Osteosarcoma

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

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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

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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.