Pigmented villonodular synovitis (PVNS)

  • Overview

    Pigmented villonodular synovitis (PVNS) is a type of benign (non-cancerous) tumor that arises from the soft connective tissue of joints. Any joint can be affected, although the tumor is most commonly found in the knee or hip joint. Although it can be painful, PVNS usually does not spread. It does, however, have a high rate of recurrence after surgery.

    • PVNS usually has a good outcome because it is usually not considered an aggressive tumor.
    • It is sometimes completely cured through surgery, although it recurs in about half of all cases.
    • It is rare in children.
    • The cause is unknown.

    How we approach pigmented villonodular synovitis

    Dana-Farber/Children’s Hospital Cancer Center provides comprehensive medical and surgical care for children and adolescents with PVNS.

    We understand that you may have a lot of questions when your child is diagnosed with an PVNS. Is it dangerous? Will it affect my child long-term? What do we do next? We’ve tried to provide some answers to those questions in the following pages, and our experts can explain your child’s condition fully. If you have further questions during your hospital stay, our experts can answer your questions fully.

    Our multidisciplinary approach to care ensures that your child’s case will be given thoughtful discussion by an integrated care from a team that includes the following specialists:

    • 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

    In addition, our cancer center offers the following services:

    • Expert diagnosis by pathologists using advanced molecular diagnostic testing to identify your child’s type of tumor. Knowing the molecular composition of a tumor helps predict which treatments are more likely to work.
    • Access to unique Phase I clinical trials, from our own investigators, and from the Children’s Oncology Group.
    • Expert surgical care from experienced pediatric surgeons and orthopaedic surgeons, several of whom developed approaches used at centers across the country. 
    • Support services to address all of your child and family’s needs.
    • A weekly survivorship clinic, which set the national standard for childhood cancer survivorship care. This weekly clinic offers ongoing care to manage late effects caused by your child’s cancer or the treatment she’s received.

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

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


  • In-Depth

    What is pigmented villonodular synovitis?
    Pigmented villonodular synovitis (PVNS) is a type of benign (non-cancerous) tumor that arises from the soft connective tissue of joints. Any joint can be affected, although the tumor is most commonly found in the knee or hip joint. Although it can be painful, PVNS usually does not spread. It does, however, have a high rate of recurrence after surgery.

    What causes pigmented villonodular synovitis?
    The exact cause of PVNS is unknown.

    Is pigmented villonodular synovitis common?
    PVNS is rare in children and mostly affects young adults.

    What are the symptoms of pigmented villonodular synovitis?
    While symptoms may vary child-to-child, the most common include:

    • swelling of a joint (usually painless)
    • joint effusion (fluid, usually bloody), in the joint
    • pain (sometimes)
    • sometimes limping or difficulty using legs, arms, hands or fee
  • Tests

    How does a doctor know that it’s pigmented villonodular synovitis?
    Diagnostic procedures for pigmented villonodular synovitis 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 to measure your child’s 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 produces detailed images of organs and structures within the body and/or spine
    • Arthography -- a special x-ray of the affected joint that involves the injection of a radiopaque substance directly into the affected area
  • Dana-Farber/Children's Hospital Cancer Center is home to some of the world's most skilled pediatric oncologists. And while we're known for our science-driven approach to treating conditions like red blood cell disorders, our doctors never forget that your child is a child, and not just a patient.

    We specialize in innovative, 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.

    Traditional treatments for pigmented villonodular synovitis
    Treatment options will vary greatly, depending on your child's situation, and your child's doctor will discuss the options with you in-depth. Treatment may include:

    Surgery
    Since PVNS can continue to grow and invade the bone, the treatment of choice is usually an operation called a synovectomy, in which the affected synovial tissue is surgically removed.

    Radiation
    Our doctors use precisely targeted and dosed radiation to kill cancer cells left behind after your child's surgery. This has been used successfully to control recurrence of PVNS, but is generally avoided in children because of growth issues and the concern about radiation associated malignancy later in life.

    What is the recommended long-term care for children treated for pigmented villonodular synovitis?

    Children treated for pigmented villonodular synovitis 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
  • Research & Innovation

    There are many ways in which your child might benefit from our medical research program. Our doctors and scientists have made many breakthrough discoveries about diseases like polio and leukemia; our ongoing innovative research continues to push the boundaries of the way pediatric medicine is practiced.

    It’s possible that your child will be eligible to participate in one of our current clinical trials. These studies are useful for a multitude of reasons:

    • Some trials are designed to evaluate the effectiveness of a particular drug, treatment or therapy on a specific disease; others help doctors to better understand how and why certain conditions occur. At any given time,
    • We have hundreds of clinical trials underway. Of course, your motives as a parent needn’t be entirely altruistic—you’ll naturally want to know how taking part in a trial can immediately benefit your child. If your child’s physician recommends participation in one of Children’s clinical trials, that likely means that your child’s physician believes that the plan outlined in that trial represents the absolute best, latest care your child can possibly receive.

    And participation in any clinical trial is completely voluntary: We will take care to fully explain all elements of the treatment plan prior to the start of the trial, and you may remove your child from the medical study at any time.

    To search current and upcoming clinical trials at Children’s, go to:
    http://www.childrenshospital.org/research/clinical/Search.cfm

    To search for a cancer trial at Dana Farber/Children’s Hospital Cancer Center, go to:
    http://www.dana-farber.org/Apps/clinical_trials/search.aspx (under Pediatric).     

    To search the NIH’s list of clinical trials taking place around the world, go to:
    http://www.clinicaltrials.gov/ct2/search

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