Pediatric Osteosarcoma
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Osteosarcoma is the third most common cancer in adolescence, occurring less frequently than only lymphomas and brain tumors. It is thought to arise from a primitive mesenchymal bone-forming cell and is characterized by production of osteoid. The mainstay of therapy is removal of the lesion. Limb-sparing procedures can often be used to preserve function. Chemotherapy is also required to treat micrometastatic disease, which is present but not detectable in most patients at diagnosis.
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Osteosarcoma is a bone tumor that can occur in any bone. It most commonly occurs in the long bones of the extremities near metaphyseal growth plates. The most common sites include the femur (42%), with 75% of tumors in the distal femur; tibia (19%), with 80% of tumors in the proximal tibia; and humerus (10%), with 90% of tumors in the proximal humerus. [1] Other locations of note include the skull or jaw (8%) and pelvis (8%).
Any sarcoma that arises from bone is technically called an osteogenic sarcoma. Therefore, this term includes fibrosarcoma, chondrosarcoma, and osteosarcoma, all named for their morphologic characteristics. The focus of this article is osteosarcoma. Numerous variants of osteosarcoma are known and include conventional types (ie, osteoblastic, chondroblastic, fibroblastic types) and telangiectatic, multifocal, parosteal, and periosteal types.
United States
The incidence is 400 cases per year (4.8 cases per million persons < 20 y). [2]
The overall 5-year survival rate for patients whose condition was diagnosed between 1974 and 1994 was 63% (59% for male patients, 70% for female patients).
The incidence is slightly higher in African Americans than in Caucasians (data from the National Cancer Institute [NCI] Surveillance, Epidemiology, and End Results [SEER] Study Pediatric Monograph, 1975-1995). [1]
In African Americans, the annual incidence is 5.2 cases per million population younger than 20 years.
In Caucasians, the annual incidence is 4.6 cases per million population younger than 20 years.
The incidence is slightly higher in male individuals than in female individuals.
In male individuals, the incidence is 5.2 cases per million population per year.
In female individuals, the incidence is 4.5 cases per million population per year.
The incidence of osteosarcoma increases steadily with age; a relatively dramatic increase in adolescence corresponds with the growth spurt.
Osteosarcoma is rarely diagnosed in patients younger than 5 years (about 1% of cases). [3]
In children aged 5-9 years, the annual incidence is 2.6 cases for African Americans and 2.1 cases for Caucasians per million population.
In children aged 10-14 years, the annual incidence is 8.3 cases for African Americans and 7 cases for Caucasians per million population.
In adolescents aged 15-19 years, the annual incidence is 8.9 cases for African Americans and 8.2 cases for Caucasians per million population.
Patients whose disease is diagnosed during their growth spurt are taller than average, although patients identified in adulthood have average height.
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Timothy P Cripe, MD, PhD, FAAP Chief, Division of Hematology/Oncology/BMT, Gordon Teter Endowed Chair in Pediatric Cancer, Nationwide Children’s Hospital; Professor of Pediatrics, Ohio State University College of Medicine
Timothy P Cripe, MD, PhD, FAAP is a member of the following medical societies: American Academy of Pediatrics, American Association for the Advancement of Science, American Association for Cancer Research, American Pediatric Society, American Society of Gene and Cell Therapy, American Society of Pediatric Hematology/Oncology, Connective Tissue Oncology Society, Society for Pediatric Research, Children’s Oncology Group
Disclosure: Nothing to disclose.
Mary L Windle, PharmD Adjunct Associate Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference
Disclosure: Nothing to disclose.
Steven K Bergstrom, MD Department of Pediatrics, Division of Hematology-Oncology, Kaiser Permanente Medical Center of Oakland
Steven K Bergstrom, MD is a member of the following medical societies: Alpha Omega Alpha, Children’s Oncology Group, American Society of Clinical Oncology, International Society for Experimental Hematology, American Society of Hematology, American Society of Pediatric Hematology/Oncology
Disclosure: Nothing to disclose.
Max J Coppes, MD, PhD, MBA Executive Vice President, Chief Medical and Academic Officer, Renown Heath
Max J Coppes, MD, PhD, MBA is a member of the following medical societies: American College of Healthcare Executives, American Society of Pediatric Hematology/Oncology, Society for Pediatric Research
Disclosure: Nothing to disclose.
Samuel Gross, MD Professor Emeritus, Department of Pediatrics, University of Florida College of Medicine; Clinical Professor, Department of Pediatrics, University of North Carolina at Chapel Hill School of Medicine; Adjunct Professor, Department of Pediatrics, Duke University School of Medicine
Samuel Gross, MD is a member of the following medical societies: American Association for Cancer Research, American Society for Blood and Marrow Transplantation, American Society of Clinical Oncology, American Society of Hematology, and Society for Pediatric Research
Disclosure: Nothing to disclose.
Pediatric Osteosarcoma
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