Sarcoma is a rare type of cancer that develops in bones or soft tissue, such as muscle, fat, nerves, blood vessels and deep skin tissues. It can grow in any part of the body, but most often starts in the arms or legs.
A painless lump that gets bigger over time is cause for concern and should be evaluated. Certain inherited conditions, exposure to chemicals such as vinyl chloride monomer (used to make some types of plastics) and long-term lymphedema can increase the risk of sarcoma.
A sarcoma is cancer that develops from bone or soft tissues, such as fat, muscles, nerves and other connective tissue. It can occur in both children and adults. The most common type of sarcoma starts in bones, while the second most common type begins in soft tissues. There are more than 70 different types of sarcoma. They vary in where they start, how quickly they grow, and how they respond to treatment.
Most sarcomas are slow-growing, but some grow faster than others. They can also grow into nearby tissues or organs, and sometimes spread to other parts of the body. The way a sarcoma grows and spreads helps doctors decide what kind of treatment to give.
Tumors are classified according to how they look under a microscope. A sarcoma is categorized as low-, intermediate- or high-grade based on how closely it looks like healthy cells and how fast they grow. The higher the grade, the more aggressive the sarcoma is.
When a doctor diagnoses a sarcoma, he or she will describe the type of tumor, where it started and how fast it is growing. A biopsy will be done to confirm the diagnosis and to find out if it is a cancer that has spread from other parts of the body.
The most common treatment for sarcoma is surgery to remove the tumor and some of the surrounding tissue. A person may also have radiation therapy before or after surgery to destroy any remaining cancer cells. There are also medicines called chemotherapy drugs that can be used to treat sarcoma. Chemotherapy uses drugs that target rapidly dividing cancer cells and other types of cells.
If a person notices a lump or bump that has not changed over time, it is important to see a doctor right away. The doctor will take a sample of the lump to do a biopsy. A person should also go to a doctor if the lump is painful, if it is getting bigger or if it hurts when touched.
In sarcoma, immature cells develop in an uncontrolled way and can eventually form a tumor that may spread to other parts of the body (metastasize). Sarcomas start in bone or soft tissue, including cartilage, fat, muscle, blood vessels, deep skin tissues and nerves. There are more than 75 types of sarcoma, and each type forms in different areas of the body.
Most sarcomas begin in the arms and legs, but they can also appear on the chest wall, pelvis or head and neck. They often grow very quickly and cause pain, but they can also be mistaken for other conditions such as a simple fatty lump (lipoma) or a bruise (haematoma).
Other symptoms of sarcoma include long-lasting bone pain, swelling and difficulty moving the affected area. They can also be caused by a tumor pressing on nearby bones and affecting blood flow, or by a blood clot that forms in the area.
If you have symptoms, you should see your doctor as soon as possible. They will take a full medical history and do a physical examination. They will feel any lumps and look at the area where you have a problem. They will then order some tests to check whether sarcoma is the cause of your symptoms.
These tests will include a blood test and an X-ray of the area where you have a lump. They will also do a CT scan, which is a painless imaging test that takes pictures of the inside of your body. This can show your doctor what the lump is made of and how big it is. It can also help them decide if surgery is needed.
If a low-grade sarcoma is removed in its early stages, it can usually be cured. But it is more difficult to cure a high-grade sarcoma, especially when it has already spread. Even after treatment, you will need regular checks to see if the cancer comes back or if it has spread. You can have these checks at your local cancer center or your family doctor.
The first step in diagnosing a sarcoma is usually taking an imaging test, such as an X-ray or a CT scan. Both benign and cancerous tumors can show up on these tests. A radiologist (a doctor who performs and interprets these tests) can look at the results of these tests to help determine whether a lump is a sarcoma. However, a biopsy is needed to make a definitive diagnosis.
A biopsy is the removal of a small piece of a suspicious tissue. A pathologist will then examine the tissue under a microscope to see if it is a sarcoma. A pathologist may also use a special stain to help determine the type of sarcoma and its grade. The higher the grade, the more aggressive the sarcoma is likely to be.
