Most sarcomas don’t cause symptoms until they grow and press against nerves, muscles, bones or blood vessels. Early signs are a painless lump or swelling.
Your provider will describe how far the cancer has spread (stage). They’ll also tell you what type of sarcoma it is. This helps them plan your treatment.
Sarcomas can grow anywhere in the bones and soft tissues that surround and support the body. They may begin in bone or in any tissue that makes up muscles, tendons, cartilage, fat, blood vessels and lymph (a thin layer of fluid that runs through the lymph nodes).
Most sarcomas don’t cause symptoms in their early stages. But a sarcoma that starts in the soft tissues of an arm or leg can grow rapidly without causing pain or other signs until it is very large. Then it might start to press on nerves or muscles, causing pain and swelling. It’s important to see a doctor right away when you notice a new lump or swelling.
The doctor will ask about your past health, symptoms and risk factors. Then the doctor will do a physical exam. They will feel the lump and look at it closely. The doctor may order an X-ray or CT scan to see the size and location of the tumor. They may also order a blood test to check your red and white blood cells and platelets. They may also do a chest X-ray to check for spread to the lungs.
If the sarcoma is in soft tissue, your doctor will probably do a biopsy to get a sample of the tumor for testing. They may do this on their own or with the help of a specialist called a pathologist. A pathologist is a doctor who studies cells and tissue under a microscope to look for abnormalities like cancer. The provider will send the biopsy to a laboratory to be tested for sarcoma.
The lab will also measure how fast the tumor is growing and whether it has spread to other areas of the body (metastasized). It will give your provider a number between 1 and 4 that describes how much the sarcoma has grown and how normal the cancer cells look. This information helps the provider decide what treatment is best for you.
Although sarcomas are rare, it is important to have any lump or bump that might be a sarcoma diagnosed by a doctor as soon as possible. If left undiagnosed, sarcoma can grow and spread. Sarcomas develop in muscle, fat, fibrous tissue, tendons and blood vessels and often appear as a lump or bump. They can form anywhere in the body, but most commonly occur in the arms and legs. The first step in diagnosing a sarcoma is usually an imaging test such as an X-ray or CT (computerized tomography) scan.
An X-ray is an image of the inside of your body that shows bones and other tissues. Bone sarcomas usually show up as a lump on X-rays. A CT scan creates detailed pictures of the structures in your body by combining X-rays with computer technology. This allows your care team to view soft-tissue and bone sarcomas and also detect whether they have spread to other parts of your body.
A CT scan can help distinguish between benign and cancerous masses by looking at size, shape, location, and how fast the tumor is growing. The results of the X-ray and CT can help your doctor determine if a sarcoma is low or high grade, which may affect treatment options.
Your care team may also recommend a fine needle aspiration or core needle biopsy to get a sample of the suspected tumor for testing. During a fine needle aspiration, a small needle is inserted into the tumor and suction is applied. The doctor can then examine the cells under a microscope to find out the type and grade of the sarcoma.
A core needle biopsy uses a larger needle than the FNA biopsy to remove a cylindrical piece of tissue from the suspected sarcoma. Your doctor can then examine the tissue under a microscope to find out if it is a low or high-grade sarcoma and whether it has spread.
Your care team may recommend chemotherapy, which helps shrink the tumor and improve the chance that all of the cancer can be removed during surgery. Chemotherapy works by targeting rapidly dividing cancer cells in your body. The type of chemotherapy your care team prescribes depends on the type of sarcoma and where it is located in your body.
Getting diagnosed with a sarcoma is stressful enough, but what you do about it next can make a huge difference in how your cancer plays out. To start with, you’ll want to seek out a cancer center that has expertise in treating sarcomas and that includes a sarcoma specialist on their medical staff. This doctor will examine you and may order tests to help diagnose your condition. They will likely order a biopsy to get a sample of the tumor tissue and work with pathologists to determine the type and stage of your sarcoma.
