The goal of the MSTP is to provide state-of-the-art, multidisciplinary care for children with a musculoskeletal tumor. Provided in a caring and compassionate environment, treatment focuses on function and a health-related quality of life.
Growing steadily since 2007, Children's Hospital Colorado is the only hospital in the Rocky Mountain region with a specialized team of professionals dedicated to the care and treatment of childhood bone and soft tissue tumors. Our multidisciplinary team consists of an orthopedic oncology surgeon, the patient's primary oncologist, radiation oncologist, prosthetist, physical therapist, social worker and psychologist if needed. As a member of the Children's Oncology Group - a national research program for childhood cancers - Children's Hospital Colorado actively participates in local and national research projects. Studies focused on quality of life issues for children with bone and soft tissue tumors are in development.
The multidisciplinary team consists of a surgeon with more than 20 years' experience specializing in orthopedic oncology, a pediatric nurse practitioner, nurse coordinator and an administrative assistant. A full-time nurse coordinator serves as a single point of contact for patients and their families providing support and education through diagnosis, treatment and recovery. The nurse coordinator facilitates communication within the team of providers and between specialists in the hospital as well as the primary care provider.
The MSTP diagnoses and manages benign bone and soft tissue tumors such as aneurysmal bone cysts, unicameral bone cysts, non-ossifying fibromas, osteochondromas, Langerhan's cell histiocytosis and osteoid osteomas. Often benign tumors are diagnosed on plain radiographs, although they may need other imaging such as MRI or CT. If diagnosis is uncertain, biopsy is indicated.
"In the initial presentation of a new tumor, patients and parents are frequently anxious and overwhelmed," said Travis Heare, MD, Medical Director of the Musculoskeletal Tumor Program at Children's Hospital Colorado. "Seeing patients, providing rapid answers and performing necessary radiographic studies in a timely fashion is important in the overall management of the problem."
It is important that a surgeon experienced with limb-sparing surgery perform the biopsy as location of the biopsy may exclude this option if the tumor is malignant. While many benign tumors are merely observed and do not require surgical intervention, some require resection or curettage and grafting. Indications for surgery include severe pain or a tumor that creates significant bone weakness increasing the risk of fracture.
X-ray of Ewing's sarcoma showing
the appearance of "hairs on end"
on the radius
Ewing's sarcoma and osteosarcoma are the most common malignant bone tumors seen at the MSTP, as they are also the most common primary malignant bone tumors in children. Synovial cell sarcoma and rhabdomyosarcoma are the most common malignant soft tissue tumors seen at the MSTP. Children's Hospital Colorado sees approximately 60 percent of Ewing's sarcoma cases and 33 percent of osteosarcoma cases in Colorado, and these percentages are increasing.
Malignant tumor treatment consists of a combination of chemotherapy, surgery for local control and possible radiation therapy.
Treatment has advanced significantly in recent decades, particularly chemotherapy. Although physicians have used chemotherapy to treat malignant musculoskeletal tumors for decades, pre-operative chemotherapy wasn't introduced until the 1980s. Today, pre-op and post-op chemotherapy are considered standard of care. Most treatment protocols use a combination of multiple drugs for approximately nine to 12 months. Patients receive about one-third of their chemotherapy before operation.
Surgical treatment for local control of the tumor occurs approximately three months after chemotherapy has started. The goal of surgery is to resect the entire tumor and some normal adjacent tissue to obtain the best margins while preserving as much function as possible. Many patients with extremity tumors can choose from one of three options: amputation, limb-salvage and rotationplasty. The MSTP team educates patients and families, both inpatient and outpatient, during pre-op treatment to help them make an informed decision. Whenever possible, a past surgical patient visits with them as well.
For treatment of cancerous tumors, the MSTP surgical team works closely with pediatric oncologists at Children's Center for Cancer and Blood Disorders, the department of orthopedic oncology at the University of Colorado Denver and an onsite pediatric radiation oncologist from the University of Colorado Denver. As a result, patients can receive their entire course of treatment at Children's Hospital Colorado.
Historically, amputation treated most bone sarcomas; however, the past decades have seen significant advances in limb-salvage operations, possible in approximately 90 percent of patients with extremity tumors. Research has demonstrated that limb-sparing surgery significantly improves limb function when compared with amputation, although many of these patients require additional surgical procedures over time to maintain a functional limb. Limb-sparing surgery has had no adverse effect on long-term survival rates when compared with amputations.
Rotationplasty offers another surgical option for patients with extremity sarcomas. These surgeries resect the tumor and reattach the ankle joint proximally with the foot - rotated 180 degrees - acting as the knee joint. Rotationplasty involves the distal femur, proximal tibia and the proximal femur on occasion. Essentially, this reconstructive technique functions as a below-knee amputation and results show good function. Patients with rotationplasty can participate in all activities while wearing their external prosthesis, including impact loading. The likelihood of them needing further surgery is low, as is failure of reconstruction. Because of the unusual cosmetic appearance, patients choose this option less frequently.
After patients have completed treatment, they visit the multidisciplinary clinic for evaluations at regularly scheduled intervals, typically every three months for the first two years. One of our goals for the multidisciplinary clinic is to help reduce the number of visits patients and their families need for follow-up.
For more information on the Musculoskeletal Tumor Program at Children's Hospital Colorado, please call (720) 777-6682.