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Malignant Fibrous Histiocystoma

  • A malignant fibrous histiocytoma is a type of cancerous tumor that can start in either bone or, most often, in the soft tissues that connect, support or surround organs and other body parts.Dana-Farber/Boston Children’s Cancer and Blood Disorders Center provides comprehensive medical and surgical care for children and adolescents with benign or malignant bone and soft tissue tumors.

    • It’s a painless mass that most often turns up in the extremities, although it can occur anywhere in the body.
    • It most commonly spreads (metastasizes) to the lungs, but can also invade the lymph nodes and bone.
    • The cause is unknown but has been linked to genetics, radiation treatment and inherited diseases.
    • It’s among the most common types of soft tissue tumors found in adults but is rarely found in children.
    • Treatment may include surgery along with radiation and/or chemotherapy.
    • The tumor can come back.

    How Dana-Farber/Boston Children’s Cancer and Blood Disorders Center approaches malignant fibrous histiocytoma

    Our multidisciplinary approach to care ensures in-depth discussion of each case and personalized treatment plans for every patient. We integrate expertise from the following specialists at Dana-Farber Cancer Institute and Boston Children’s Hospital.  

    • pediatric oncologists, surgical oncologists and radiation oncologists
    • pediatric experts from every medical subspecialty, such as orthopedics, ophthalmology, physical therapy and radiology, among others
    • 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

    We are conducting numerous research studies to help better understand and treat soft tissue and bone sarcomas.

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

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

  • What is malignant fibrous histiocytoma?

    A malignant fibrous histiocytoma is a type of cancerous tumor that can start in either bone or, most often, in the soft tissues that connect, support or surround organs and other body parts. It’s a painless mass that most often turns up in the extremities, although it can occur anywhere in the body. It most commonly spreads (metastasizes) to the lungs, but can also invade the lymph nodes and bone.

    What causes malignant fibrous histiocytoma?

    The exact cause of malignant fibrous histiocytoma is not entirely understood, however, studies have indicated that genetics may play a role. In addition:

    • Scientists are looking into the role of an associated genetic abnormality on the short arm of chromosome 19 that may give rise to this disease.
    • It has also been linked to radiation treatment for breast cancer, retinoblastoma and Hodgkin disease.
    • Malignant fibrous histiocytoma originating in the bone has been linked to pre-existing conditions such as Paget's disease or fibrous dysplasia of the bone.

    Is malignant fibrous histiocytoma common?

    While it’s among the most common types of soft tissue tumors found in adults, it is rarely found in children. It makes up only a tiny percentage of all soft tissue sarcomas, which as a group account for only 1 percent of new cancer cases in children each year.

    What are the symptoms of malignant fibrous histiocytoma?

    Because soft tissue sarcomas affect 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. Symptoms vary greatly with the size, location and spread of the tumor, but may include:

    • painless swelling or mass anywhere on the body
    • pain or soreness caused by compressed nerves or muscles
    • limping or other difficulty using the legs, feet, arms or hands
  • How does a doctor know that it’s malignant fibrous histiocytoma?

    Diagnostic procedures for malignant fibrous histiocytoma are used to determine the exact type of tumor your child has and whether the tumor has spread. These may include a:

    • Physical exam, including neurologic function tests including: reflexes, muscle strength, eye and mouth movement, coordination and alertness.
    • X-rays to produce images of internal tissues, bones, and organs onto film.
    • Magnetic resonance imaging (MRI) to produce detailed images of organs and structures within the body and/or spine.
    • Computerized tomography scan (also called a 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 diseases and tumors as well as to determine the cause of bone pain or inflammation.
    • Complete blood count (CBC), which measures size, number and maturity of different blood cells in a specific volume of blood.
    • Blood tests including blood chemistries
  • Traditional treatments for malignant fibrous histiocytoma

    Treatments for malignant fibrous histiocytoma may involve a combination of therapies including surgery, radiation, and/or chemotherapy. The most common procedures include:

    Surgery

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

    • Limb-salvage surgery helps preserve the limb by removing the tumor and wide margins of healthy tissue surrounding the tumor.
    • Amputation may be needed if the tumor cannot be removed (for example, if it involves the nerves and blood vessels).

    Radiation therapy

    Our doctors use precisely targeted and dosed radiation to kill cancer cells left behind after your child's surgery.

    Chemotherapy

    Chemotherapy is a drug 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 do not 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

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

    What is the recommended long-term care for children treated for malignant fibrous histiocytoma?

    Children treated for malignant fibrous histiocytoma 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
  • Summary

    Boston Children's Hospital and Dana-Farber Cancer Institute are also conducting numerous research studies to help better understand and treat soft tissue and bone sarcomas.

    Research Underway

    Types of treatment currently being studied include:

    • We are studying angiogenesis inhibitors, substances that may be able to prevent the growth of tumors.
    • We are also studying 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 neoadjuvant chemotherapy and radiotherapy, treatments using chemotherapy and radiotherapy before resecting the primary tumor are being investigated.
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