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INTRODUCTION

Osteosarcoma is a type of cancer that produces immature bone. It is

the most common type of cancer that arises in bones, and it is

usually found at the end of long bones, often around the knee. Most

people diagnosed with osteosarcoma are under the age of 25, and it

is thought to occur more often in males than females.

Osteosarcoma is the eighth leading cancer in children under age

15. Osteosarcoma affects 400 children under age 20 and 500 adults

(most between the ages of 15-30) every year. Approximately 300 of

the 900 die each year.

Osteosarcoma tends to occur in the bones of the:

Shin (near the knee)

Thigh (near the knee)

Upper arm (near the shoulder)

Osteosarcomas range from low grade tumors that only require

surgery to high grade tumors that require an aggressive treatment

regimen. Patients with osteosarcoma are best treated at a cancer

center where an expert sarcoma team and resources are available to

provide specialized and responsive care.

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PROGNOSIS

Prognosis is separated into three groups.

Stage I osteosarcoma is rare and includes parosteal osteosarcoma

or low-grade central osteosarcoma. It has an excellent prognosis

(>90%) with wide resection.

Stage II prognosis depends on the site of the tumor (proximal

tibia, femur, pelvis, etc.), size of the tumor mass (in cm.), and the

degree of necrosis from neoadjuvant chemotherapy

(chemotherapy prior to surgery). Other pathological factors such

as the degree of p-glycoprotein, whether the tumor is Her2-

positive are also important, as these are associated with distant

metastases to the lung.

The prognosis for patients with metastatic osteosarcoma

improves with longer times to metastases, (more than 12 months-

24 months), a smaller number of metastases, and their

resectability. It is better to have fewer metastases than longer

time to metastases. Those with a longer length of

time(>24months) and few nodules (two or fewer) have the best

prognosis with a 2-year survival after the metastases of 50%, 5-

year of 40% and 10 year of 20%. If metastases are both local and

regional, the prognosis is worse.

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Initial presentation of stage III osteosarcoma with lung

metastases depends on the resectability of the primary tumor and

lung nodules, degree of necrosis of the primary tumor, and maybe

the number of metastases. Overall survival prognosis is about

30%.

CAUSES OF OSTEOSARCOMA

The causes of osteosarcoma are not known. Several research groups

are investigating cancer stem cells and their potential to cause

tumors. Radiotherapy for unrelated conditions may be a rare cause.

Osteosarcoma runs in families. At least one gene has been linked to

an increased risk. This gene is also associated with

familial retinoblastoma. This is a cancer of the eye that occurs in

children.

It is apparent that two suppressor genes, p53 and Rb, have major

roles in tumor genesis in osteosarcoma. Approximately 3-4

percent of children with osteosarcoma carry constitutional germline

mutations in p53.

The majority of these cases with germline p53 mutations occur in

patients with a strong family history of cancer or with family

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histories suggestive of the Li-Fraumeni syndrome (a familial cancer

syndrome) or in patients with multiple cancers.

By far the strongest genetic predisposition to osteosarcoma is found

in patients with hereditary retinoblastoma. In hereditary

retinoblastoma, germline mutations of the Rb gene are common.

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SYMPTOMS Many patients first complain of pain that may be worse at night, and

may have been occurring for some time. If the tumor is large, it can

appear as a swelling. The affected bone is not as strong as normal

bones and may fracture with minor trauma (a pathological

fracture).The pain may come and go and vary in intensity. The

swelling will not be visible if it is not near the surface of the body

such as on the pelvis.

Bone fracture (may occur after a routine movement)

Bone pain

Limitation of motion

Limping (if the tumor is in the leg)

Pain when lifting (if the tumor is in the arm)

Tenderness, swelling, or redness at the site of the tumor

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DIAGNOSIS Bone x-ray: Doctors can often recognize or at least suspect

osteosarcoma on plain x-rays of the bone. But other imaging tests

may be needed as well.

Magnetic resonance imaging (MRI) scan

MRI scans can usually tell if the mass is likely to be a tumor, an

infection, or some type of bone damage from other causes. MRI

scans can also help show the exact extent of a tumor, as they provide

a detailed view of the marrow inside bones and the soft tissues

around the tumor.