A biopsy involves inserting a needle-like instrument or cutting into the lump to obtain a small piece of abnormal tissue that can be tested under a microscope for cancer cells. Depending on where your sarcoma is, doctors can use ultrasound or CT scans to guide the needle or cut. In bone sarcomas, they can also insert a device called a bone scan to see how the cancer is affecting surrounding tissues.
After a biopsy, your sarcoma specialist can create a treatment plan. They will consider the type and stage of your sarcoma, where it is located, your age, fitness level, overall health, and personal preferences when creating this plan. They will then walk you through the options and answer any questions you might have.
Treatment for sarcoma can involve surgery, chemotherapy and/or radiation therapy. They may be used alone or in combination, and the type of treatment will depend on the type of sarcoma you have, where it is located, and whether it has spread.
For soft tissue sarcomas, your doctor will most likely recommend surgery to remove the tumor and some of the surrounding healthy tissue. This may be done by a surgeon or surgical oncologist who has experience treating sarcomas.
Chemotherapy for sarcomas typically uses drugs that are designed to kill cancer cells and stop them from growing. This type of treatment is very effective for most sarcomas, but it does not always lead to a cure. The types of chemotherapy medications used will vary depending on the type of sarcoma and your previous treatments.
A sarcoma is a cancer that forms from bone or soft tissue. The tumors can form anywhere in the body, but they are most common in the arms and legs. Less than one percent of all cancers are sarcomas. Sarcomas may grow slowly and not cause any symptoms at all. Others grow quickly and may feel like a lump under the skin.
The treatment for a sarcoma will depend on where the tumor is and how big it is. You might have surgery to remove the tumor. It is often possible to remove all the cancer and save the arm or leg. This type of surgery is called limb-sparing surgery. You may have radiation or chemotherapy after surgery to kill any remaining cancer cells. You may also take part in a clinical trial to try new treatments for sarcoma.
Your doctor will ask about your health history and do an exam. Then they will order tests to find out if you have a sarcoma. You may have a CT scan or MRI to see the inside of your body and help find the location of the tumor. These tests can also show how the tumor is growing and if it has spread.
You may have a biopsy to get samples of the tumor for testing. Then a pathologist will look at the samples under a microscope to find out what type of sarcoma you have. They will also do special stains to learn more about the cells. The type of sarcoma is important to know because different types of sarcoma grow and spread differently.
Some types of sarcoma are more likely to spread (metastasize) and have a worse prognosis than other types of sarcoma. Your doctor will use information about your type of sarcoma and other factors to make a prediction (or estimate) of how well you are likely to do. These factors are called prognostic and predictive factors.
The first symptom of sarcoma is often a painless lump that grows over time. It may grow into the bone or muscle, and it can cause pain if it presses on nerves or muscles.
Sarcomas can start in any type of soft tissue, including fat, muscles, nerves and blood vessels. They can grow almost anywhere in the body, but they are most common in the arms and legs.
Sarcomas can be found throughout the body, but they most often grow in bone or soft tissues like muscle, fat, nerves, fibrous tissue and tendons. Usually, they don’t cause symptoms until they get large enough to put pressure on nearby nerves or muscles. As a result, most people don’t know they have a sarcoma until it is diagnosed after surgery or as a side effect from other cancer treatments. Some sarcomas, such as osteosarcoma, develop in the bones of the arms and legs, but they can also occur in the pelvis, chest wall, head and neck.
For a diagnosis, doctors need to perform tests that can help them determine if a tumor is a sarcoma and what type it is. They may order an ultrasound, CT scan or MRI to look at the tumor and the surrounding area. They might also order a biopsy, which is the removal of a small sample of the tumor for lab testing. This can help them decide the best treatment options.
Bone sarcomas tend to develop as a painful lump or mass in the bones of the arms and legs, although they can occur in other areas of the body as well. In the early stages, they’re often painless and are sometimes mistaken for growing pains or sports injuries in children. But as they grow, they may cause pain or tenderness to the touch and restrict movement in the affected limb.
Soft tissue sarcomas are more difficult to diagnose than other types of cancer because they rarely cause pain in the early stages. They also don’t show up on medical tests until they are quite large, so many people only discover them after seeing a doctor about pain or swelling in the arm or leg.
