Sarcomas — malignant or benign tumors that occur in the bone and connective tissues — are made up of more than 50 types and are very rare. Combined, malignant sarcomas account for about 1 percent of all new cases of cancer. Because they are unique in how they present and involve a complex array of treatment options, it is important that patients seek experienced care. According to the National Cancer Institute, sarcomas located in body organs are hard to distinguish from other kinds of cancer and are frequently misdiagnosed, which leads to delays in appropriate treatment.

High-Quality Sarcoma Specialty Care Matters

With prompt and accurate diagnosis and appropriate treatment, many primary sarcomas are curable. Sarcoma outcomes and survivability have been shown to be significantly improved when people are diagnosed and cared for by a team of sarcoma specialists. Sarcoma is all we do — we offer a dedicated Sarcoma Program with a multidisciplinary team of specialists who will ensure your best possible outcome. We are known for our skill in accurate diagnosis and for performing limb-preserving surgery to avoid amputation, an important factor for quality of life and daily functioning.

Simple to Connect to Our Sarcoma Team

If you have been diagnosed with sarcoma, we make it as simple as possible to pursue treatment. Our new patient coordinators will gather your medical records so you don’t have to. They also coordinate appointments, tests, treatments, follow-up care and any support services you may need. We strive to see new patients within one week of the appointment request or doctor’s referral. If the need is urgent, we’ll schedule appointments with specialists same day whenever possible.

Bone Sarcomas, Soft Tissue Sarcomas, Ewing’s Sarcoma, Osteosarcoma, Desmoid Tumors, GIST

Cancers that occur in the bone and connective tissues affect people of all ages, and they can occur at any location throughout the body. They include tumors that develop in bone, muscle, tendons, nerves, blood vessels and fat. These tumors, also called musculoskeletal tumors, can also be benign.

Common sarcomas include:

Sarcoma originates in the bone, as opposed to metastatic bone disease that spreads from another location to the bone and spine. Sarcoma and metastatic bone disease follow different treatment protocols. 

Sarcoma Diagnosis

An accurate diagnosis is a crucial first step in the effective treatment of bone and soft tissue sarcoma. Patients with suspected bone or soft tissue sarcoma should receive a thorough evaluation and diagnostic workup by sarcoma specialists before beginning any treatment. We see a large volume of sarcoma patients each year, and have the expertise to confirm a cancer that can be difficult to diagnose. The team specializes in minimally invasive biopsy techniques that provide fast, accurate diagnoses of musculoskeletal tumors. The type of tumor, its grade and size, and whether it has spread are all factors that determine the tumor’s stage and your treatment plan.

The program also offers second opinions for a sarcoma diagnosis through the Cancer Second Opinion Program. A second opinion provides patients with the reassurance that a diagnosis is accurate and that they know all of the treatment options available to them.

Sarcoma Treatment Approaches

Treatment for patients in the Sarcoma Program centers on the person and the disease. World-class specialists from multiple disciplines collaborate on individualized plans that are developed to destroy the cancer and to preserve or restore function. 

Treatment planning begins in a multidisciplinary tumor board that focuses solely on sarcoma patients. Physicians and other experts from every specialty involved in sarcoma care gather weekly to review and discuss cases and recommend treatment to destroy cancer and to preserve or restore limb function. They pool their collective experience, knowledge and research findings to arrive at the best approach. In this way, each patient receives completely coordinated care that takes advantage of every appropriate treatment option.

Explore Options with a Second Opinion

No matter where you are in your sarcoma experience — even mid-treatment, we are happy to offer a second opinion to make you aware of treatment choices you may not know you have or to give you peace of mind that you are on the right path. Video visits are available along with in-person appointments.

Clinical Trials May Expand Your Options

Because our sarcoma team is constantly pursuing research to find new ways to detect and treat sarcoma or improve patients’ quality of life, they are knowledgeable about the latest and most effective treatments possible, including sarcoma clinical trials. Patients are often eligible to take part in clinical trials that give them access to new therapies and treatment approaches — some of which are available only through our Cancer Network.

Support Through Sarcoma Treatment

Because you will have different needs during different phases of treatment, we offer many support resources including financial navigation and psycho-oncology experts to help you and your family cope during a stressful time. We also offer a 24-Hour Cancer Clinic for cancer-related problems like pain or high fever that may need immediate attention outside of usual clinic hours.

