Pigmented Villonodular Synovitis

PVNS Treatment Experts in Pune

We treat Pigmented Villonodular Synovitis at Sancheti Hospital’s Ortho Onco Department with advanced and minimally invasive care.

Overview

Pigmented Villonodular Synovitis, commonly called PVNS, is a rare disease that affects the joints. It happens when the synovium – the soft tissue that lines the inside of joints – becomes thick and overgrown. The synovium normally makes fluid that helps joints move smoothly, but in PVNS, this tissue forms an abnormal mass or tumor.

The good news is that PVNS is not cancerous. The tumor is benign, which means it won’t spread to other parts of your body. However, it can be locally aggressive and may cause serious damage to the affected joint if not treated properly.

PVNS usually affects only one joint at a time. The knee is most commonly involved, accounting for about 66% to 88% of all cases. The hip is the second most common location, followed by the ankle, shoulder, and elbow.

Symptoms

PVNS symptoms often develop slowly over time, which can make diagnosis challenging. Many people experience symptoms for years before getting the correct diagnosis. The most common signs include:

  • Joint pain that may come and go
  • Swelling in the affected joint
  • Stiffness or reduced range of motion
  • Joint instability or feeling like the joint might give out
  • Locking or catching sensations in the joint
  • Warmth and tenderness around the joint
  • Sometimes a soft tissue mass that can be felt

The joint fluid in PVNS often appears bloody or brownish due to repeated bleeding inside the joint. This happens because of iron deposits that build up over time.

Because these symptoms are similar to other common joint problems like arthritis or meniscus tears, PVNS is often misdiagnosed initially. If you have persistent joint pain or swelling in just one joint without a clear injury, it’s important to see a doctor.

Causes

The exact cause of PVNS is still unknown. Medical researchers have several theories about what might trigger this condition:

  • Chronic inflammation within the joint
  • Previous trauma or injury to the joint (though this connection isn’t proven)
  • Genetic changes that cause abnormal cell growth
  • Neoplastic process – evidence suggests PVNS may be a tumor-forming process involving specific chromosome changes
  • Unknown factors that cause abnormal reactions in the joint

Recent research points to PVNS being related to overproduction of a protein called colony-stimulating factor 1 (CSF1), which can lead to abnormal cell clusters and overgrowth of synovial tissue.

Risk Factors

PVNS is extremely rare. Here are the key risk factors:

  • Age: Most commonly diagnosed in young adults between 20 and 50 years old
  • Gender: Studies show mixed results, with some suggesting equal rates between men and women, while others indicate slightly higher rates in women
  • Family history: PVNS doesn’t appear to run in families
  • Occupation or activities: No specific jobs or activities have been linked to increased risk

Currently, there are no known definitive risk factors that can predict who will develop PVNS.

Diagnosis

Diagnosing PVNS can be challenging because its symptoms mimic other joint conditions. An orthopedic specialist typically leads the diagnostic process using several approaches:

Physical Examination: Your doctor will check the affected joint for appearance, range of motion, swelling, and tenderness.

Imaging Tests:

  • X-rays: May appear normal in early stages but can show swelling or bone changes in advanced cases
  • MRI: The most important test for diagnosing PVNS. It shows thickened synovium and characteristic iron deposits that appear dark on the scan
  • CT scan: Helps assess bone involvement and damage

Joint Aspiration: Removing fluid from the joint with a needle. The fluid often appears bloody or brownish in PVNS.

Biopsy: A small tissue sample is taken and examined under a microscope to confirm the diagnosis and rule out cancer.

Types

PVNS is classified into two main types:

Localized PVNS:

  • Affects only a small, specific area of the synovium
  • Appears as a distinct mass with a stalk-like attachment
  • Easier to treat completely
  • Lower chance of coming back after treatment
  • Often found in the front part of the knee

Diffuse PVNS:

  • Spreads throughout most or all of the synovium in the joint
  • More aggressive and harder to treat
  • Symptoms are usually more severe
  • Higher chance of returning after treatment (10% to 50%)
  • Can extend beyond the joint capsule into surrounding tissues

Stages

While PVNS doesn’t have formal stages like cancer, it does progress over time if left untreated:

Early Stage: Few or no symptoms, normal X-rays, might be mistaken for minor joint problems.

Progressive Stage: More noticeable symptoms including pain, swelling, and limited movement. Iron deposits become visible on MRI.

Advanced Stage: Severe joint damage, bone damage, cartilage destruction, and significant osteoarthritis. Can lead to major disability and difficulty walking.

Early diagnosis and treatment are crucial to prevent progression and minimize long-term joint damage.

Treatment

The main treatment for PVNS is surgery to remove the tumor and affected synovium. Treatment options include:

Surgical Options:

  • Synovectomy: Surgical removal of the diseased synovium
  • Arthroscopic synovectomy: Minimally invasive surgery using small incisions and a camera
  • Open synovectomy: Traditional surgery with larger incisions for extensive disease
  • Total joint replacement: May be needed if the joint is severely damaged

Additional Treatments:

  • Radiation therapy: Often used after surgery to prevent recurrence
  • Drug therapy: Newer medications like pexidartinib may help in cases where surgery isn’t possible
  • External beam radiation: Directed from outside the body
  • Radiosynoviorthesis: Radioactive fluid injected directly into the joint

The choice of treatment depends on the type and extent of PVNS, as well as the patient’s overall health.