Other tests that may be used to help diagnose a soft tissue sarcoma include a blood test and an electrocardiogram, which takes pictures of the heart with sound waves. These can help doctors find out if the tumor is in or near a blood vessel or nearby lymph nodes.
If the sarcoma is in a bone, a chest X-ray and a bone scan may be done to check for signs of spread. A PET scan can also be helpful. This is a test that uses a tiny amount of radioactive glucose to look for malignant cells. Cancer cells take up more glucose than normal cells, so they show up brighter on the PET scan.
MRI (magnetic resonance imaging) can create very detailed images of body tissues and structures. This can be very useful in finding out if a suspected tumor is a sarcoma and if it can be removed with surgery. It can also help doctors figure out the size of a sarcoma.
A sarcoma is a rare disease and it is important to have a biopsy by a specialist who often sees this type of cancer. If the biopsy shows that a person has a sarcoma, the health care team will discuss treatment options with the patient. Getting the right diagnosis is important because a sarcoma can grow quickly and spread to other parts of the body.
The most common treatment for sarcoma is surgery to remove the tumor. Sometimes, doctors use radiation or chemotherapy to treat the sarcoma or to stop it from coming back (recurring).
Soft tissue sarcomas often grow without causing any symptoms. But they can get bigger and cause pain or trouble moving an arm or leg if they press against nerves or muscles. If you have a lump that is growing and not going away, or if it causes pain or other problems, see your doctor right away. A sarcoma may be the problem, but it could also be another type of cancer or a noncancerous (benign) growth.
Your sarcoma treatment team might include doctors who specialize in treating rare cancers. They might suggest that you take part in a clinical trial. You can learn about clinical trials from the Australia and New Zealand Sarcoma Association or Rare Cancers Australia.
If your sarcoma is in the bone, doctors might recommend surgery to remove as much of the tumor as possible. They might also do a more extensive surgery with removal of the lining of the limb or the bone itself (decortication). They might also give you radiation after the operation to kill any remaining cancer cells or prevent them from growing.
Radiation uses high-powered energy beams, such as X-rays or protons, to destroy cancer cells or keep them from growing. You might have radiation treatments from a machine that is outside your body (external beam radiation). Or you might have radiation delivered into your sarcoma site, which is called internal radiotherapy or brachytherapy.
Chemotherapy uses drugs to kill cancer cells or slow their growth. There are many types of chemotherapy drugs, and the one that your doctor prescribes will depend on the type of sarcoma you have and where it is located in your body.
Targeted therapy uses drugs to find and kill cancer cells that are likely to grow, while sparing healthy cells. Examples of targeted therapies for sarcoma include medicines that block the growth of tumors, such as tyrosine kinase inhibitors (such as imatinib). These drugs are used to treat gastrointestinal stromal tumors (GIST), and some other types of sarcoma.
A sarcoma is a type of cancer that develops in bones or soft tissues such as muscle, nerves, fat, fibrous tissue and tendons. It often forms as a painless lump (tumor) in the arms or legs. It may also affect bone and the abdomen/pelvis.
Sarcoma symptoms vary from person to person and depend on the location of the tumor. Symptoms may include pain, swelling and trouble moving.
Sarcomas can develop anywhere in the body in bone or soft tissues like muscle, nerves, fat, fibrous tissue, tendons and blood vessels. They often appear as a lump or growth. It’s important to get any lump or mass checked by your doctor right away if you notice it suddenly or if it grows fast or is painful. If a sarcoma is in the bones, it will most commonly be felt as pain or tenderness that may come and go as the tumor grows or shrinks. If the lump is in a place where it won’t be easily felt, your doctor will use imaging tests to evaluate it, such as X-rays, CT (computerized tomography) scan or ultrasound.
These noninvasive tests can show your care team things about the lump and help guide other diagnostic tests. X-rays are usually the first test a doctor will order, but they may also want to check your heart and lungs for signs of sarcoma spreading to these areas. A CT scan takes a series of X-rays and converts them into images on a computer screen. The resulting pictures can show your doctor the size and shape of your lump or growth as well as any other abnormalities. An MRI (magnetic resonance imaging) is another way of seeing what’s inside your body. These images can help doctors see softer areas of your body, like muscles or fluids, that might not show up on an X-ray.