Sarcoma Treatment FAQ

  • There are no established common risk factors for developing bone or soft tissue sarcomas. It has not been shown that any environmental factors such as water, radon, pollution, living near electrical wires, or any other normal daily exposures place people at increased risk for sarcomas.

    Patients who have had radiation to an area previously are at increased risk for developing sarcomas within the treatment field. In addition, patients who have received certain types of chemotherapy are at increased risk for developing sarcomas. Radiation and chemotherapy-induced sarcomas are rare and tend to happen many years following the initial treatment.

    Certain rare genetic disorders can predispose patients to developing sarcomas. Smoking and other types of tobacco use, and alcohol have not been shown to result in sarcoma formation or to give patients an increased risk for sarcomas. In general, sarcoma development is thought to be a largely unpredictable random event at this time.

  • To our knowledge, sarcomas are not something that can be transferred to another person. Patients do not have to take special precautions with regards to contact with family, friends, or their work environment from a contagious standpoint. There are no restrictions in exposures for patients with sarcoma.

    Sarcomas are not spread through kissing, hugging, or sexual relations of any kind. Patients who are undergoing chemotherapy for their sarcoma may be at risk for contracting other viral illnesses from their surroundings, and the medical oncologist will discuss recommendations with regards to protecting yourself from routine viruses and other illnesses.

  • Prognosis is a word that indicates how doctors expect the patient to do with a particular diagnosis. Each sarcoma patient is an individual, and the prognosis will be different for each person.

    Prognosis is determined by a number of factors. The most important is whether the tumor has shown evidence that it has already spread or metastasized.

    Another prognostic indicator is the size of the tumor, with larger tumors having, in general, a worse prognosis. Most studies on sarcomas categorize large tumors as those that are greater than 5 to 7 cm. in any direction.

    Another important prognostic indicator is how aggressive the tumor is. This is called its grade and is determined by the pathologists after evaluating the biopsy specimen. In general, the grade is based on how rapidly the tumor is dividing and growing, and how abnormal the cells have become.

    Location may also play a role in prognosis. The sub-type of sarcoma can also play an important role. The interdisciplinary team will look at all of these factors in determining the prognosis for an individual patient.

    The prognosis is based on previous studies of sarcoma patients. An example of prognosis would be a patient with a small, (less than 5 cm.) subcutaneous (under the skin) leiomyosarcoma in the forearm. He or she would typically have a greater than 90 percent five year disease free survival. This means if we took 100 patients with a leiomyosarcoma of less than 5 cm. in the subcutaneous tissue of the forearm, we would anticipate that more than 90 of them would still be alive five years after diagnosis without evidence of any disease returning locally or any metastatic tumor that had spread from the local site to other spots in the body.

    Prognosis is typically described in five year and 10 year increments. The vast majority of sarcoma patients who make it beyond five years will remain disease free for the rest of their lives. Overall, five year survival indicates patients who are still alive at five years after their diagnosis. This includes patients who are alive with evidence of disease and patients who are alive without evidence of disease.

  • Musculoskeletal oncologists often have a good idea what type of tumor they may be encountering on imaging scans alone, but, for a definitive diagnosis, a biopsy is almost always necessary. The location and type of biopsy performed is a very complex decision-making process for the physician and can have a tremendous impact on the overall treatment outcome for patients.

    If a sarcoma is introduced to an area outside of the tumor mass, it will grow in that area. This is often called a sarcoma seed wound. Therefore, the surgeon needs to pay particular attention to approaching the biopsy in the correct manner. The goal is to avoid contaminating any of important surrounding structures, so we can preserve as much function as possible for the patient. At the time of definitive resection or removal of the tumor, a margin of normal tissue is removed around the tumor. This will include the entire biopsy tract from the skin down to the tumor mass. The surgeon who plans on completely removing the tumor and treating it definitively should be the one performing the biopsy.

    There are several different types of biopsy that may be selected, depending on your individual case.

  • The most important thing to your providers is that you feel confident in your care and comfortable with your sarcoma team. Undergoing treatment for cancer is one of the most difficult things anyone can go through. Being emotionally committed to your care and your providers is critical in obtaining the best result possible. Because of this, we encourage you to obtain second opinions if you so desire, and we will help you in any way we can.

