Volunteer/Request Ambassador

Thank you! Your submission has been received!

Oops! Something went wrong while submitting the form.

About Osteosarcoma

What is Osteosarcoma?

Osteosarcoma is a rare and aggressive form of bone cancer that primarily affects children, adolescents, and young adults. There are an estimated 1,000 new cases of osteosarcoma diagnosed each year in the United States. Of those new cases, half are children. It accounts for approximately 2% of all childhood cancers and is the most common primary bone tumor in children.¹ The worldwide incidence rate is 3.4 cases per million people per year.²

Osteosarcoma can occur at any age but the most common age for diagnosis is in children and young adults during growth spurt times. More than 75% of the cases are people less than 25 years old, with the average age of diagnosis of osteosarcoma being 15.³

Osteosarcoma often occurs in long bones near joints, such as the femur, tibia, or humerus. Less often, osteosarcoma occurs in other bones like the pelvis, shoulder, and jaw. It is common to be misdiagnosed as a benign tumor or dismissed as growing pains.

As the treatment protocol for osteosarcoma has not changed since 1982, the prognosis for osteosarcoma patients has remained largely unchanged over the past four decades. The five-year survival rate for patients with localized osteosarcoma is estimated to be 59%.⁴ For those with distant metastatic disease or recurrent tumors, survival drops significantly to 24%.⁴ It is important to note that patients may survive beyond the five-year mark, but ultimately succumb to this devastating disease, resulting in a lower overall survival rate.

Current treatment approaches, which include aggressive chemotherapy, surgery, and radiation therapy, are often accompanied by debilitating side effects and long-term complications, such as limb amputation or impaired growth. There is an urgent need for new treatments to improve the low survival rates and improve the quality of life for survivors. The current chemotherapy treatments are highly toxic and can leave lasting health issues for those who endure them. 87% of osteosarcoma survivors experience at least one chronic medical condition and over 50% experience two or more conditions.⁵

Ongoing research is urgently needed to improve outcomes, especially for patients with resistant or metastatic disease. An estimated 15-20% of patients have measurable metastatic disease at diagnosis.⁶ Recent advances in understanding the molecular mechanisms and metabolic pathways of osteosarcoma offer hope for novel therapeutic interventions. However, funding gaps remain a critical barrier to translating these discoveries into effective treatments.

‍

¹American Cancer Society

²Amirhossein Misaghi, Amanda Goldin¹, Moayd Awad and Anna Kulidjian, Musculoskeletal tumors: Current approaches and controversies, SICOT-J, Volume 4, 2018

³Cleveland Clinic

⁴National Cancer Institute Surveillance, Epidemiology, and End Results (SEER) Program

⁵Rajaram Nagarajan MD, MS et al, Twenty years of follow-up of survivors of childhood osteosarcoma, Cancer 2011, Volume 117, Issue 3

⁶St. Jude Children’s Research Hospital

‍

Osteosarcoma Facts

24%

the survival rate when Osteosarcoma has spread to the lungs (it often does)

Side Effects

Heart or lung problems, Loss of hearing, Slowed growth and development, Changes in sexual development and ability to have children, Learning problems in younger children, Development of another cancer

87%

percentage of Osteosarcoma survivors who experience at least 1 chronic medical condition

42+

number of years since Osteosarcoma has had a new drug

the amount of the NIH budget spent on ALL childhood cancer research.

1,000

cases diagnosed annually

How is Osteosarcoma diagnosed?

Types of tests used for osteosarcoma.

Osteosarcoma is diagnosed with a biopsy. Often osteosarcoma may be suspected from an x-ray or MRI but the only way to definitively diagnose osteosarcoma is for a pathologist to examine the tumor cells under a microscope.

Biopsy

A biopsy can be done in two different ways. The first method involves a small incision and a piece of tumor removed, this is called an open biopsy. The second method is called a needle biopsy where a needle is inserted into the tumor and a core of cells is removed for diagnosis.

PET Scan

A PET scan is typically not a standard scan for osteosarcoma, but your doctor may use it in conjunction with, or instead of a bone scan. This scan uses a sugar particle tagged with a tracer to evaluate tissue metabolism. Cancers often have increased metabolism so they tend to pick up a lot of the sugar.

X-Ray

An X-Ray allows the doctor to visualize the bone and tumor. The doctor can measure and determine the amount of destruction of the bone and if there are concerns for stability or fracture.

Tests used to determine stage and plan for potential surgery:
After a diagnosis of osteosarcoma, additional testing is often done to determine the stage and prepare a treatment plan. Osteosarcoma is not staged by a traditional scale of 1-4 like many cancers. Instead osteosarcoma is usually staged as localized or metastatic. Localized osteosarcoma indicates that there is only one tumor in the primary site and no other evidence of disease elsewhere in the body. Metastatic indicates that there are two or more areas of disease in the body. Additional tests used to determine stage and treatment options include:

A bone scan is used to determine if there are other bones in the body with disease. This test involves an injection of a substance (nuclear tracer) into the vein that is taken up by bone that is actively growing. This nuclear tracer is detected by the medical equipment and creates an image of the patient’s skeletal structure that helps your doctor evaluate changes in bone metabolism. Bone metabolism is increased in areas of growth and in tumor(s).