In a fine needle aspiration (FNA) biopsy, a doctor uses a thin needle to remove a small amount of tissue from the tumor and sends it for testing. In a core needle biopsy, the doctor uses a larger needle to remove a cylindrical piece of tissue from the tumor with help from a CT or MRI scan. A pathologist looks at the tissue under a microscope to see if it’s a sarcoma and what kind.
Sarcomas are cancers that arise from cells that form soft tissues in the body, such as muscles, nerves, fat, tendons and blood and lymph vessel tissue. These cancers can spread, or metastasize, to other parts of the body. This can cause pain, swelling and other symptoms in these areas.
Uncomfortable or unexplained swelling is a common sign of sarcoma. It may be a symptom in the arms or legs, but it can also affect other parts of the body such as the chest and abdomen. The swelling is caused by the growth of a tumor in the area. In some cases, the swelling is so severe that it restricts a person’s mobility.
Swelling from a sarcoma can sometimes be mistaken for other conditions like a hematoma (bad bruise), abscess, pulled muscle or a lump made of fat cells called a lipoma. For this reason, many people don’t get diagnosed with sarcoma until the cancer has grown too large to be ignored.
Most sarcomas start in bones, but some types begin in the soft tissues of the body. If they are not treated, they can spread to the lungs or other organs. This is known as secondary sarcoma, or metastatic sarcoma.
If you have pain and a swelling in your arms or legs, make an appointment with your doctor. They will examine you and do some tests to find out what is causing the problem. This will include an X-ray and a CT scan or MRI of the area. These tests will show your care team what kind of sarcoma you have and where it is located. They will also determine the stage of your sarcoma, which describes how developed it is and whether or not it has spread.
Some sarcomas occur in the blood vessels and some develop when immature bone or soft tissue cells change and grow abnormally. Other sarcomas are linked to certain genetic conditions such as von Recklinghausen disease, Li-Fraumeni syndrome and retinoblastoma. Others are related to being exposed to some chemicals including vinyl chloride monomer used to make plastics, arsenic and high doses of herbicides (weedkillers). Some people are more likely to get a specific type of sarcoma because they have a rare inherited condition.
Sarcomas affect the tissues that connect, support and surround organs and other body structures. They may form in the fat, muscle, blood vessels and deep skin tissue, as well as tendons and ligaments. They can grow anywhere in the body, but most often develop in the arms and legs.
Early symptoms include a painless lump or swelling in an area of the body. The cancer may grow and press on nearby nerves, muscles or bones. It can also cause an area of the body to feel full or swell up, especially if it is near a blood vessel or major muscle. Some types of sarcoma can grow in the stomach and intestines, causing abdominal pain or a feeling of fullness (nausea).
If you notice a lump, see your doctor right away. He or she will do a physical exam to look at the lump and find out where it’s located. Some types of sarcoma are named for the normal cells they most closely resemble, and others are named for the part of the body where they start. Healthcare providers also use other tests to figure out the type of sarcoma you have and its stage. The stages of a tumor help your provider know whether it grows fast, grows slowly or spreads (metastasizes).
X-rays and CT scans are noninvasive tests that allow your care team to see inside the body without touching you. They take data from X-rays and convert it into pictures of your bones and soft tissue on a monitor. A CT scan takes more detailed data than an X-ray and shows the inside of your chest and abdomen. If your sarcoma starts in the bone, a chest X-ray is also done to check for signs of cancer spreading to the lungs.
Other noninvasive diagnostic tests include a blood test to measure certain proteins in your body that can be used to help diagnose the type of sarcoma and its stage. Your doctor may also order a biopsy to remove some of the tissue and send it to a lab for testing. This can tell your provider how much of the sarcoma is dead or alive. A biopsy can also help your doctor decide the best treatment for you.
Fatigue is a common problem for people with cancer. It can happen at any time and can be hard to describe. It can affect how you feel, your daily activities and your relationships with others. Sometimes it is not understood by the people around you, especially if they haven’t had cancer themselves, and can lead to feelings of stress and depression.
