Osteosarcoma
Background
Name the 2 most common types of malignant bone tumors in the pediatric population.
The 2 most common types of malignant bone tumors in the pediatric population are osteosarcoma and Ewing sarcoma.
Osteosarcoma is associated with what other pediatric tumor?
Pts with retinoblastoma have an increased risk of osteosarcoma, both within and outside the irradiated tissue.
Describe the distribution of osteosarcoma cases as a function of population age.
Osteosarcoma has a bimodal distribution as a function of age, with cases arising during the teenage years as well as cases associated with other conditions (Paget Dz, fibrous dysplasia) that arise in an older (age >65 yrs) population.
What is the incidence of osteosarcoma in the U.S. population?
400 cases/yr of osteosarcoma are diagnosed in the U.S. population, and most occur during the teenage years. Osteosarcoma is ~2 times more common than Ewing sarcoma.
What are the most common risk factors associated with the development of osteosarcoma?
High rate of bone production and turnover (as in puberty) are associated with the development of −osteosarcoma.
Describe sex and ethnic factors associated with osteosarcoma.
Osteosarcoma is more common in boys (> girls) and in blacks (> whites).
What is another name for the shaft of the bone? End of the bone?
The diaphysis is the shaft of the bone. The epiphysis is the end of the bone, and the growth plate is located in this region. The conical area of bone between the diaphysis and epiphysis is the metaphysis.
Osteosarcoma is most likely to develop in what part of the bone?
Osteosarcoma arises most frequently in the appendicular skeleton (80% of cases) at the metaphyseal portions of the femur, tibia, and humerus.
Osteosarcoma most commonly arises in which bone?
The femur is the bone in which osteosarcoma most commonly arises.
Describe the histologic defining feature of osteosarcoma.
Production of osteoid bone is the defining feature of osteosarcoma.
Describe 2 genetic syndromes associated with osteosarcoma.
Osteosarcoma is associated with Li-Fraumeni syndrome as well as retinoblastoma.
What is the difference between conventional osteosarcoma and juxtacortical osteosarcoma?
Conventional (sometimes called classic) osteosarcoma refers to the most common (75% of all cases) variant of osteosarcoma, which typically presents within areas of rapidly proliferating skeletal bone. Juxtacortical osteosarcoma refers to a set of more rare osteosarcoma variants that arise adjacent to the outer surface of −cortical bone.