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Alveolar Soft Part Sarcoma

  • The diagnosis of a rare cancer, like alveolar soft part sarcoma, is especially distressing for parents. Not only does this soft tissue tumor require your child to get intensive treatment, it may also be difficult to find accurate information on it. The experts at the Dana-Farber/Children's Hospital Cancer Center are here to help. 

    An important first step in understanding what lies ahead is familiarizing yourself with the basics about alveolar soft part sarcoma (ASPS): 

    • ASPS is a very rare cancer in children.
    • It may take a long time before you notice any symptoms in your child.
    • It starts in soft connective tissues of the body, such as fat, muscles or nerves.
    • ASPS is a slow-growing tumor and usually starts in the legs or arms, although it can also start growing in the head and neck.
    • The tumor can spread to other parts of the body and can come back many years later.
    • The cause of ASPS is unknown, but may be connected to genes.
    • ASPS is a serious condition requiring surgical removal of the tumor and, in some cases, chemotherapy and radiation. Once the tumor is removed, children need to ongoing follow-up care because the tumor can return. 

    The detailed information on the following pages will help you gain a better understanding of ASPS and a clearer picture of what to expect.

    How Dana-Farber/Boston Children’s Cancer and Blood Disorders Center approaches alveolar soft part sarcoma

    Here at Dana-Farber/Boston Children’s Cancer and Blood Disorders Center, we offer the ideal setting, utilizing world-recognized cancer expertise at Dana-Farber and internationally renowned pediatric expertise at Children’s Hospital Boston within the research and educational setting of Harvard Medical School. We are a top cancer center and top children’s hospital. 

    Children with ASPS are treated through Dana-Farber/Boston Children’s Cancer and Blood Disorders Center Bone and Soft Tissue Tumor Program. Kids who need surgery are also treated through Children’s Orthopedic Center

    Our multidisciplinary approach to care ensures in-depth discussion of each case and personalized treatment plans for every child. We integrate expertise from the following specialists: 

    • pediatric oncologists, surgical oncologists and radiation oncologists
    • pediatric experts from every medical subspecialty, such as orthopedics, pathology, physical therapy and radiology
    • highly skilled and experienced pediatric oncology nurses
    • Child Life specialists, psychologists, social workers and resource specialists who provide supportive care before, during and after treatment 

    At Dana-Farber/Boston Children’s Cancer and Blood Disorders Center, we’re conducting research studies that will help clinicians better understand and treat children with soft tissue sarcomas. 


    Alveolar soft part sarcoma: Reviewed by Megan Anderson, MD, orthopedic surgeon
    © Boston Children’s Hospital , 2011

    Contact Us

    Boston Children's Hospital
    300 Longwood Ave
    Fegan 2
    Boston MA 02115 

    617-355-6021
    fax: 617-730-0456 

  • We understand that you may have a lot of questions when your child is diagnosed with alveolar soft part sarcoma, such as: 

    • What exactly is it?
    • What are potential complications in my child’s case?
    • What are the treatments?
    • What are possible side effects from treatment?
    • How will it affect my child in the long term?

    We’ve provided some answers to those questions here, and when you meet with the experts at the Dana-Farber/Children's Hospital Cancer Center, we can explain your child’s condition and treatment options fully.

    What is alveolar soft part sarcoma?
    Alveolar soft part sarcoma (ASPS) is a malignant soft tissue tumor, a rare type of cancer that starts in soft connective tissues of the body such as fat, muscles or nerves. ASPS is slow-growing and usually starts in the legs or arms, although it can also be found in the head and neck. It can spread to other parts of the body and tends to come back years later.

    Is alveolar soft part sarcoma common?
    No. As a group, soft tissue sarcomas (other than rhabdomyosarcoma), account for less than 1 percent of all new cancer cases each year and 3 percent of all childhood tumors. This particular type of tumor is very rare among children.

    Is it curable?
    In some cases, ASPS can be cured with a combination of surgery, chemotherapy and radiation therapy. Unfortunately, however, this tumor can often return after treatment, even many years later.

    Causes

    What causes alveolar soft part sarcoma?
    The exact cause of alveolar soft part sarcoma is not entirely understood; however, studies have indicated that specific genetic defects may play a role.

    Signs and symptoms

    What are the symptoms of alveolar soft part sarcoma?
    Alveolar soft part sarcoma affects the body’s soft tissues, which are elastic and easily moved. This means a tumor may exist for a long time before being discovered and can grow very large and pushing aside surrounding tissue. 

    While symptoms may vary child-to-child, the most common include: 

    • a painless swelling or lump
    • pain or soreness caused by compressed nerves or muscles
    • limping or other difficulty using the legs and feet
    • diminished range of motion in the affected area

    It’s important to understand that the symptoms of an alveolar soft part sarcoma may resemble other medical problems, some of them which are quite common and easy to treat, others which could be more serious.

