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Hemangiopericytoma

  • Overview

    Hemangiopericytoma is a rare tumor that grows in the body’s soft tissue, which includes fat, muscles, tendons, nerves, blood vessels and other fibrous tissue.

    • In a baby, this condition is called "infantile myofibromatosis," and the tumors typically go away on their own.
    • In adolescence, the tumor can be benign or malignant; it’s often found in bones, but can spread to the teenager’s lungs.
    • In the United States, about 900 children and adolescents are diagnosed with soft-tissue tumors each year.

    The latest research from Dana-Farber/Children's Hospital Cancer Center

    Dana-Farber/Children's Hospital Cancer Center researchers are conducting numerous research studies that will help clinicians better understand and treat soft-tissue sarcomas. Innovative types of treatment currently being studied include:

    • Angiogenesis inhibitors — substances that may be able to prevent the growth of tumors by blocking the formation of new blood vessels that feed the tumors.
    • 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.


    Boston Children's Hospital 
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  • In-Depth

    What causes hemangiopericytoma?

    We don’t know exactly, but studies have indicated that genetic alterations may play a role in the formation of all soft-tissue sarcomas (tumors). In addition, limited studies have shown a possible link between soft-tissue sarcomas and the development of other types of cancer.

    What are the symptoms of hemangiopericytoma?

    Because hemangiopericytoma affects tissue that is elastic and easily moved, a tumor may exist for a long time before being discovered, growing large and pushing aside surrounding tissue.

    Each child may experience symptoms differently. The symptoms can vary greatly depending on size, location and spread of the tumor. Here’s a list of some of the symptoms your child may be experiencing:

    • a painless swelling or lump
    • pain or soreness
    • limping or other difficulty using her legs or feet
    • hypoglycemia (low blood sugar)

    The symptoms can be different in tumors that affect the lungs:

    • cough (with or without blood)
    • pain
    • night sweats
    • difficulty breathing
  • Tests

    How is hemangiopericytoma diagnosed?

    In addition to a complete medical history and physical examination, the most conclusive diagnostic procedure for hemangiopericytoma is a biopsy—a single tissue sample taken from the tumor through a simple surgical procedure.

    The tumor's cellular appearance under a microscope enables doctors to distinguish it from other types of cancer and determine whether it is malignant or benign. Your child will likely have various imaging studies that will include one or more of the following:

    • X-ray —X-rays are useful in the diagnosis of bone tumors and frequently allow physicians to distinguish benign from malignant bone tumors. It is the first diagnostic study, and often gives your doctor information regarding the need for further testing.
    • Bone scans — A nuclear imaging method used to detect bone and metastatic tumors. Bone scans can determine if there are abnormalities in other bones. This test does not distinguish between tumor, infection or fractures.
    • Magnetic resonance imaging (MRI) —This test outlines the extent of the tumor within the bone and joint and the relationship of the tumor to the muscles, nerves and blood vessels.
    • Computerized tomography scan (also called a CT or CAT scan) — A CT scan shows detailed images of any part of the body, including the bones, muscles, fat, and organs. CT scans are more detailed than general x-rays. It is used primarily to assess the chest and lung for metastatic tumors.
    • Your child’s doctor may also ask for a complete blood count and other blood chemistry tests that can provide further help in making a diagnosis.

    Staging

    If your child’s doctor determines that the hemangiopericytoma is malignant, the tumor is staged. This process indicates how far the tumor has spread from its original location.

    • The stage of a tumor and how aggressive the tumor is suggests which form of treatment is most appropriate, and gives some indication of the prognosis.
    • A hemangiopericytoma may be localized, meaning it hasn’t spread beyond the joint where it arose or beyond nearby tissues, or metastatic, meaning it has spread to the lungs, bones or to other organs or structures of your child’s body.
  • What's the treatment for a hemangiopericytoma?

    You'll discuss specific treatment for your child's tumor with your doctor. We've described some your treatment options below.

    Surgery

    Surgery includes biopsy and surgical removal of the entire tumors, and surrounding healthy tissue. Depending on the location and size of the tumor, it may be necessary to remove all or part of the limb. In most cases, we perform limb-sparing surgery to avoid amputation.

    Limb-sparing surgery

    It is sometimes necessary to remove all or part of a limb. In most cases, however, limb-sparing surgery is used to avoid amputation.

    • Through limb-sparing surgery, all of the bone and cartilage involved with the tumor, including some degree of muscle surrounding it, is removed, while nearby tendons, nerves and vessels are saved.
    • The bone that is removed is replaced with a bone graft or with a metal prosthesis.
    • Subsequent surgery may be needed to repair or replace rods, which can become loose or break.

    Patients who have undergone limb-sparing surgery need intensive rehabilitation. It may take as long as a year for your child to regain full use of a limb following limb-sparing surgery.

    Rarely, patients who undergo limb-sparing surgery may eventually have to have the limb amputated because of a severe complication or tumor recurrence.

    Amputation

    If your child's orthopedic surgeon determines that the tumor cannot be removed because it involves important nerves and blood vessels, amputation is the only surgical option.

    During the operation, doctors ensure that muscles and skin form a cuff around the amputated bone. As the swelling decreases, (10 to 14 days), your child will be fitted for a plastic, temporary socket and prosthesis, which is used for two to four months until her stump is healed sufficiently to accept a permanent artificial limb.

    The advantages of an amputation are that it is a simple operation with minimal chances of surgical complication and it definitively removes the local tumor. The functional outcome is good, since children can use modern prostheses. Children often also use "immediate-fit" prostheses applied in the operating room.

    Although your child will probably have a limp with above-the-knee amputations, the procedure is functional and stable.

    She will be able to walk, climb stairs, swim (with the prosthesis on or off) and participate in many sports such as skiing, basketball, baseball and tennis, although running will be limited.

    Radiation

    This is sometimes used in conjunction with surgery for hemangiopericytoma, either before or after removal of the tumor if it is malignant. On rare occasions, radiation alone is used for treatment of the primary tumor.

    Chemotherapy

    If the tumor is malignant, chemotherapy may be part of your doctor's recommended treatment.

    Chemotherapy is a drug treatment that works by interfering 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 (directly to the bloodstream)
    • intrathecally (directly into the spinal column with a needle)

    Rehabilitation

    Rehabilitation includes physical and occupational therapy along with psychosocial counseling.

    Supportive care

    Supportive care includes any type of treatment to prevent and treat infections, side effects of treatments, and complications, and to keep your child comfortable during treatment.

    Continual follow-up care

    A schedule of follow-up care will be determined by your child's physician and other members of your care team to monitor ongoing response to treatment and possible late effects of treatment.

    What is the long-term outlook for a child with hemangiopericytoma?

    The prognosis for your child greatly depends on:

    • the extent of the disease
    • the size and location of the tumor
    • presence or absence of metastasis
    • the tumor's response to therapy
    • the age and overall health of your child
    • your child's tolerance of specific medications, procedures, or therapies
    • new developments in treatment

    Every child is unique and treatment and prognosis is structured around your child's needs. Prompt medical attention and aggressive therapy are important for the best outcome.

    What is the recommended long-term care for children treated for hemangiopericytoma?

    Your child 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
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