    We feel very confident that we are providing world-class sarcoma care here. We also understand that there are many different ways to approach some sarcomas. Other institutions may have different preferences with regards to their approach. You should feel comfortable asking about a second opinion and understand that our goal is to get you the best care possible.

    A word of caution regarding second opinions: Patients often become frustrated at the amount of time it takes to obtain a second opinion. Most institutions and physicians require all of the lab and test results to be in their hands before they will even schedule an appointment. Many institutions require a coordinated visit with an interdisciplinary group of oncologists and surgeons, which may result in an appointment that is a number of weeks away.

    Please understand that it is very frustrating for our care providers when this happens, but that we have essentially no control over the way other institutions practice or take care of their patients.

    As a final note, please understand that we are able to provide you with state-of-the-art technologies to treat your sarcoma. If you encounter a sarcoma situation, whether it is a recurrence, metastases, or a relapse, and we feel you could benefit from some treatment at another institution, we will present that opportunity to you and encourage you to pursue that.

  • The length of treatment for sarcoma will depend on many factors. Some patients receive surgery alone. Others receive radiation therapy and surgery. Some patients receive a combination of chemotherapy, radiation therapy and surgery.

    The sarcoma team will help you lay out a treatment calendar to assist in planning and coordinating your care on an individual basis. General guidelines, however, are listed here.

    Patients who receive pre-operative chemotherapy will likely receive a couple of cycles, which take approximately two to three months. This is generally followed by radiation therapy over a five to six week course. Four to six weeks of recovery time follow radiation therapy prior to surgical resection. Following surgical resection, there may be, in rare cases, additional radiation therapy given and possible further cycles of chemotherapy.

  • There are a number of reasons the team may decide not to cut the tumor out right away. Radiotherapy may be recommended before surgery and has been shown to decrease the risk of local recurrence.

    One advantage of pre-operative radiation is the delivery of a smaller radiation dose to a smaller target volume when compared with post-operative radiotherapy. This translates into fewer long-term treatment complications and better function of the extremity, as demonstrated in Phase III randomized clinical trials. Other potential advantages of giving radiotherapy before surgery include shrinking the tumor and reducing the risk of tumor cell seeding at the time of surgery.

    The main concern about pre-operative radiotherapy is the risk of increasing the rate of delayed wound healing when compared with postoperative radiation.

    An additional reason to treat the tumor before surgically removing it is that your team will be able to assess the impact pre-operative treatments have on the tumor. As an example, consider a tumor that is given pre-operative chemotherapy. Once the tumor is removed, it is evaluated by pathologists. Perhaps there has been very little tumor that has been killed by the chemotherapy. If the tumor recurs locally or shows up in a distant site, the medical oncologist would likely try a different type of chemotherapy with different drugs, as opposed to using drugs that did not work as effectively as anticipated the first time.

    In some cases, the tumor may be inoperable or impossible to remove without sacrificing important nerves, blood vessels or muscles. In this situation, pre-operative treatment (chemotherapy and/or radiotherapy) is often recommended in the hope that the treatment may shrink the tumor and make it possible to remove it surgically, while sparing important structures.

  • Surgical side effects will be discussed in detail prior to your surgical procedure by the surgeon. The only person who can appropriately discuss the side effects and complications of a surgical procedure is a member of the surgical team. Every sarcoma surgery is unique, depending on the location, size, or type of sarcoma the patient has. Therefore, each surgery will have its unique set of potential complications and side effects.

    The surgeon and surgical team will discuss many different risks and complications that are possible in your surgery. The vast majority have a minimal risk of actually occurring. The surgeon will likely emphasize side effects he anticipates as a normal course of surgery. Given the unique nature of each sarcoma surgery, some side effects or complications may occur that are unforeseen by the surgical team.

    One of the most common side effects of surgery includes temporary or long-term “chronic” swelling in the affected extremity. This is called edema. The condition may require compression garments or frequent elevation to control. Other side effects include nerve injury resulting in areas of numbness in the skin and/or weakness in the extremity. Some patients may develop chronic nerve pain following surgery.

