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Sarcoma/Connective Tissue Oncology Information

Sarcoma: An Overview

Information Guide: Adult Sarcoma

Information Guide: Sarcomas of the Bone(Pediatric)

Information Guide: Soft Tissue Sarcoma(Pediatric)

Information Guide: Wilm's Tumor (Pediatric)

See Also:

Sarcoma-Questions & Answers (pediatric)

Wilm's Tumor-Questions & Answers (pediatric)
Clinical Trials


Sarcoma: An Overview


Introduction

"Sarcoma" is a term used to describe a whole family of cancers that arise in the body's connective tissues, which include fat, muscle, blood vessels, deep skin tissues, nerves, bones, and cartilage.

Sarcoma is broken down into two types:

soft tissue tumors and bone tumors.


Bone sarcomas
are rare types of cancer that mainly affect children and young adults. There are several types of bone sarcomas that typically affect different parts of bones and joints. The cancerous tumors can grow in any bone in the body, however, most occur in the arms or legs.

The most common bone sarcomas include:

    • Osteosarcoma (tumor usually develops in the ends of long bones where new bone tissue forms),
    • Ewing's sarcoma (tumor usually develops in the middle of large bones such as pelvis, thigh, upper arms and ribs), and
    • Chondrosaroma (found mainly in adults, this type of tumor forms in the cartilage that cushions joints).

Bone sarcomas account for only 0.2% of all cancers in the United States. There are approximately 2500 new cases a year. Bone tumors have a higher incidence of spreading to other parts of the body, especially the lungs so extra tests are taken to determine if spread of the disease has occurred.

Patients diagnosed with bone sarcomas find the terms related to the disease to be very confusing. Without a good understanding of the treatment plan presented to you by your doctor it is difficult to cope with your new diagnosis of cancer.

This page will first give you a better understanding of what "cancer" is in general and then it will take you through the steps of diagnosing and treating bone sarcomas, here at the U-M Comprehensive Cancer Center.

Throughout the many steps in the treatment plan you will encounter different types of doctors to help manage your care such as an orthopedic surgical oncologist, medical oncologist and a radiation oncologist. This multidisciplinary team will meet and discuss your case with a pathologist and a radiologist to come up with an individualized treatment plan for you.


Soft tissue sarcomas
come in many forms (see also Bone Tumors):

  • angiosarcoma (blood vessels
  • fibrosarcoma (connective tissue)
  • gastrointestinal stromal tumor (digestive system)
  • Kaposi's sarcoma (skin)
  • liposarcoma (fat)
  • leiomyosarcoma (smooth muscle)
  • malignant fibrous histiocytoma (connective tissue),
  • neurofibrosarcoma (nerves)
  • rhabdomyosarcoma (skeletal muscle) and
  • synovial sarcoma (often near joints, but can occur anywhere).

Soft tissue sarcomas account for only 1% of all cancers in adults. There are approximately 7000 new cases a year in the United States.

The most common sites of origin are the extremities (legs and arms), however sarcomas can arise in any part of the body - including the abdomen, pelvis and head/neck region.

Patients diagnosed with soft tissue sarcomas find the terms related to the disease to be very confusing. Without a good understanding of the treatment plan presented to you by your doctor it is difficult to cope with your new diagnosis of cancer.

This web page will first give you a better understanding of what "cancer" is in general and then it will take you through the steps of diagnosing and treating soft tissue sarcomas.

Throughout the many steps in the treatment plan you will encounter different types of doctors to help manage your care such as an orthopedic surgical oncologist, surgical oncologist, medical oncologist and a radiation oncologist. This multidisciplinary team will meet and discuss your case with a pathologist and a radiologist to come up with an individualized treatment plan for you.

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What is Cancer?

One of the most confusing aspects about being told you have sarcoma is that doctors and other health care providers use terms such as cancer, malignancy, tumor, growth, lump, and nodule. It is difficult for patients to sort out what all of these terms mean.

Cancer is a form of abnormal cell growth in tissues of the body. Under normal circumstances, as we grow and age the individual cells in the tissues of the body replace themselves through a regular and organized process of cell growth. When the cells of an organ grow out of control, the resulting tissue may form a lump of abnormal cells called a nodule or tumor. This is a growth of tissue made up of cells that are no longer functioning in a normal way.

Here are some definitions to help explain the terms used to describe soft tissue growths that might occur due to abnormal cell growth.

Growth: A non-specific term that describes a collection of tissue that is growing in a way that is different from normal tissue. Growths can be benign (such as a cyst or wart) or they can be cancerous.

Tumor: A lump of tissue that is growing out of control. Tumors can be benign (not a threat to life) or they can be cancerous.

Malignant tumor:
A rapidly growing tumor made up of abnormal cells.

Another term for a malignant tumor is cancer. A malignant tumor will first grow only in the area where it started. This is known as the primary tumor. The primary tumor cells may spread to other tissues or organs through the bloodstream or lymph system. Once the tumor cells have spread to other parts of the body, they will take root and start to grow other tumors. This process is called metastasizing. The new tumor name in other parts of the body is called metastatic tumor.

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Diagnosing of Your Sarcoma

Most commonly, bone sarcoma patients present with a pain in the area of the lesion. In more advanced lesions, patients may notice swelling in the area or present with a fracture of the affected bone. A complete patient history is extremely important when diagnosing bone tumors.