An ultrasound uses sound waves to see your organs and other structures in your body. This is the best test to see if your lump or growth is made of solid, soft or liquid tissue. It can also tell your care team how big or deep the lump is.
If the sarcoma is in a muscle or bone, surgery is typically needed to remove it. The type of surgery depends on where the sarcoma is, how fast it’s growing and whether it’s in an area that can be safely removed without removing part of your arm or leg. Chemotherapy and radiation treatments are often used with these types of sarcoma to keep the cancer from coming back.
There are a lot of different types of sarcoma, and they can start in any part of the body. Some types, like bone sarcoma, may cause pain when they grow and press on nerves or muscles. But most sarcomas don’t cause any pain in the early stages. This is because they often develop in soft, elastic tissues or deep spaces inside the body and can push normal tissue out of the way as they grow.
For this reason, sarcomas are hard to diagnose in the early stages. They can also look a lot like other, more common medical problems, such as a fatty lump (lipoma) or a bruise. So it’s important to see your doctor if you notice any new lump or swelling, especially if it is big or getting bigger.
Sarcomas can be diagnosed with tests that examine the blood, bones, and tissue around it. These include X-rays, CT or MRI scans, and a biopsy. A biopsy is when a small piece of the tumor is removed and examined under a microscope to find out what type of sarcoma it is.
Once a sarcoma has spread to other parts of the body, it’s more difficult to treat because it’s no longer under the control of the body’s immune system. But treatments like chemotherapy, radiation therapy, and targeted drugs can help ease symptoms and slow down the cancer’s growth.
Targeted therapy uses drugs that work in a very specific way to kill cancer cells or block their growth. It’s used along with surgery and other treatments.
If sarcoma cells break away and enter the bloodstream, they can spread to other parts of the body and form new areas of cancer. This is called secondary sarcoma or metastasis.
If sarcoma spreads to the lungs, it can cause breathing difficulties. You might also feel tired or weak, and have a fever. Sometimes the spread of sarcoma causes blood vessels to close up, which can lead to a lack of oxygen in the body, and this can be fatal. If the sarcoma is spreading quickly, it can be very serious and needs to be treated right away.
Many sarcomas grow in soft tissues like muscle, nerves, fat, fibrous tissue or blood vessels. They can grow anywhere in the body but are most common in the arms and legs. They may grow slowly or quickly. If they get too large, they can press on nerves or other tissues and cause pain, a feeling of fullness or breathing problems.
If a lump or swelling appears, it’s important to see your doctor right away. If you have a painless lump in your arm or leg that gets bigger over time, it’s especially important to see a doctor. Even if the lump isn’t painful, it may be a sign of cancer or something else that needs to be checked.
You’ll have a physical exam and your doctor will look at the lump or swelling. They’ll also ask about your past health and any symptoms you have. A history of exposure to chemicals such as vinyl chloride monomer (a chemical used to make some types of plastics) or dioxin, or radiation during treatment for another type of cancer, can increase your risk of getting a sarcoma.
For sarcomas that occur in the bones or chest, you may have an X-ray or CT scan to check for tumors. For a sarcoma that starts in the heart’s soft tissue (cardiomyosarcoma), your doctor will use an echocardiogram, which uses sound waves to take pictures of the inside of your chest. A special kind of echo, called a transesophageal echo, allows your doctor to see the inside of your heart and your lungs more clearly.
Sarcomas can spread when cells break off and travel through the blood to other parts of the body. This can lead to new areas of cancer, called metastases. Sometimes sarcomas spread to the lungs, which can make it harder to breathe.
Surgery can help remove a sarcoma. If it has spread, your doctors may treat you with chemotherapy or other medicines that kill cancer cells as they grow. You may also need radiation or a stem cell transplant to reduce your chances of the sarcoma coming back.