Computed tomography (CT) scan

CT scans are sometimes used to look at the affected bone to see if

the tumor has grown into nearby muscle, fat, or tendons. A CT scan

of the chest is often done to look for spread of the cancer to the

lungs.

Chest x-ray

This test is sometimes done to see if cancer has spread to the lungs.

It can find larger tumors, but it is not as good as a CT scan for

spotting smaller tumors. If a CT scan of the chest is done, a chest x-

ray may not be needed.

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Bone scan

Areas of active bone changes appear as “hot spots” on the skeleton

because they attract the radioactivity. Hot spots may suggest areas

of cancer, but other bone diseases can also cause the same pattern.

Positron emission tomography (PET) scan

PET scans can help show the spread of osteosarcomas to the lungs,

other bones, or other parts of the body, and can also help in

following the response to treatment.

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CASE STUDY Name of the patient: Sahil Panchal

Age: 15

Sex: Male

Name of the hospital: Shalby hospital

Name of the doctor: Dr Parag Talekar

Symptoms: Bone fracture, limitation of motion,

tenderness, swelling, redness at the site of tumor.

Diagnosis: PET Scan

PET scans show the spread of osteosarcoma in the legs

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An MRI of the entire bone where the primary tumor is located. This test

can rule out "skip metastases" (spread of the tumor to other areas of

the bone).

CT scan shows a fractured bone and masses of tumor

including cancer.

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Treatment: Treatment usually starts after a biopsy of the tumor is

done.

Before major surgery to remove the tumor, chemotherapy was

given. This shrunk the tumor and made surgery easier. It also killed

cancer cells that have spread to other parts of the body.

Chemotherapy medicines include:

Carboplatin (Paraplatin)

Cisplatin

Cyclophosphamide (Cytoxan)

Doxorubicin (Adriamycin)

Epirubicin

Etoposide

Ifosfamide (Ifex)

Methotrexate (high dose) with leucovorin

Surgery is used after chemotherapy to remove any remaining tumor,

surgery can done to remove the tumor while saving the affected

limb. This is called limb-salvage surgery..

Follow-up after treatment: It includes appointments every three

months for the first two years, every four months for the third year,

every six months for the fourth and fifth year, and annually

thereafter. Most appointments include a physical exam, imagining of

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the original tumor’s location and imaging of the lungs. The patient

who have been treated with chemotherapy, tests are also done

periodically to monitor the heart, liver and kidneys; to test for

hearing loss and to check hormone levels, bone density and

cholesterol. If recurrence is detected at follow-up, further

chemotherapy and/or surgery is usually recommended.

Complications:

Spread of cancer to the lungs

Side effects of chemotherapy

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CONCLUSION Osteosarcoma is a rare primary malignant tumor arising in bone, the

tumor cells producing immature bone, or osteoid

Patients who have skeletal lesions that are at risk for fracture should be

protected to avoid fracture. The type of protection required will vary

depending on the site of involvement, but care should be taken to avoid

weight bearing for individuals with worrisome lesions in the lower

extremity or pelvis.

Any child or adolescent presenting with pain, and especially swelling, in

or around any bone needs urgent and thorough assessment.

Biopsy is best undertaken by a specialist who will eventually perform

the definitive surgical resection.

After a patient is diagnosed via biopsy with osteosarcoma, the

treatment typically involves neoadjuvant chemotherapy, surgical

resection and postoperative chemotherapy.

The most common area of metastatic disease is the lungs and they

should always be carefully evaluated for disease.

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BIBLIOGRAPHY

The necessary information for the project is collected from the

references stated below:

Orthopedic surgeon Dr. Parag Talekar

Osteosarcoma-Wikipedia

en.wikipedia.org/wiki/Osteosarcoma

Osteosarcoma in children-Cancer information Macmillian Cancer

Support

http://www.macmillan.org.uk

School of medicine at the university of Virginia

http://www.medicine.virginia.edu/

Osteosarcoma-St. Jude children research hospital

http://www.stjude.org