    Vascular injury or blood vessel damage is an uncommon, but potentially very significant complication of surgery. Your surgeon may need to remove important veins that drain the area of the tumor to achieve an adequate margin. He or she may even need to remove important arteries that will require vascular reconstruction by the plastic surgery, or vascular surgery teams. Removal of vessels will typically increase the risk of chronic swelling. Deep venous thrombosis (DVT) or blood clots in the veins are a possible complication of surgery. Your surgeon will typically prescribe some sort of blood thinning agent following surgery to prevent clots within the veins of the lower extremities. If the risk of bleeding after surgery is too great, the surgeon may need to hold off on administering blood thinning agents. This may increase the risk of blood clots.

    Occasionally, the surgical team will recommend an IVC filter be placed in the large vein draining to the lungs (the inferior vena cava) prior to surgery. This will not affect the risk of deep vein thrombosis, but will prevent clots from traveling to the lungs. Blood clots, or DVTs traveling to the lungs are called pulmonary embolisms (PE); they are a potentially life threatening complication in the peri-operative period (time from surgery until you are released to go home).

    Surgery may result in significant blood loss (hemorrhage) with the need for blood transfusions. There is a very small risk of contracting Hepatitis B, Hepatitis C, or HIV from a donated blood transfusion. In Wisconsin, this risk is currently estimated at well less than a one in a one million chance.

    Any surgery carries the inherent risk of infection. Surgeries that remove large tumors create space that can often fill up with blood or fluid products. An accumulation of serous (clear) fluid is called a seroma. An accumulation of blood is called a hematoma. Seromas and hematomas can increase the risk of infection. Your surgeon may use one or multiple drainage tubes (drains) to help eliminate the fluid from these potential pockets. Plastic surgery may be required to fill in the defect created by removal of the tumor to prevent this problem and minimize the risk of infection.

    There are many different types of infections. A deep infection is the type your surgeon will be most concerned about. Deep infection will likely require a return to the operating room for an irrigation and debridement procedure. At times, patients will get redness and a superficial skin infection called cellulitis following surgery. This is generally treated with antibiotics administered orally or through an IV.

    A common issue, particularly following radiation and surgery, is the occurrence of suture abscesses. Suture abscesses occur when the body reacts to stitches used to close the surgical wound. The incision may open up and try to spit these sutures out four to eight weeks following surgery. This may delay wound healing and may or may not need treatment with antibiotics to minimize the risk for deeper infection.

    Occasionally, a wound will actually open and not heal — a condition called a wound dehiscence. This is more common following pre-operative radiation therapy. It may result in the need to return to the operating room and may require a consultation with a plastic surgeon for tissue transfer.

    Any or all of these complications are considered normal possibilities during the treatment.

    In addition to wound healing problems associated with radiation treatment, there are many other acute or chronic side effects associated with radiation treatment. The most common acute side effects include skin reactions similar to sunburn (skin erythema, swelling and itching), hair loss, muscle aches and joint pain in the radiation field. Chronic side effects include subcutaneous fibrosis, joint stiffness, lymphedema, bony fractures and second primary tumor formation in the radiation field.

    Patients should discuss side effects with the radiation oncologist, since the sarcoma treatment field is individualized. For example, a patient may experience significant nausea and diarrhea if he or she receives radiation to a retroperitoneal sarcoma, since the stomach and bowels are often in this radiation field.

  • It is critically important to adhere to the follow-up schedule prescribed by the sarcoma team. Sarcomas have the ability to show up locally at the site of tumor removal or in distant locations as metastases many years following diagnosis and surgical excision. More aggressive (higher grade) tumors tend to recur and develop metastatic disease more quickly than lower grade (slower growing) tumors.

    Because these tumors can show up in the local resection site or in distant areas, you will require surveillance scans following completion of your treatment regimen. The surveillance scans will need to be performed regardless of specific treatments you have undergone and regardless of the margin status.

    As a general rule, patients have imaging of the chest and possibly abdomen and pelvis with a CT scan to evaluate whether there is metastatic disease. Some patients will be evaluated instead with a PET/CT scan, which is a newer imaging modality. The role of PET/CT for sarcoma has not been completely determined, and many insurance companies do not authorize these very expensive tests.