A CT without contrast (or a CAT scan) will evaluate if there are any nodules in the lungs. This scan is performed to view thin slices (every 2-3 millimeters) of the lungs. Most protocols consider nodules 1 centimeter or larger to be concerning. Having a few very small nodules (2-3 mm) can be normal is people especially if they have recently had a cold. Usually contrast (a substance injected into the patient) is not used in CT scans of the lungs for osteosarcoma as the contrast is used to evaluate lymph nodes or other structures in the chest. This is usually not necessary information for osteosarcoma as lymph nodes are generally not impacted.

An MRI can be ordered with or without contrast to evaluate the primary tumor size and extension into the soft tissue/ muscle/ bone marrow (center of the bone). This test will show where the blood vessels and nerves are in relation to the tumor which will help with surgery planning.

Tests used prior to chemotherapy:
Understanding how a patient’s body is functioning prior to chemotherapy is important to gauge any impact that chemotherapy could or does have upon treatment. Treatment is harsh on the body and doctors will always be considering the risks and benefits of treatment to the patient. Killing all the cancer cells with little or no future impact on the person’s body is important but each person and their bodies ability to handle these harsh treatments is different. So understanding a patient’s body before treatment and watching how their body is impacted by treatment is crucial.

An echocardiogram and EKG are done to test the functioning of the patient’s heart. Some of the chemotherapy agents can impact the function of heart muscle.

Blood tests are done to check for the functioning of bone marrow, kidneys and liver.

An audiogram is a test to assess hearing. One of the chemotherapies often used, cisplatin, can impact hearing.

Occasionally neurological functioning tests are done to determine if treatment has an impact on memory.

What is the treatment for Osteosarcoma?

Generally treatment of osteosarcoma involves 10 weeks of chemotherapy followed by surgery to remove the tumor(s) and then a final 13 weeks of chemotherapy. Chemotherapy treatment can involve lots of needles and pokes. To minimize pokes and protect small veins from chemotherapy, a central line is often placed in a patient in a short surgical procedure. There are different types of central lines including; venous catheter (usually called a port) or Hickmann line. Your medical team will determine which is best for your situation.

Chemotherapy

Chemotherapy prior to surgery is called neoadjuvant chemotherapy. This is done to start killing the cancer cells as quickly as possible since choosing a surgical option and planning necessary to surgery can take longer than is optimal to begin treatment. The traditional chemotherapy regimen suggested for osteosarcoma by COG (Children’s Oncology Group in the United States) is called MAP. MAP consists of methotrexate, adriamycin (also called doxorubicin) and cisplatin (also called platinol).

Surgical options

Surgical options will differ depending upon the bone(s) impacted by the osteosarcoma, spread, ability to surgically get all the cancer out, future function requests and preference by the patient. For the long bones where traditionally osteosarcoma is found, arms and legs, surgical options often consist of limb salvage surgery or amputation (either traditional or rotationplasty for the leg). For lung nodules surgical options considered are thoracotomy or thoracoscopy. Rarer bone tumors in the pelvis, spine or jaw will often require a more unique approach to surgery and be specialized for the exact placement of the patient’s tumor.

Chemotherapy after surgery is called adjuvant therapy. This is done to continue killing any osteosarcoma cells that may be left in the body after surgery. Since the addition of adjuvant chemotherapy to osteosarcoma treatment, the relapse rates have decreased dramatically indicating the need for additional chemotherapy after surgery, even when the patient appears to be cancer free.

Osteosarcoma resources osteosarcoma hotline

Methotrexate: what to expect

Glossary

Trial Status

LSS

LSS is a shorthand for Limb-Salvage or Limb-Sparing Surgery. It is a complex surgery that removes the entire tumor along with the surrounding bone. If the tumor extends out of the bone then the bone along with the muscle and tissue the tumor touches are all removed. The bone is typically replaced with a bone graft or an internal prosthesis to preserve the majority of functionality.

MAP

MAP is a shorthand representation for the three chemotherapies most commonly used in the treatment of osteosarcoma; M=Methotrexate, A= Doxorubicin (Adriamycin) and C= Cisplatin (also called Platinol®).

MAPIE

MAPIE is a shorthand representation for the five chemotherapies commonly used in the treatment of osteosarcoma when a poor response is seen post-surgery; M= Methotrexate, A= Doxorubicin (Adriamycin), C= Cisplatin, I= Ifosfamide, and E= Etoposide. MTX: MTX is a shorthand representation for Methotrexate, chemotherapy used in the treatment of osteosarcoma. (the “M” in MAP or MAPIE).

HDMTX

HDMTX is a shorthand representation for High-Dose Methotrexate, the high-dose is often used in the treatment of osteosarcoma if tolerated.

Blood Tests

ANC

ANC stands for Absolute Neutrophil Count. Neutrophils are a type of white blood cell that helps fight infection. ANC is part of the report from a CBC (complete blood count) test. If the ANC is too low a condition called Neutropenia exists and doctors will recommend special instructions to minimize the risk of infection while the body is in this state of low tolerance to infections.