Many people don’t have any symptoms from their sarcoma. If you do have symptoms, they depend on where the tumor is and what type of sarcoma it is. For example, bone sarcomas cause pain and swelling in the arms or legs, but they don’t always hurt. Soft-tissue sarcomas often cause lumps in the arms, legs or torso, but they may be painless. The most common symptom is a growth or lump that doesn’t go away.
You may also notice that the part of your body where the sarcoma is grows or shrinks in size. This can make you feel weak or unable to move that part of your body. Some sarcomas grow very fast and can quickly spread to other parts of the body. If this happens, you may have more symptoms like pain and breathing problems.
The first symptom of soft-tissue sarcoma is usually a painless lump or growth that doesn’t go away. These can be very small, but they can also be very large. Some of these lumps are made from fat cells, but most aren’t. They are sometimes mistaken for benign (not cancer) lumps called lipomas.
If the sarcoma is in the lungs or heart, you may have chest pain or trouble breathing. Some types of sarcomas in the stomach bleed, which can lead to abdominal pain, vomiting or stools with a black appearance.
Some kinds of sarcomas can be caused by genetic mutations or by certain medical conditions. You can reduce your risk of getting sarcoma by not smoking and staying healthy. You should get any new lump or change in a lump checked by your family doctor (GP) or oncologist, especially if it’s big or gets bigger over time.
Your GP or oncologist will ask you about your past health and symptoms. They will then do tests to check if you have a sarcoma. These may include an X-ray or computed tomography (CT). A CT scan uses X-rays and computers to show cross-sectional images of your body. It can help your provider find a sarcoma in the bones or soft tissues of your body. A PET scan can also help your provider find a sarcoma. This test involves injecting you with a small amount of radioactive solution. Then the scanner measures the movement of atoms inside your body, including the cells of any sarcoma.
Many sarcomas don’t cause any symptoms in their early stages. If they do, they can be mistaken for other medical problems. Bone pain, for example, might be dismissed as growing pains.
Doctors diagnose sarcomas by doing a physical exam and taking X-rays or CT scans of the tumor area. They also may do a biopsy.
Chemotherapy is a cancer treatment that uses drugs to kill cancer cells. It may be used alone or in combination with surgery and radiation therapy. Sometimes chemotherapy is given before surgery to shrink the tumor or make it easier to remove. This is called preoperative chemotherapy or neoadjuvant chemotherapy. It can also be given after surgery to destroy any microscopic cancer cells that might remain. This is called adjuvant chemotherapy.
If your sarcoma is in the early stages and has not spread from where it started, surgical removal of the tumor is usually very successful and many people are cured. In some cases, however, the sarcoma can return (recur) and will need to be treated again.
Sarcomas that have spread to nearby tissues are more difficult to treat. If you have a soft tissue sarcoma that has spread to the lymph nodes, your doctor may prescribe a combination of treatments including surgery, radiation, and chemotherapy. In addition, if you have a soft tissue sarcoma, you might be able to benefit from a new drug that targets specific mutations in the DNA of your cancer cells. These are called tyrosine kinase inhibitors, which include larotrectinib (Vitrakvi) and entrectinib (Rozlytrek).
Your doctor will use medical imaging to find the location of your sarcoma and its size. They will then remove a sample of the tissue for testing (biopsy). This helps them know what type of sarcoma you have and its stage. The stage of your sarcoma describes how abnormal the cells look under a microscope and how fast they are growing. The information from the biopsy and other tests is used to develop your treatment plan.
A positron emission tomography (PET) scan is another way that doctors can find malignant tumor cells. For this test, a small amount of radioactive glucose is injected into a vein. Then a scanner takes pictures of your body and makes a picture of where the glucose is being used by the tumor cells. Cancer cells take up the glucose more than healthy cells, so they show up brighter in the picture.
Whether your sarcoma is curable depends on the type, where it is and whether or not it has spread (metastasized). Your treatment plan will be based on these factors. Your doctor will work with you to develop a plan that includes surgery, chemotherapy and radiation therapy. The plan also may include other treatments, such as targeted therapy or clinical trials.