    The local site of the tumor will be evaluated with a local imaging study. The most common local imaging study is an MRI with gadolinium. Gadolinium is an injection of a material that helps the radiologists and the surgeon differentiate between scar tissue and recurrent tumor.

    When patients have had extensive hardware or metal placed into the local site to reconstruct the bone, it is usually more difficult to get an accurate picture of the area with an MRI. These patients may receive CT scans of the local area or plain radiographs.

    In addition, it is critical for the physician to physically examine the patient to correlate imaging findings. The goal of surveillance scanning and follow-up exam at the local site is to identify tumors that have recurred at an earlier stage when they are easier to remove and before they can spread to other parts of the body. The goal of the CT or PET/CT is to evaluate for metastatic disease and identify tumors while they are still small and there are not many of them.

    In general, patients who develop metastatic sarcoma do not feel any different until the tumors have progressed dramatically. Therefore, it is critical to obtain these imaging studies to catch recurrent or metastatic tumors at an early stage. There are no blood tests we can use to evaluate for sarcoma.

  • Sarcoma care is interdisciplinary and often complex. Many providers will be involved in your treatment and care. Sarcomas are also very rare neoplasms. Most providers in the community do not have a tremendous amount of experience dealing with sarcoma. Sarcomas present unique issues that require special expertise.

    Therefore, in general, it is optimal to follow up with the care providers you have used throughout your course of treatment. However, there will be instances when it is either inconvenient or clearly impossible to follow up our facilities. In those cases, your providers will work very hard to coordinate appropriate care closer to home.

    Imaging studies such as CT scans and MRIs can often be done closer to home. It is imperative, however, that the actual pictures of the images get sent for review by your physicians. In addition, it is critical that the surgeon who performed the surgery examine you on a regular basis, as he or she is the only one who will truly understand the resection and what represents scar tissue, versus recurrent tumor.

    General guidelines for local surveillance scans of sarcoma include an MRI six months following surgical resection of the local site, followed by MRI scans of the local site every four months for two years. At two years, we then switch to MRIs every six month for another three years to take you out to year five after resection.

    For surveillance scans to rule out metastases, we typically recommend CT scans every four months for the first two years, followed by every six months after that. The reason we wait six months following the tumor resection before an MRI as opposed to four months is that it typically takes six months for the soft tissues to heal and give an adequate picture of the post-operative wound bed. The first MRI typically serves as a baseline for comparison for subsequent MRIs. It would be very rare for a tumor to show up at six months.

  • If a tumor returns at the site of the surgical resection, it is called a local tumor recurrence. This is a rare occurrence in the Froedtert & MCW Cancer Network. First, it is important to realize that local tumor recurrence for most sarcomas does not mean the tumor has spread or that it is necessarily going to spread to other sites.

    In general, with a local tumor recurrence, options for treatment are more limited, due to the fact that a near maximal amount of radiation has already been administered to the area. If a tumor recurs outside of the radiation field, radiation could serve as another option. In general, for local recurrence, a wide surgical re-excision is the best and most reliable option. Typically, this involves the resection of a significant amount of tissue and will usually require plastic surgery to fill in the deficit from the resection. There may be other radiation options including brachytherapy or a radiation boost to the area following resection that may be considered by the interdisciplinary team. In general, local recurrence does not result in a need for chemotherapy.

    If your physician suspects your tumor has come back at the local site based on imaging studies or a physical exam, he or she will often recommend a biopsy of the mass, which is often done under image-guidance from a skilled radiologist or as an open procedure from your surgeon.

  • You will be given a considerable amount of information regarding sarcoma. In addition, there are a number of useful Web sites for sarcoma patients.

    We would like to extend a note of caution regarding the Internet and the information that can be obtained there. While there are very good, reliable Web sites available, others can be a source of great misinformation and create significant anxiety for patients. We encourage patients to look for further information regarding sarcoma and ask that you contact a member of the sarcoma team for clarification of any concerning or contradictory information you may discover.

  • The vast majority of patients with sarcoma are not at any increased risk of other cancers. A very small subset of patients has hereditary genetic defects that place them at increased risk for sarcoma and other cancers. For example, the tumor suppressor gene Rb is lost or not functioning in patients with retinoblastoma. These patients are often at greater risk of developing other types of tumors, including osteosarcoma.