People with soft tissue sarcomas (STS) often have pain and fatigue that is difficult to measure. This is because the tumors start in cells that make up the body’s connective tissues – that’s what holds you and everything else together, including bones, muscles, tendons and ligaments, and fat. The tumors can also spread to other parts of the body – this is called metastasis.
Because sarcomas begin in flexible, elastic tissues or deep spaces of the body, they often grow very quickly and may be hidden until it is too late. This is why it is important to see your doctor if you develop a lump or bump that is not painful or does not hurt over time, even if it is in an unusual location in the body.
In the early stages of STS, a painless lump can become larger without causing any symptoms, and it may begin to press against nerves or muscles, leading to pain or soreness. The lump may be mistaken for a benign fatty lump (lipoma) or bruise (haematoma), which can delay tests to diagnose the sarcoma.
Fatigue is a very common side effect of treatments for sarcoma and can persist for many months or years after the treatment has finished. The tiredness can be a reminder of the disease and that you might not have as much energy as you used to, which can be upsetting. It is important to talk to your medical team about your tiredness and how it is affecting your life.
It can be helpful to ask for help and support from your family, friends and colleagues. Your doctor or nurse can give you advice on how to manage your symptoms and ways to get relief from the fatigue. You might need to change your daily routine to save your energy for essential tasks and social activities. You might have to stop working or reduce the number of hours you work. If you are unable to work or don’t have enough income, it can be stressful and lead to financial difficulties.
Sarcomas are cancers that can occur in the bones or soft tissues of your body. If left untreated, sarcoma can spread to other parts of your body (metastasize).
There is evidence that routine referral to a sarcoma centre improves clinical outcomes for patients. However, this is not yet the case in most health care systems.
Surgical excision is the main treatment for most types of soft tissue sarcoma. It involves removing the tumor and some healthy tissue around it (called a margin). The surgeon uses a surgical knife to cut out the cancer. The surgeon then closes the area using stitches. The surgeon may also use reconstructive surgery to fix the area after surgery. Before the operation, you will receive general or local anesthesia. The surgeon will test the removed tissue for cancer cells under a microscope to make sure that all of the cancer is gone.
If the sarcoma is in an arm or leg, doctors can usually remove it without amputation. This is called limb-sparing surgery. The surgeon may use neoadjuvant radiation therapy and chemotherapy to shrink the tumor before surgery. They can also use these treatments to reduce the chance that the tumor will come back in the same spot after surgery.
Amputation used to be the only option for sarcoma in an arm or leg, but advances in surgical techniques and new treatments like neoadjuvant radiation and chemotherapy have made it possible for doctors to save more limbs. However, amputation may still be needed if the tumor is large or is in a place where it would be hard to reach with a knife, such as near important nerves or blood vessels.
Sometimes a surgeon will use a technique called compartmental excision to remove a large part of the limb. This is based on the idea that solid tumours tend to grow in well-defined barriers of tissue called fasciae, which are tight and densely connected to muscles. When a surgeon cuts away these barriers to the tumour, it is difficult for the tumour to spread into surrounding tissues. This can lead to better local control of the tumour than traditional wide-margin excisions.
If a doctor uses compartmental excision to remove a part of the limb, they can often use a piece of skin from another area of the body (called a skin graft). This means that you will not need an artificial limb. If you do have to get an artificial limb, modern prostheses can allow you to function at the same level as before you had the operation.
Radiation therapy can help prevent sarcomas from coming back in the same place (local recurrence) after surgery. It can also be used to relieve pain and other symptoms. The type of radiation your doctor uses depends on the type and stage of sarcoma, as well as where it is located in your body.
Your doctor will use special imaging tests to find out the size and location of your sarcoma and how it’s spread. This information is used to plan your treatment. For example, if your sarcoma is in the lungs, doctors may give you chemotherapy before surgery to shrink the tumor so it’s easier to remove. This is called neoadjuvant therapy. Chemotherapy can also be given after surgery to destroy any cancer cells that remain.