If you have stage I or stage II soft tissue sarcoma, it is often curable. Stage III sarcoma is not always curable, but you can receive treatment to control the cancer and keep it from coming back.
Doctors will use imaging tests and a biopsy to find out the stage of your sarcoma. A biopsy is when your provider removes a sample of the tumor and sends it to a lab for testing. A specialist called a pathologist examines the tissue under a microscope to see what kind of sarcoma you have and what types of treatment might be best.
Chemotherapy uses drugs to kill cancer cells. Your doctor will choose the drug or combination of drugs that is right for you based on the type and stage of your sarcoma, where it is in your body, and what your previous treatments have been. You might be able to take some of these drugs by mouth or through an IV infusion.
Radiation therapy uses high-powered energy beams, such as X-rays or protons, to destroy cancer cells. You might have this treatment in a machine that moves around your body (external beam radiation) or in your arm or leg through a temporary implant. A doctor who specializes in giving radiation treatment for sarcomas is called a radiation oncologist.
Targeted therapy drugs treat sarcomas by targeting specific proteins that help cancer cells grow. Bevacizumab, for example, stops tumors from making new blood vessels that feed them, essentially cutting off their food supply. The side effects of these drugs vary, and your doctor will tell you what to expect.
For soft tissue sarcoma, radiation therapy may be used alone or in combination with other treatment options. It uses high-powered energy beams, such as X-rays and protons, to kill cancer cells. It can be given from outside the body (external beam radiation), from a machine that moves around you and delivers radiation to different parts of your body in short bursts, or through a special device placed inside the body temporarily, called brachytherapy. City of Hope experts specialize in image-driven radiation planning, which is the use of medical imaging to help “sculpt” the radiation delivery and limit exposure to normal tissues nearby.
Surgery is an important part of treating sarcoma, especially for tumors that have not spread from where they began (localized). Your surgeon will remove the cancerous tissue and some surrounding healthy tissue. Our Surgical Oncologists and Orthopedic Oncologists have extensive experience in performing sarcoma surgery. They are known for their commitment to preserving normal tissue and their innovative approaches that can reduce pain, blood loss, hospital stay and recovery time.
Many sarcomas are not cured, but treatment can often control the disease. Our specialists work together to develop a treatment plan tailored to your specific needs. They may recommend clinical trials that test new treatments for sarcoma.
Some types of sarcoma can be treated with targeted therapy, which uses drugs to target specific molecules on the surface of cancer cells or inside them that send signals to make them grow faster than they should. These drugs, called kinase inhibitors, can help prevent the growth of sarcomas and may be combined with chemotherapy or radiation therapy.
Your care team will discuss your options and answer any questions you have. They will also review the results of your biopsy and scans and explain what to expect based on the type of sarcoma you have, how advanced it is and whether it has spread from its original site in your body, or metastasized. They will also explain the benefits and risks of each treatment option.
Surgical treatment is often effective for small, low-grade sarcomas. The surgeon’s goal is to remove the tumor and enough surrounding tissue to get a “clean margin” — a border of healthy tissue all the way around the cancer. This reduces the chance that the sarcoma will come back. But it’s important to follow up with regular surveillance scans because sarcomas have a tendency to grow and spread again even years after diagnosis and surgery.
The type of surgery depends on the location, size and grade of the sarcoma, and whether it has spread (metastasized). The surgeon will also perform a biopsy to collect cells for lab testing. These tests can tell the doctor how aggressive the sarcoma is and which treatments might be most effective.
A biopsy may be done with a needle inserted into the skin or during surgery to remove the tumor. Sometimes the tissue is taken with a piece of equipment called a resectoscope, which enables the surgeon to remove large amounts of tissue with minimal damage to nearby structures.
Some sarcomas that occur in the arms or legs might need to be removed through an incision in the skin (surgery). But this is very rare because surgeons are now able to remove larger tumors without having to amputate the arm or leg. The surgery is called limb sparing surgery.