    We have genetic counselors available to assist patients in determining if they are at risk for other cancers. In most cases, your treating physician will not deem this necessary.

  • When a tumor shows up at a site distant from the initial tumor, it is called metastatic bone disease. If it appears that there is a mass of concern that has shown up somewhere else in the body, it is possible your providers will order additional testing such as a PET/CT scan. If there is still clinical suspicion, they will likely recommend a biopsy. This will often be done percutaneously via a needle with or without CT guidance.

    Lung nodules or masses may be identified that require consultation with a thoracic surgeon. We have thoracic surgeons who specialize in sarcoma care. They have a tremendous amount of experience in determining the best biopsy options.

    If a diagnosis of metastatic sarcoma is obtained, a number of options are available to the patient. In general, the interdisciplinary group will discuss the patient’s situation, and, depending on the location of the metastatic disease, as well as the extent of it, will recommend various treatment options. These options may include radiation therapy followed by surgical resection, surgical resection alone, radiofrequency ablation or cryoablation by the interventional radiology staff, and chemotherapy which may or may not be used in conjunction with the other treatment modalities.

    It is very possible that the team will recommend starting a couple cycles of chemotherapy to see if there is a response from the tumor, prior to addressing any of the lesions surgically or with radiation therapy. This gives the doctors the chance to see if the drugs they are giving are effective against the sarcoma.

  • The vast majority of our biopsies are done using local anesthesia. We have found that with careful local pain control, we can perform most biopsies quickly and comfortably, without the need for IV sedation or other medications. Any time you eliminate medications from the procedure, you decrease the risk of a medication or allergic reaction. Some cases require sedation and this will be discussed with you before your scheduled biopsy.

    For patients who require IV medications, the patient will need to have gone without food for six hours prior to the procedure. Clear liquids can be had up until two hours before the biopsy. If sedation is given, the patient must have a ride home.

  • Every patient’s unique situation is taken into consideration when deciding upon the need for a biopsy. Some lesions are not palpable or are near structures that make a biopsy without accompanying imaging risky.

    In these cases, imaging guidance is required. Depending upon the location of the lesion, some are better accessed by CT and others by ultrasound. We try to do as many as possible under ultrasound, since there is no radiation exposure. However, not all lesions can be seen well with ultrasound and CT is used in these cases. In some cases, the lesion is not able to biopsied by a radiologist, and for those cases, the biopsy may have to be done in the operating room. Prior to biopsy, the radiologist will discuss with the surgeon the appropriate area of the tumor to sample and the location of the biopsy tract.

  • We try to limit the radiation dose given to any patient at any time. However, the value of CT scanning in detecting, staging, and following disease is so important that the risk of the radiation exposure becomes a secondary concern. This does not mean that you should have CT exams performed without a good reason and the timing and frequency of the exams will depend upon your particular situation. Our radiologists work diligently to minimize the radiation exposure of each exam while ensuring high quality images.

  • A CT scan is currently the best way to look at a large area of the body quickly. An MRI or ultrasound tends to be a more focused exam. To have an MRI that would cover the large area comprising the chest, abdomen, and pelvis would take hours to complete and would still lack a great deal of the information that the CT can provide.

  • Both of these systems use powerful magnets and powerful computers to create and display images of the body. A regular MRI is a tube (called the ‘bore) in which a patient, or part of a patient’s body is placed.

    For people with severe claustrophobia, this can be an issue. The open MRI was created to help with these situations. Open MRI’s are usually open on the sides, but there is still a table you lay upon and equipment above your body. Some severely claustrophobic patients even have trouble with this machine.

    Regular MRI machines tend to be more powerful and the pictures they generate are therefore better. We always recommend a scan be done on a regular MRI for this reason. The open MRI should be reserved for severe claustrophobia, or for those patients who cannot physically fit into a regular MRI.

    Sedation can be given to help with anxiety and claustrophobia prior to an MRI.

Virtual Visits Are Available

Safe and convenient virtual visits by video let you get the care you need via a mobile device, tablet or computer wherever you are. We’ll gather your medical records for you and get our experts’ input so we can offer treatment options without an in-person visit. To schedule a virtual visit, call 1-866-680-0505.