When giving radiation, doctors try to avoid damaging nearby tissues and organs. They do this by using advanced technology, including computer-aided radiation planning and image-guided treatments. For example, your care team might use stereotactic body radiation therapy (SBRT) or three-dimensional conformal radiation therapy (3D-CRT). These treatments use shaped energy beams that match the shape of your tumor. They can also use intensity modulated radiation therapy (IMRT), which focuses more radiation on the tumor and less on healthy tissue.
Your sarcoma might spread to other parts of your body before you even notice it. When this happens, it’s important for your sarcoma doctor to know if the cancer has spread so that they can plan additional treatment. Your doctor can learn about this by performing a chest CT scan, an MRI or a PET scan.
If your sarcoma has spread to your lungs, we might suggest you take part in a clinical trial that’s exploring new treatments for sarcomas in the lungs. These trials might include drugs that target specific weaknesses in your cancer cells, immunotherapies, biologic therapies or other types of experimental treatments.
After your sarcoma treatment, your doctor will recommend follow-up care to watch for any long-term side effects or late effects (which happen after your sarcoma treatment is over). Your sarcoma survivor support group can offer you support and resources.
Many types of cancer are treated with chemotherapy, a group of drugs that kills cancer cells. Doctors use a special type of chemotherapy called targeted therapy to treat certain types of sarcoma. This treatment targets specific molecules on or inside the cancer cells that help them grow and spread, limiting damage to normal tissue. Examples of drugs used in this type of treatment include doxorubicin (Adriamycin), gemcitabine (Gemzar), docetaxel (taxotere) and ifosfamide (Ifex). A drug that inhibits the growth of blood vessels around a tumor is also sometimes used, such as mesna (mesna) or pazopanib (Votrient).
For soft-tissue sarcomas or bone sarcomas, doctors often give chemotherapy before surgery to shrink the tumor and make it easier to remove. They may also combine radiation and chemotherapy to prevent the cancer from coming back after surgery.
Your medical team will discuss all of your treatment options and the results of your tests with you. This discussion is called shared decision-making. Your team will take into account the type, grade and stage of your sarcoma, possible side effects and your preferences and overall health.
Doctors determine the stage of a tumor by measuring how large it is and whether it has spread from where it started. They also look at other factors, such as your age and whether the cancer has spread to lymph nodes or other parts of the body. This information helps them decide which treatments to recommend.
If a sarcoma is found at an early stage and has not spread from where it began, surgery can usually be very effective. The chances of a cure increase with the smaller and lower-grade the tumor is.
When a sarcoma has spread, it is harder to remove and the chance of a cure decreases. The best treatment is a combination of surgery, radiation and chemotherapy.
Surgical treatment is the main way to treat soft tissue sarcoma. The goal of surgery is to remove all traces of cancer from the body with a clear margin (area of healthy tissue around the tumour). Your surgeon may also recommend drug therapies or radiation therapy after the operation to help destroy any remaining cancer cells. For some sarcomas in the trunk, such as gastrointestinal stromal tumors (GISTs), plastic and reconstructive surgeons may also be involved. If the sarcoma wraps around important blood vessels or nerves, specialist vascular and neurosurgeons may also join your cancer care team.
Some types of sarcoma develop in the limbs, such as arms or legs. If the sarcoma is very small, your surgeon may be able to cut out only part of it and leave the rest of the limb. This is called limb sparing surgery. This type of surgery is usually done with a local anesthetic. A skin graft or flap may be used to cover the area where the surgeon removed the cancer.
A skin graft is a thin piece of skin from another part of your body, such as the inner thigh or buttock. A plastic surgeon puts the graft over the area where the cancer was. The surgeon then stitches it in place and covers it with a bandage. The graft and donor site heal within 1 to 2 weeks.
For some sarcomas, such as GISTs in the gastrointestinal tract, a bone reconstruction is done with a metal implant or special cement to make sure you can walk and move normally. This is done at the same time as the sarcoma surgery.