    Your child may experience symptoms differently. Therefore, it’s important that he is evaluated by a doctor to get an accurate diagnosis. Always consult your child's physician if you have concerns.


    Questions to ask your doctor

    You and your family are key players in your child’s medical care. It’s important that you share your observations and ideas with your child’s health care provider, and that you understand your doctor’s recommendations.
     
    If your child is has been diagnosed with an alveolar soft part sarcoma, you probably have a lot on your mind. So it’s often helpful to write questions down. Some of the questions you may want to ask include: 

    • What does a diagnosis of a alveolar soft part sarcoma mean for my child?
    • How will you manage my child’s symptoms?
    • What kind of surgery will my child have?
    • How long will recovery take?
    • What are the possible short and long-term complications of treatment?
    • What is the long-term outlook for my child?
    • How likely is it that the tumor will come back?
    • What services are available to help my child and my family cope?

    FAQ

    Q: How serious is alveolar soft part sarcoma?
    A:
    Alveolar soft part sarcoma is a malignant tumor that can spread to other parts of the body. It is a serious condition requiring surgical removal of the tumor and chemotherapy and radiation in some cases. Once the tumor is removed, children need to receive ongoing follow-up care to monitor for recurrence. Unfortunately, this type of tumor can often return after treatment, even years later. 

    Q: Will my child be OK?
    A: Your child’s long-term health depends on a number of factors, including the size and location of the tumor and whether or not the tumor has spread. In general, alveolar soft part sarcomas that have spread are much more difficult to treat. Unfortunately, ASPS can also come back after surgery. Your doctor will discuss treatment options with you and your family, including clinical trials and supportive care.

    Q: Will my child need chemotherapy or radiation?
    A:
    Possibly, depending on her individual circumstances. The primary treatment for ASPS is surgery. However, chemotherapy and/or radiation may also be used in some cases. 

    Q: Where will my child be treated?
    A: Children with ASPS are treated through our joint Dana-Farber/Children’s Hospital Cancer Center. 

    • Children treated through our Bone and Soft Tissue Tumors Program receive inpatient (overnight) at Boston Children’s Hospital and outpatient (day) care at Dana-Farber Cancer Institute.
    • If your child needs surgery, he will see doctors in Boston Children's Orthopedic Center

    Q: What services are available to help my child and my family cope?
    A: We offer several support services to help you, your child and your family get through the challenges and stresses of dealing with your child’s illness. 


  • The first step in treating your child is forming an accurate and complete diagnosis. 

    How does my child’s doctor know that it’s alveolar soft part sarcoma?
    Here at Dana-Farber/Boston Children’s Cancer and Blood Disorders Center, your child's doctor may use several tests and procedures to determine the exact type of tumor and whether it has spread. These tests may include: 

    • a physical exam, including checking the limb where the tumor is located
    • x-rays, which produce images of internal tissues, bones, and organs onto film
    • magnetic resonance imaging (MR or MRI), which produces detailed images of the area where the tumor is located
    • computerized tomography scan (CT or CAT scan) to capture a detailed view of the body, in some cases
    • biopsy or tissue sample from the tumor to provide definitive information about the type of tumor; this is collected during surgery
    • bone scan to detect bone involvement
    • complete blood count (CBC), which measures size, number and maturity of different blood cells in a specific volume of blood
    • other blood tests, including blood chemistries 

    After we complete all necessary tests, our experts meet to review and discuss what they have learned about your child's condition. Then we will meet with you and your family to discuss the results and outline the best treatment options.


  • We know how stressful a diagnosis of alveolar soft part sarcoma can be, both for your child and for your whole family. That's why our physicians are focused on family-centered care: From your first visit, you'll work with a team of professionals who are committed to supporting all of your family's physical and psychosocial needs. We'll work with you to create a care plan that's best for your child.  

    Your child's physician will determine a specific course of treatment based on several factors, including: 

    • your child's age, overall health and medical history
    • the location and size of the tumor
    • whether the disease has recurred 

    Treatment for alveolar soft part sarcoma can vary greatly, depending on your child's unique situation. Your child's doctor and other members of your care team will discuss the options with you in-depth. 

    Traditional treatments for alveolar soft part sarcoma
    Surgery is the most common and effective treatment for most soft tissue sarcomas, particularly for alveolar soft part sarcoma that has not spread. These tumors tend to be unresponsive to chemotherapy.

     Depending on the size and location of the tumor, your child may either need: 

    • limb-salvage surgery to help preserve the limb by removing the tumor and some healthy tissue around the tumor, or
    • amputation, which may be necessary if the tumor cannot be removed (for example, if it involves the nerves and blood vessels). 