Chemotherapy and radiation therapy might be used before surgery to shrink the tumor and make it easier to remove. They might also be used after surgery to kill any cancer cells left behind. The radiation might be delivered by a machine that moves around your body (external beam radiation) or it might be given through a tube placed in the body temporarily (brachytherapy).
Some types of sarcoma have specific gene mutations that can be targeted with drugs that block certain proteins needed for growth or cell division. These drugs are sometimes called chemo-agnostic therapies. Two drugs, larotrectinib (Vitrakvi) and entrectinib (Rozlytrek), have been approved for use in sarcomas that have a certain gene fusion, but more research is needed to find more drugs that might target different sarcoma mutations.
There are more than 50 different types of sarcoma. They start in bones and connective tissue (soft tissues). Some types are more common in children. Others are more common in adults.
Your provider will tell you the stage of your sarcoma. The staging information describes how much the cancer has grown locally and if it has spread to other organs.
Leiomyosarcomas are cancers of the smooth muscle tissue that lines hollow organs such as blood vessels, the bladder, stomach and uterus. This type of sarcoma is very rare and often occurs in the uterus (which is called uterine leiomyosarcoma). Leiomyosarcoma can also occur in the heart and blood vessels and other parts of the body.
These tumors grow and spread quickly. They are usually found in the legs or arms, but can be found anywhere in the body. The most common treatment is surgery to remove the cancer and some surrounding healthy muscle and tissue. Radiation and chemotherapy can also be used, but these treatments are less effective against this particular type of sarcoma.
Fibrosarcoma usually develops in middle-aged or older adults. It most often starts in deep fibrous tissue in the arms or legs, the trunk of the body or the head and neck. It may also occur in tissues that were treated with radiation for another condition.
Your doctor will use tests to find out if you have leiomyosarcoma and what type it is. Then your doctor will talk with you about your treatment options.
Healthcare providers stage sarcoma based on how much the tumor has grown and spread and how the cells look under a microscope. The stages range from 1 to 4, with higher numbers indicating more advanced cancer.
After surgery, you will need follow-up care. Your doctor will do regular checkups and may give you medicine to keep the sarcoma from coming back. You can also join a clinical trial to try new medicines that are being tested for treating soft-tissue sarcoma/leiomyosarcoma. Your doctor can help you find a trial that is right for you.
Hemangioendothelioma is a rare group of mesenchymal vascular neoplasms that can be either benign or malignant and occurs around medium to large venous structures. It is a tumor of the mesenchyme and has four subtypes: epithelioid hemangioendothelioma, kaposiform hemangioendothelioma, and retiform hemangioendothelioma. It may affect the head, neck, lungs, abdominal lymph nodes, spleen, stomach, retroperitoneum and heart. It is usually symptomatic at the time of diagnosis and patients are typically referred for evaluation because of an enlarging mass.
Surgical resection, radiotherapy and chemotherapy are the standard treatment of hemangioendothelioma. Symptoms vary depending on the location of the neoplasm, but include weight loss, pain and swelling in the affected area. Some cases of hemangioendothelioma are very slow-growing and are not associated with any symptoms at all. Other cases have a more rapid growth rate and may cause more serious complications.
The prognosis of hemangioendothelioma depends on the histopathologic characteristics, including mitotic count and cellularity. However, the relationship between these features and clinical behavior remains unknown. This study analyzed the clinical course of 137 patients with EHE of the liver to determine whether any variables might predict tumor behavior.
The authors found that the prognosis of EHE was poor, and the overall survival was just 4 months after diagnosis. In addition, most of the patients in this series had metastatic disease. The most common site of metastasis was the lungs, followed by the abdominal lymph nodes and spleen. Some patients also had a local recurrence of the tumor, with two cases causing death. Other sites included the heart, testis, kidney and pancreas.
Infantile fibrosarcoma is a malignant soft tissue tumor that usually appears at birth or within the first year of life. This type of sarcoma is more common in children under five years old, but it may also occur in older children and adolescents between 10 and 15 years old. It is usually slow-growing and rarely metastasizes. It is often misdiagnosed because it shares histological characteristics with benign tumors of infancy and childhood. Local recurrence is reported in 7-34 percent of cases.