CBC

CBC stands for Complete Blood Count and is a type of test doctors request on a patient’s blood. It provides information about the type, count and concentration of the blood’s makeup.

Hct

Hct stands for Hematocrit. Hct is the percentage of red blood cells in the blood. If this number is too low the body’s ability to move oxygen from the lungs to other parts of the body may be impacted.

Hgb

Hgb stands for Hemoglobin. Hgb is the part of red blood cells that carry oxygen from the lungs to other parts of the body and brings carbon dioxide back to the lungs.

Plts

Plts stands for Platelets. Platelets are the part of the blood associated with promoting clotting or clumping to stop bleeding when the body is injured. Treatments for osteosarcoma can lower the body’s platelet count. If the count drops too low, doctors may recommend a transfusion.

RBC

RBC stands for Red Blood Cells. RBC’s are also called erythrocytes and are the part of the blood associated with carrying oxygen throughout the body in the hemoglobin contained in the RBCs. It is typically part of the CBC Blood test ordered by doctors.

WBC

WBC stands for White Blood Cells. WBC’s are also called leukocytes and are the part of the blood associated with fighting infection. During osteosarcoma treatments, the number is reported from blood tests to determine the impact of chemotherapy on the body’s ability to fight infections

Lines and Ports

IV is short for intravenous and refers to the apparatus used to deliver drugs or fluids directly into the bloodstream via a vein. IV is also used to refer specifically to the needle and tubing inserted in the arm to deliver drugs or fluid. Other devices used to provide IV therapy are PICC lines and Port-a-Caths.

PICC

PICC is short for Peripherally Inserted Central Catheter. A PICC line is a catheter surgically inserted in a central vein, typically in an arm. While it is installed under the skin, a portion of the catheter, “tail”, sticks out of the skin. The PICC line requires special care to keep it clean and sterile. The benefit of having a PICC line is reducing the stress and damage that repeated placement of IV’s can cause on the veins.

PORT

Port is short for Port-a-Cath which is an intravenous catheter device that is placed under the skin and used to give treatments like fluids, drugs, and blood product transfusions. It is also used to draw blood for tests. The Port is typically placed in the chest. The benefit of having a Port is reducing the stress and damage that repeated placement of IV’s can cause on the veins. It is also less susceptible to infections than other approaches. It appears as a bump in the skin and often compared to what a pacemaker looks like. For some chemotherapies, a port is required for the safety of the patient.

DE-PORT

De-Port is a humorous term used by many patients and their caregivers to refer to the time and procedure to remove the Port-a-Cath once treatments are complete and it is no longer needed. Similar to the celebration of the “end of chemo”, the removal of the port is a milestone to celebrate. Often a “Port Party” ensues.

Scans and Tests

BONE SCAN

Bone Scans use nuclear imaging to create a full body image of the skeletal structure and any differences in bone metabolism. The process involves an injection of the tracer and requiring a couple hours for it to be absorbed into the bone. Then the imaging is taken in a process that requires the patient to lie still on a table for about 30 or more minutes while the scanning equipment moves across the body. For osteosarcoma, bone scans are used to isolate the area of the tumor and determine if it has metastasized to any other bones in the body. In addition, it is used during follow up scans to see if any recurrence has occurred.

CT or CAT scan is short for Computed Tomography and is an imaging tool used for diagnostics. It’s a computer-guided combination of many x-rays taking cross-sectional images to create a threedimensional image of organs, bones and soft tissue. For osteosarcoma chest CT’s are frequently used to see if it has metastasized to the lungs. A CT can be easily visualized by thinking about slicing into a loaf of raisin bread. Some ‘slices’ yield a raisin and some don’t. In a CT scan takes photos of slices capturing a possible tumor is analogous to slicing bread and ‘catching’ a raisin.

MRI

MRI stands for Magnetic Resonance Imaging. It is a medical imaging approach used to obtain pictures of organs and soft tissue in the body. It uses a magnetic field instead of radiation like X-rays and CT scans. For osteosarcoma, MRI’s are used in the initial diagnosis and as a pre-operative tool to determine the size and location of the tumor.

PET

PET is short for Positron Emission Tomography and is a scan that uses a tracer or die to create imaging that allows doctors to see how tissues and organs are functioning. PET scans are routinely used in the diagnosis and treatment of other cancers. In osteosarcoma, doctors may use a PET-CT but are often referred to just as a PET scan. The combination of PET and CT allows doctors to see better abnormal activity associated with cancer along with details on location and size. Many osteosarcoma patients never have a PET scan as there is some debate of the usefulness of the data and false readings.

Status

N.E.D.

NED stands for No Evidence of Disease. Generally, a patient is considered NED from their end of therapy clear scans to 5 years post-treatment after which they are considered a survivor.

N.E.R.D.

NERD stands for No Evidence of Recurrent Disease. Generally, a patient is considered NERD from their end of relapse therapy clear scans to 5 years post-relapse treatment after which they are considered a survivor.