    In addition to surgery, your child may need other types of treatment, including:

    Radiation therapy
    Our doctors use precisely targeted and dosed radiation to kill cancer cells left behind after your child's surgery. Radiation can also be given before surgery in order to shrink the tumor or in place of surgery to try to control tumor growth if surgery is not possible. 

    Arterial embolization
    There is potential for blood loss during an operation to remove an alveolar soft part sarcoma because of the abnormal blood vessels that may be involved. As a result, surgery involves very careful planning. 

    • In some cases, before you and your child's treatment team decide on surgery, a radiologist may perform a procedure called arterial embolization. This blocks the blood flow in the abnormal vessels that are involved with the tumor. This reduces the risk of bleeding during surgery.
    • To determine whether your child can undergo arterial embolization without complications, your doctor may use an angiogram, a special x-ray that helps doctors to visualize the involved blood vessels.

    Chemotherapy
    In some cases, chemotherapy may be used to treat an alveolar soft part sarcoma. Chemotherapy is class of drugs that interferes with the cancer cell's ability to grow or reproduce. 

    • Different groups of chemotherapy drugs work in different ways to fight cancer cells and shrink tumors.
    • Often, a combination of chemotherapy drugs is used.
    • Certain chemotherapy drugs may be given in a specific order depending on the type of cancer it is being used to treat. 

    While chemotherapy can be quite effective in treating certain cancers, the agents don't differentiate normal healthy cells from cancer cells. Because of this, there can be many adverse side effects during treatment. Being able to anticipate these side effects can help the care team, parents, and child prepare, and, in some cases, prevent these symptoms from occurring, if possible. 

    Chemotherapy is systemic treatment, meaning it is introduced to the bloodstream and travels throughout the body to kill cancer cells. Chemotherapy can be given: 

    • as a pill to swallow
    • as an injection into the muscle or fat tissue
    • intravenously (IV), directly to the bloodstream
    • intrathecally, directly into the spinal column with a needle

    Supportive care

    The term “supportive care” refers to any type of treatment to prevent and treat infections, side effects of treatments and complications, and to keep your child comfortable during treatment. It is an important part of our care for all children receiving cancer treatment. 

    What is the recommended long-term care regimen for a child treated for alveolar soft part sarcoma?

    Children treated for alveolar soft part sarcoma should visit a survivorship clinic every year to: 

    • manage disease complications
    • screen for early recurrence of cancer
    • manage late effects of treatment

    A typical follow-up visit may include some or all of the following: 

    • a physical exam
    • laboratory testing
    • imaging scans 

    Through the David B. Perini, Jr. Quality of Life Clinic at Dana-Farber Cancer Institute, childhood cancer survivors receive a comprehensive follow-up evaluation from their cancer care team. 

    • Our childhood cancer survivorship clinic is held weekly.
    • In addition to meeting with your pediatric oncologists, your child may see one of our endocrinologists, cardiologists, neurologists, neuro-psychologists or alternative/complementary therapy specialists.
    • We also offer the following services:
      • patient and family education
      • psychosocial assessment
      • genetic counseling
      • reproductive and fertility evaluation and counseling
      • opportunities to speak with other childhood cancer survivors


    Coping and support

    In addition to providing medical care, the Dana-Farber/Boston Children’s Cancer and Blood Disorders Center strives to also meet all of your family's emotional and quality-of-life needs.

    We know that unfamiliar places, especially hospitals, often create anxiety and fear for a child. We also know this isn't an easy time or task for parents, who have their own fears and concerns about the situation. Our family support services will help address many of your needs and concerns.

    Read more about our resources and support services. 


  • At Dana-Farber/Boston Children’s Cancer and Blood Disorders Center, our care is informed by our research. We have a long and distinguished history of research and innovation in pediatric orthopedics. 

    Clinical and basic scientists at Boston Children's Hospital and Dana-Farber Cancer Institute are conducting numerous research studies that will help us better understand and treat soft tissue sarcomas. 

    • We are studying angiogenesis inhibitors, substances that may be able to prevent the growth of tumors.
    • We are investigating the use of biological therapies, a wide range of substances that may be able to involve the body’s own immune system to fight cancer or lessen harmful side effects of some treatments.
    • Researchers are looking into different chemotherapy drugs that may prove effective for children with alveolar soft part sarcoma. 

    Children’s is a world leader in opening new avenues of "translational research," bringing laboratory advances to the bedside and doctor's office as quickly as possible. All of our senior medical staff members of the Bone and Soft Tissue Tumors Program participate in clinical research activities. Our program director, Carlos Rodriguez-Galindo, MD, is also the co-director of our new Clinical and Translational Investigation Program, which aims to dramatically improve translational research in pediatric hematology and oncology.

    In addition to a variety of clinical trials, Children’s also conducts extensive laboratory research to identify new treatments and improve therapies for benign and malignant bone and soft tissue tumors. 

    Learn more about Boston Children's Hospital's groundbreaking research enterprise.


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