OSTEOSARCOMA

Definition:
• a malignant primary neoplasm of bone composed of a malignant
connective tissue stroma with evidence of malignant osteoid, bone or
cartilage formation.
• Osteosarcoma is a cancerous (malignant) bone tumor that usually
develops during the period of rapid growth that occurs in adolescence, as
a teenager matures into an adult.

A.K.A: Osteogenic Sarcoma

Incidence:
• More common in male adolescents
• Common occurs between ages 10 and 25 years old

Risk and Predisposing Factors:

• The cause is not known.
• In some cases, osteosarcoma runs in families, and at least one gene has
been linked to increased risk.
• This gene is also associated with familial retinoblastoma, a cancer of the
eye that occurs in children. Presence of certain rare, inherited cancers,
such as the following:
o Li-Fraumeni syndrome - a rare family predisposition to multiple
types of cancers (such as soft tissue sarcomas, breast cancer,
brain tumors, osteosarcoma, leukemia, melanoma, cancer of the
adrenal cortex, and others) caused by a mutation in a gene - the
p53 tumor-suppressor gene - that normally curbs cancer.
o retinoblastoma - a malignant tumor of the retina that usually occurs
in children younger than 4 years old.
• teenage growth spurts
• being tall for a specific age
• previous treatment with radiation for another cancer, especially at a young
age or with high doses of radiation
• presence of a benign (non-cancerous) bone disease

Manifestations:
• Localized pain at the site of the tumor
• Swelling, warmth, and redness caused by the vascularity of the
tumor
• Cough
• Dyspnea
• A child may limp or suffer a pathologic fracture
• Trauma may call attention to a pre-existing tumor
 • Bone fracture (may occur after what seems like a routine
movement)
• Bone pain
• Limitation of motion
• Pain when lifting (if the tumor is in the arm)
• Muscle atrophy

Stages of osteosarcoma

• Once osteosarcoma has been found, more tests may be done to find out if
cancer cells have spread to other parts of the body. This is called staging.
• At present, there is no staging system for osteosarcoma.
• Instead, most patients are grouped depending on whether cancer is found
in only one part of the body (localized disease) or whether the cancer has
spread from one part of the body to another (metastatic disease).
• The following groups are used for osteosarcoma:
1. Localized osteosarcoma
 The cancer cells have not spread beyond the bone or nearby
tissue in which the cancer began.
2. Metastatic osteosarcoma
 The cancer cells have spread from the bone in which the
cancer began to other parts of the body. The cancer most
often spreads to the lungs. It may also spread to other
bones.
 In multifocal osteosarcoma, tumors appear in 2 or more
bones, but have not spread to the lungs.
3. Recurrent
 Recurrent disease means that the cancer has come back
(recurred) after it has been treated. It may come back in the
tissues where it first started or it may come back in another
part of the body. Osteosarcoma most often recurs in the
lung. When osteosarcoma recurs, it is usually within 2 to 3
years after treatment is completed. Later recurrence is
possible, but rare.

Different types of osteosarcoma

There are many different types of osteosarcoma that are quite different in
natural history and their potential for lethal growth. The “grade” is a term
used to indicate the level of aggressive growth and potential for
metastasis, usually based upon the appearance of the tumor under
microscopic analysis.
1. Classical or conventional high grade central type
 which accounts for 90% of all osteosarcomas.
 By far the most common osteosarcoma.
Several other unusual high grade types:
 2. Telangiectatic
3. Small cell
4. Secondary (associated with another pre-existing disease such as
Paget’s disease or prior radiation exposure) type.
There are low grade types of osteosarcoma too.
5. Surface or juxtacortical low grade osteosarcoma
 also known by the names parosteal or periosteal
osteosarcoma
6. Low grade central osteosarcoma.
Pathophysiology:
A. Osteosarcoma occurs mainly in the metaphyses of long bones, sites of
active epiphyseal growth.
 Distal femur
 Proximal tibia
 Proximal humerus
B. As a tumor of mesenchymal cells, osteosarcoma demonstrates production
of osteoid cells.
C. It is a bulky tumorthat extends beyond the bone the bone into a soft tissue.
D. This may encircle the bone and destroy the trabeculae of affected area.
E. Osteosarcoma disseminates through bloodstream, usually to the lung.
F. Other sites of metastatic spread include other bones and visceral organs
Predisposing Factors

High grade mesenchymal tumor

Distal femur
Proximal tibia
Proximal humerus

Formation of osteoid

Bulky tumor that

destroys trabeculae of
diseased area

Metastasize through blood

streams
Diagnostic Studies:

Lungs, bones, visceral

organs
 • Bone Marrow aspiration
• Increased serum alkaline phosphatase
• Blood tests
• Bone scan to see if the cancer has spread to other bones
• CT scan of the chest to see if the cancer has spread to the lungs
• CT scan of the affected area
• Open biopsy (at time of surgery for diagnosis)
• X-ray of the affected area

Management:
Medical:
• Chemotherapy
High-dose methotrexate with leucovorin
citrovorum factor rescue
Doxorubicin (Adriamycin)
combinations of bleomycin, actinomycin, cyclophosphamide(Cytoxan),
ifosfamide(Ifex), and cisplatin are used Carboplatin (Paraplatin)

Surgical
• Amputation of affected extremity
• In most cases, surgery can remove the tumor while sparing the
affected limb (this is called limb-salvage surgery).
Nursing Diagnosis:
 Acute pain related to physical injuring agents
 Ineffective breathing pattern related to musculoskeletal impairment as
evidenced by usage of accessory muscles in breathing
 Activity intolerance related to pain
 Impaired social interaction related to limited physical mobility
 Ineffective role performance related to body image alteration; physical
illness
Nursing Responsibilities:
• Assist child and family to cope with the problem
• Provide time for child and family to accept the diagnosis
• Encourage family and client to verbalize
• Post-operatively, child is fitted with a prosthesis
• Provide nursing care of post-op amputation
• Provide with normal growth and development activities
Illustration:

This x-ray shows a malignant bone tumor (osteogenic sarcoma) of the knee. This
type of tumor is usually seen in adolescents (around 15 years old). This tumor
extends from the bone into the surrounding tissue.

An abnormal growth of cells within the bone that may be benign or malignant
(cancerous).

References:
http://www.nlm.nih.gov/medlineplus/
http://www.fpnotebook.com/Hemeonc/Ortho/Ostsrcm.htm
http://www.reininsarcoma.org/content/types-bone-sarcomas
Huether, Sue. Understanding Pathophysiology 2nd ed.
Black, Joyce, Medical-Surgical 7th ed.