The treatment of infantile fibrosarcoma depends on the age and location of the tumor. The most common treatment is surgical resection and chemotherapy. Complete surgical resection is usually curative in children with this type of tumor. It is important to ensure that the patient has access to an experienced musculoskeletal oncologist.
During surgery, the tumor is removed and looked at under a microscope. This will help determine the stage of the disease and if it has spread. A biopsy is also taken to test for the presence of cancer cells. Other tests can include CT scans, MRI, radioisotope scans and bone marrow aspiration.
In addition to surgery and chemotherapy, patients with infantile fibrosarcoma need to follow up regularly with their health care providers. Regular visits can help prevent the tumor from coming back and causing more damage. In some cases, the tumor may return and require additional treatment, such as radiation or surgery. In these situations, the child will need to visit a specialist who has experience treating children with fibrosarcoma. This can help prevent the disease from recurring and damaging other organs. This condition is rare, but it is important to get prompt diagnosis and treatment to improve the child’s quality of life.
Symptoms of undifferentiated pleomorphic sarcoma may include long-lasting bone pain or swelling in the arms or legs. It’s also possible for this cancer to spread to other areas of the body, such as the lungs. The severity of symptoms depends on where this cancer develops, how large it grows and what type of cells it contains.
Researchers don’t know what causes this type of cancer, but people who have had other soft tissue sarcomas or radiation therapy in the past seem to have a higher risk for developing it. Typically, this type of sarcoma occurs in people over the age of 50. It’s also more common in men than women.
To diagnose undifferentiated pleomorphic cancer, your healthcare provider will ask you about your medical history and perform a physical examination. They may order one or more imaging tests, such as a computed tomography (CT) scan, magnetic resonance imaging (MRI) scan or positron emission tomography (PET) scan. They will also take a biopsy (a sample of tissue).
A specialist will examine the tissue under a microscope to determine what type of sarcoma you have and what its grade is. The lower the grade, the more normal the tumor cells look. The healthcare provider will use this information to help plan your treatment and predict your prognosis.
When undifferentiated pleomorphic (UPS) cancer is found early, it is less likely to spread and is easier to treat. But it’s important to keep up with follow-up appointments so that the doctor can monitor you for any changes in your condition. This can include the appearance of new, unexplained lumps and the development of new symptoms like fever or fatigue.
Dermatofibrosarcoma protuberans is a low-grade cutaneous sarcoma that rarely metastasizes. It often begins as a small, firm patch of skin that is reddish or purplish in color and grows slowly. It is usually found on the torso, but can also occur on the arms, legs, and head. In some cases, it develops into a raised nodule or lump. The prognosis for dermatofibrosarcoma protuberans depends on the location, size, and thickness of the tumor. Patients with a larger tumor and thicker layer of tissue have a higher risk of local recurrence than those with smaller tumors and thinner skin layers.
The disease is rare and a high index of suspicion is necessary to make the diagnosis. The tumor has a tendency to recur locally but has low metastatic potential. Aggressive surgical resection with widely negative margins is essential to treatment. Radiotherapy can also be useful, especially in cosmetically sensitive locations.
DFSP is associated with a genetic mutation in the p53 gene that results in the formation of a fusion transcript that encodes COL1A1-PDGFB. It has a characteristic morphology and immunohistochemical profile that distinguishes it from other soft-tissue sarcomas.
The incidence and survival of dermatofibrosarcoma protuberans are poorly understood because it is such a rare tumor. A recent study examined data from 18 registries in the Surveillance, Epidemiology, and End Results program to determine the incidence of the disease and its overall survival rates. The authors used the standardized mortality ratio to calculate the chance of death from this type of tumor. They found that DFSP has a lower risk of spread than Kaposi’s Sarcoma, malignant fibrous histiocytoma (MFH), and sebaceous carcinoma (SC). The survival rate is very high in 98 percent of the cases when these growths are treated with Mohs surgery.