In some cases, especially with sarcomas in the arms or legs, your doctor might use neoadjuvant radiation therapy and chemotherapy to shrink the cancer before surgery. This is especially important if the sarcoma has affected essential nerves, blood vessels or other critical structures and organs.
Some sarcomas spread to the lungs, and surgery is used to remove these metastases. This is done under a general anesthetic in the hospital operating room. If your sarcoma has spread to the lymph nodes in your chest, your surgeon may perform a sentinel lymph node biopsy or a lymphadenectomy.
There are more than 70 different types of sarcoma. Each one needs a different treatment approach.
Sarcomas can grow into other tissues, bones, or internal organs (metastasis). They often develop in children and young adults. Some types of sarcoma are slow-growing and don’t spread much. Others are more aggressive and more likely to spread to the lungs or bone.
Undifferentiated pleomorphic sarcoma (UPS) is the most common type of adult soft tissue sarcoma (STS). It was formerly known as malignant fibrous histiocytoma (MFH). It grows quickly and often forms in the extremities or trunk, although it can also appear in the head and neck, retroperitoneum, bones, or other organs. It’s a high-grade STS and can be difficult to treat. UPS is more common in men than in women.
Scientists aren’t sure what causes this cancer, but they believe genetic changes play a role. Some people who get this tumor develop it near an area that had radiation therapy to treat another type of cancer. It’s rare for a radiation-associated sarcoma to form in the same place again, but it does happen.
To diagnose UPS, your healthcare provider will do a physical examination of the affected area and review your symptoms. He or she will also order imaging tests to create pictures of the area and help your doctor understand the size and location of the growth. X-rays, CT scans, and magnetic resonance imaging (MRI) may be used to look for the tumor. A biopsy, the removal of a small piece of tissue for examination under a microscope, can help your doctor make a diagnosis of UPS.
If your healthcare provider finds that you have UPS, he or she will use information about the size of the tumor, the types of cells it contains, and whether it has spread to other parts of your body to determine its stage. Staging helps your healthcare provider recommend the best treatment options for you.
The treatment for UPS usually includes surgery, radiation therapy, and chemotherapy. Surgery involves removing the tumor and surrounding tissue. Radiation therapy uses high-energy rays to kill cancer cells. Chemotherapy is a combination of drugs that kill cancer cells and stop them from growing or spreading. It’s most commonly used to treat sarcoma that comes back after surgery or that spreads to other areas of your body.
After your treatment, your doctor will schedule regular follow-up visits to monitor the condition and check for recurrent tumors or any new ones that have formed. These visits will likely last for at least 10 years.
Dermatofibrosarcoma protuberans (DFSP) is a slow-growing malignant soft tissue tumor of the skin that can spread to deeper structures such as fat, muscle, and bone. It can also metastasize to distant organs such as the lungs. DFSP is one of the rarest cancers that affects the subcutaneous layer of the skin. NewYork-Presbyterian’s multidisciplinary team of skin cancer and sarcoma specialists is experienced in treating this aggressive tumor, so the sooner it is diagnosed, the better your prognosis will be.
DFSP occurs most often in middle-aged adults, but can occur at any age. It can appear in the trunk (50%), extremities (35%), and head and neck (15%). It is characterized by multiple raised, violaceus to red-brown indurated plaques that resemble keloid or scar tissue. DFSPs grow slowly and may ulcerate, especially when they are located on the extremities.
Invasive DFSPs tend to invade radially through preexisting collagen bundles and deeply along connective tissue septae. Consequently, it is challenging to obtain adequate surgical margins to prevent local recurrence or metastatic spread. In addition, the uniform spindle cell morphology and immunoreactivity of DFSP overlaps with a broad range of benign and malignant neoplasms, which must be differentiated from it.
It is important to see your healthcare provider any time you notice a change in your skin. Some changes, such as a skin-colored or red-brown spot that grows rapidly and doesn’t heal after a few weeks, may be signs of a more serious health condition like a sarcoma.
