Pediatric Osteosarcoma

Updated: Oct 21, 2015
  • Author: Timothy P Cripe, MD, PhD, FAAP; Chief Editor: Max J Coppes, MD, PhD, MBA  more...
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Overview

Background

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.

See the following image below.

Lateral plain radiograph of the knee reveals an os Lateral plain radiograph of the knee reveals an osteosarcoma of the distal femur. The lesion is mainly posterior, with disruption and elevation of the periosteum (Codman triangle), and extends beyond the bone into the soft tissue.
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Pathophysiology

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.

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Epidemiology

Frequency

United States

The incidence is 400 cases per year (4.8 cases per million persons < 20 y). [2]

Mortality/Morbidity

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).

Race

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.

Sex

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.

Age

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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