Pigmented Villonodular Synovitis (PVNS)

What are the symptoms of PVNS?

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 feet

What causes PVNS?

PVNS is rare in children and mostly affects young adults. The exact cause of PVNS is unknown.

How is PVNS diagnosed?

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
• arthrography, a special x-ray of the affected joint that involves the injection of a radiopaque substance directly into the affected area

The Dana-Farber/Boston Children's 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.

What are the treatment options for PVNS?

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 with PVNS?

Children treated for PVNS 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

How we approach pigmented villonodular synovitis

Dana-Farber/Boston Children’s 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, 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 orthopedic 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.