In general, sarcomas are treated with surgery followed by radiation and chemotherapy. NewYork-Presbyterian’s specialized sarcoma surgeons are expert in the treatment of dermatofibrosarcoma protuberans and other soft tissue sarcomas. They use Mohs micrographic surgery to ensure that all abnormal cells are removed during the initial surgery. They also offer radiation therapy and tyrosine kinase inhibitors to treat recurrent or advanced DFSP, including the COL1A1-PDGFB fusion variant. These therapies are effective in preventing the disease from spreading to other parts of the body and increasing survival. Patients with recurrent or advanced sarcoma are followed regularly for signs of disease progression by the sarcoma tumor board to discuss the best treatment plan.
A malignant peripheral nerve sheath tumor is a cancer that starts in the sheaths that cover the nerves. This type of sarcoma usually forms in the arms or legs, but can also form in other parts of the body. These types of tumors are slow-growing and often grow into surrounding tissue before they spread to other organs.
Like other soft-tissue sarcomas, MPNST is not well understood. It is thought that the sarcoma develops when cells in the sheath begin to grow and divide more quickly than normal, which eventually leads to tumor formation. It is not clear what causes this to happen, but it is believed that NF1 may play a role in the development of MPNST.
The prognosis of people with MPNST depends on the location and the size of the tumor. People with smaller tumors and those whose tumors have not spread to other parts of the body tend to live longer than those who have larger or more advanced tumors.
When a person is diagnosed with a sarcoma, it’s important to work with a team of specialists. It’s possible to improve a person’s chances of survival by getting the right treatment as soon as possible.
If you have a lump or growth that’s growing in your arm, leg, trunk, or head, it’s important to make an appointment with your health care provider as soon as possible. Your doctor will examine you, and then order tests to check for a sarcoma or another health condition.
Some sarcomas look so similar to healthy tissue that they can be mistaken for other, more common conditions. As a result, it can be challenging for health care providers to diagnose sarcoma early. To help, researchers have developed a liquid biopsy ctDNA test to detect mutations in cancerous tissue. The test is more accurate than previous methods, such as FDG-PET and MRI. It can also identify other genetic mutations and copy number alterations, such as those found in plexiform neurofibromas, which can transform into deadly MPNST.
Ewing sarcoma develops when cells get changes (mutations) that make them grow out of control. These changes usually happen in the bone cells that make up bones, muscle and other soft tissues. These cancerous cells may break away from the bones and spread to other parts of the body. Doctors don’t know exactly what causes this cancer, but they do know that it isn’t caused by any diet, lifestyle or other environmental factors.
The first symptom of Ewing sarcoma is often pain or a lump on the bone where the tumor is located. The tumor can also cause the bones to weaken and bend. The condition may also be diagnosed based on physical exam and the results of lab tests, including a complete blood count and a chest X-ray or CT scan. A sample of the cancerous cells is needed to confirm the diagnosis and see how the cancer is growing. These samples can be taken from the bone with a needle or from a small sample of the surrounding tissue. The cells are checked for cancer by doctors trained in special pathology.
After a biopsy, the doctor will tell you how to treat your child’s Ewing sarcoma. Treatment may include surgery, radiation therapy and chemotherapy. The goal is to destroy all of the cancer cells and prevent the cancer from returning.
For Ewing sarcoma that hasn’t spread, cure rates are as high as 80%. The chances of survival drop significantly if the tumor has spread, though. That’s why it’s important to see a doctor right away if your child has symptoms of this disease.
In most cases, Ewing sarcoma that starts in the long bones of the arms and legs is treated with surgery and radiation. At CHOP, we are experts in limb-sparing and reconstructive surgery for children with these kinds of tumors. Our team is always working to improve these procedures so we can give children the best chance of a full recovery.
After surgery, your child will receive chemotherapy to kill any remaining cancer cells and prevent the tumor from returning. The chemotherapy can be given by mouth or injected into the vein or muscle. Your child may also have combination chemotherapy, which uses more than one anticancer drug. In some cases, your child will be offered a place on a clinical trial of an experimental medicine to help with treatment.