General Information
The National Cancer Institute (NCI) provides the PDQ pediatric cancer treatment information summaries as a public service to increase the availability of evidence-based cancer information to health professionals, patients, and the public.
Cancer in children and adolescents is rare. Children and adolescents with
cancer should be referred to medical centers that have a multidisciplinary team
of cancer specialists with experience treating the cancers that occur during
childhood and adolescence. This multidisciplinary team approach incorporates the skills
of the primary care physician, an orthopedic surgeon experienced in bone
tumors, a pathologist, radiation oncologists, pediatric oncologists,
rehabilitation specialists, pediatric nurse specialists, social workers, and
others to ensure that children receive treatment, supportive care, and
rehabilitation that will achieve optimal survival and quality of life.
(Refer to the PDQ Supportive Care summaries for specific information about supportive care for children and adolescents with cancer.)
Guidelines for pediatric cancer centers and their role in the treatment of
pediatric patients with cancer have been outlined by the American Academy of
Pediatrics.[1] At these pediatric cancer centers, clinical trials are
available for most types of cancer that occur in children and
adolescents, and the opportunity to participate in these trials is offered to
most patients/families. Clinical trials for children and adolescents with
cancer are generally designed to compare potentially better therapy with
therapy that is currently accepted as standard. Most of the progress
made in identifying curative therapies for childhood cancers has been achieved
through clinical trials. Information about ongoing clinical trials is
available from the NCI Web site.
In recent decades, dramatic improvements in survival have been achieved for children and adolescents with cancer. Childhood and adolescent cancer survivors require close follow-up because cancer therapy side effects may persist or develop months or years after treatment. (Refer to the PDQ Late Effects of Treatment for Childhood Cancer summary for specific information about the incidence, type, and monitoring of late effects in childhood and adolescent cancer survivors.)
Osteosarcoma is a bone tumor that occurs predominantly in adolescents and young
adults. It accounts for approximately 5% of childhood tumors. In
children and adolescents, more than 50% of these tumors arise from the bones around the
knee. There appears to be no difference in presenting symptoms, tumor location, and outcome for younger patients (<10 years) compared with adolescents.[2] Two trials conducted in the 1980s were designed to address the natural
history of surgically treated localized, resectable osteosarcoma of the
extremity. The outcome of patients in these trials who were treated with
surgical removal of the primary tumor recapitulated the historical
experience before 1970; more than half of these patients developed metastases
within 6 months of diagnosis, and overall, almost 90% [3] developed recurrent
disease within 2 years of diagnosis.[4] Overall survival for patients
treated with surgery alone was statistically inferior.[5] The natural history
of osteosarcoma has not changed over time, and fewer than 20% of patients with
localized resectable primary tumors treated with surgery alone can be expected
to survive free of relapse.[4,6-8]
References
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Guidelines for the pediatric cancer center and role of such centers in diagnosis and treatment. American Academy of Pediatrics Section Statement Section on Hematology/Oncology. Pediatrics 99 (1): 139-41, 1997.
[PUBMED Abstract]
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Bacci G, Longhi A, Bertoni F, et al.: Primary high-grade osteosarcoma: comparison between preadolescent and older patients. J Pediatr Hematol Oncol 27 (3): 129-34, 2005.
[PUBMED Abstract]
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Link MP, Goorin AM, Horowitz M, et al.: Adjuvant chemotherapy of high-grade osteosarcoma of the extremity. Updated results of the Multi-Institutional Osteosarcoma Study. Clin Orthop (270): 8-14, 1991.
[PUBMED Abstract]
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Link MP, Goorin AM, Miser AW, et al.: The effect of adjuvant chemotherapy on relapse-free survival in patients with osteosarcoma of the extremity. N Engl J Med 314 (25): 1600-6, 1986.
[PUBMED Abstract]
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Link MP: The multi-institutional osteosarcoma study: an update. Cancer Treat Res 62: 261-7, 1993.
[PUBMED Abstract]
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Eilber F, Giuliano A, Eckardt J, et al.: Adjuvant chemotherapy for osteosarcoma: a randomized prospective trial. J Clin Oncol 5 (1): 21-6, 1987.
[PUBMED Abstract]
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Harris MB, Gieser P, Goorin AM, et al.: Treatment of metastatic osteosarcoma at diagnosis: a Pediatric Oncology Group Study. J Clin Oncol 16 (11): 3641-8, 1998.
[PUBMED Abstract]
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Bacci G, Ferrari S, Longhi A, et al.: Nonmetastatic osteosarcoma of the extremity with pathologic fracture at presentation: local and systemic control by amputation or limb salvage after preoperative chemotherapy. Acta Orthop Scand 74 (4): 449-54, 2003.
[PUBMED Abstract]
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