Physical Examination
A physician will do a complete examination of the painful area to evaluate the surrounding skin, blood vessels, nerves or bone and any surrounding lymph nodes.

Radiological Imaging (X-rays and scans)

  • Plain X-ray Films:
    Standard x-rays are taken to evaluate the bone. They can show tumor location. Usually more detailed imaging such, as a MRI or CT scan is needed to further evaluate. Chest x-rays are also useful to evaluate for metastatic tumors in the lung.
  • Magnetic Resonance Imaging (MRI):
    This is one of the most useful methods to image tumors in the extremities. It provides the physician with a detailed view of the tumor and its surroundings.
    • Computed Tomography (CT scan):
      This is another way to image a tumor. Especially useful in tumors which are in the abdomen and pelvis.

      CT scan of the chest is also frequently recommended to assess for metastases of sarcoma, or disease which might have traveled to the lungs.
  • Positron Emission Tomograhy (PET scan):
    This is a newer study done in Nuclear Medicine, which looks at the metabolic activity of cells in the body. Tumor cells often have very active metabolism and therefore can be seen on a PET scan. However, other conditions involved increased metabolism (including healing scars) sometimes show up on PET scans as well. PET scans are occasionally used for sarcoma patients, but usually are not as useful as CT and MRI scans.
  • Bone Scan:
    This is study done in nuclear medicine, which uses radioactive dye to highlight the bones. This scan is sometimes used to look for cancer in other parts of the bone that has metastasized.

NOTE: Each physician chooses which method of imaging they prefer to give them the best view of the primary tumor and any other tumors, which may be present.

Biopsy
A biopsy is the only way to tell for sure if you have a malignant tumor and, if so, what kind of cancer it is. Because sarcomas are rare, a specialist in sarcoma pathology generally reviews the results of the biopsy before any treatment starts. Tumor location determines what type of biopsy will be used.

Generally two types of biopsies can be used:

  • Core needle biopsy:
    This procedure is less invasive than the other method described next. It can be done in the office by your surgeon using local anesthesia. The surgeon will numb the area around the tumor and extract a small amount of tissue from the tumor with a special biopsy needle for the pathologist to examine.
  • Incisional biopsy:
    A small incision (about an inch) is made, and tissue is removed for examination. This is a more invasive procedure and is most often done in the operating room under either local or general anesthesia. The advantage of this procedure is that is provides more tissue for the pathologist to review.

NOTE: Once the pathologist has tissue from a biopsy, the type of soft tissue sarcoma and how aggressive it is can be determined.



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Treatment of Your Sarcoma 

Once your physicians know the type of bone sarcoma, the location of the primary tumor and if there are any metastatic tumors in the body, all the physicians who are involved in the multidisciplinary discussion of your case can come up with a plan for treatment.

Remember: the order of treatment is determined by the size of the tumor and its location, as well as whether there is metastatic tumor anywhere else in the body.

Surgery
Surgery is considered to be the primary treatment for bone sarcomas, and for some, is the only treatment.

The first goal of the orthopedic surgeon is to remove the tumor, along with some healthy tissue around it. This ensures that no cancer cells remain.

The second goal is to retain as much function as possible to the operative site. Sometimes manmade material is used to replace bone, artificial joints and devices are inserted and sometimes amputation is needed. The involvement of the surrounding tissue, muscle, nerves and blood vessels determine the extent to which that is possible.

Your orthopedic oncologist prior to your surgery will discuss all your options. A thoracic surgeon performs surgery for sarcomas inside the chest or lungs (usually metastatic tumors).

RadiationTherapy
Radiation usually is administered after surgery, and is given to patients who are at risk for local tumor reoccurrence if only surgery was used for control of the tumor locally.

The goal of radiation therapy is to direct additional therapy at the tumor site to kill any remaining cancer cells that could remain after surgery.

The radiation oncologist uses your preoperative x-rays, information from your surgeon and their own treatment plan to determine the region that needs to be treated.

Radiation treatment is given daily Monday through Friday for 5-7 weeks depending on the plan discussed by your radiation oncologist.

Chemotherapy
Many factors influence the use of chemotherapy or drug treatment of sarcoma.

These factors include:

  • the aggressiveness of the tumor or grade
  • the size of the tumor and
  • if the tumor has already spread to other areas in the body.

There are many cases in which chemotherapy is given prior to surgery to help shrink down the primary tumor so the surgeon can fully remove the tumor and maintain function of the affected area. It is also used after surgery to destroy any stray cancer cells and to prevent cancer from recurring.

Chemotherapy is often given in high doses and it can cause you to feel sick at times. The details of the chemotherapy regimen and toxicities (side effects) related to the chemotherapy will be discussed by your medical oncologist and staff. (you can also visit our web page: What to Expect: A Guide to Chemotherapy).

The chemotherapy is given to you in the outpatient clinic or in the hospital and the length of treatment is determined by which regimen your medical oncologist presents to you.

 

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Conclusion

Hopefully this page has helped you better understand your sarcoma and has helped you understand how a treatment plan is formed.

You need to remember that every case is individualized.

Your team of physicians has worked closely together to come up with a plan that will give you the best chance of survival and a good quality of life. They are very willing to answer questions and want to be sure you understand exactly what is being recommended and why.

Please make sure to ask questions of the doctors and staff to be sure you understand all of your options.

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