Rehabilitation

Recovery from PVNS treatment, especially surgery, requires dedicated rehabilitation:

Physical Therapy: Essential for regaining strength and range of motion. A physical therapist will design specific exercises for your needs.

Activity Modifications:

  • May need crutches or other assistive devices initially
  • Gradual return to normal activities over several months
  • Weight-bearing restrictions may apply, especially for knee or hip surgery

Pain Management: Anti-inflammatory medications can help control pain and swelling during recovery.

Follow-up Care: Regular check-ups and imaging studies to monitor for recurrence.

Complications

If left untreated, PVNS can cause serious complications:

Joint Damage:

  • Bone damage and erosion
  • Cartilage destruction
  • Severe osteoarthritis
  • Joint deformity and limited mobility
  • Significantly reduced quality of life

Treatment Complications:

  • Recurrence of the disease (especially with diffuse type)
  • Surgical complications including stiffness and scarring
  • Radiation therapy side effects
  • Drug therapy side effects, including liver problems with some medications

Rare Complications:

  • Extremely rare cases of malignant transformation (becoming cancerous)
  • Spread to distant sites in very rare malignant cases

Prevention

Currently, there are no known ways to prevent PVNS because its exact cause is unknown. Since it doesn’t appear to be related to specific activities, injuries, or lifestyle factors, prevention strategies haven’t been established.

Living With PVNS

Living with PVNS requires ongoing management and monitoring:

Key Management Strategies:

  • Seek early medical attention for joint symptoms
  • Follow your treatment plan carefully
  • Attend all follow-up appointments
  • Stay active with appropriate physical therapy
  • Make necessary lifestyle adjustments

Long-term Monitoring: Regular check-ups are essential, especially for diffuse PVNS, due to its high recurrence rate. This typically includes physical exams and periodic MRI scans.

Team Approach: The best outcomes often result from a healthcare team including orthopedic surgeons, radiologists, oncologists, and physical therapists working together.

Quality of Life: With proper treatment and management, many people with PVNS can maintain good function and quality of life.

Key Takeaways

  • PVNS is a rare disease involving benign but locally aggressive overgrowth of joint lining tissue
  • The knee is most commonly affected, followed by the hip
  • Symptoms include joint pain, swelling, and stiffness that develop gradually
  • MRI is the most important diagnostic tool for identifying PVNS
  • Two main types exist: localized (easier to treat) and diffuse (more aggressive)
  • Surgical excision is the primary treatment, often combined with radiation therapy
  • Recurrence is possible, particularly with diffuse PVNS
  • Early diagnosis and treatment are crucial for preventing joint damage

At Sancheti Hospital, we understand the challenges that come with rare conditions like PVNS. Our experienced team of orthopedic specialists, radiologists, and rehabilitation professionals work together to provide comprehensive care for patients with PVNS. We utilize advanced diagnostic imaging including high-resolution MRI to accurately diagnose the condition and determine the best treatment approach. 

Our surgical team is skilled in both arthroscopic and open synovectomy procedures, ensuring that each patient receives personalized treatment based on their specific type and extent of PVNS

We also provide comprehensive rehabilitation services and long-term follow-up care to monitor for recurrence and ensure optimal outcomes. Our multidisciplinary approach ensures that patients receive not just excellent medical care, but also the support and guidance needed to manage this complex condition effectively.

Meet Our Ortho Onco Specialist

Patient Stories & Experiences

Vinita Singh
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The pain in my left knee left me feeling helpless for years. After my treatment here, I can finally say I'm pain-free.

Vinita Singh

Parvati
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The nerves were swollen, and the body went numb. But thanks to Sancheti Hospital, I got a second life!

Parvati

Balaji Kharat
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I finally could walk again, a relief I've only felt after the hip pain surgery. I thank the doctors at Sancheti Hospital for their help.

Balaji Kharat

Shantilal
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I'm a police officer, and I'm extremely thankful to Sancheti Hospital for treating my fracture without surgery.

Shantilal

Kalpana Lepcha
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My life has completely changed after the knee replacement surgery at Sancheti Hospital. It's like I can finally live again!

Kalpana Lepcha

Karuna
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The knee pain I've carried for years finally went away with the help of Sancheti Hospital.

Karuna

Kishore Bhosle
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I can't believe that I get to finally live a normal and happy life, all thanks to the knee surgery I had at Sancheti Hospital.

Kishore Bhosle

Frequently Asked Questions

How long does it take to recover from PVNS surgery?

Recovery time varies depending on the type of surgery. Arthroscopic procedures typically require 6-8 weeks, while open surgery may take 3-6 months. Most patients can return to normal activities within 4-6 months with proper rehabilitation.

Yes, most patients need 2-4 weeks off for desk jobs and 6-12 weeks for physical jobs. The exact time depends on your surgery type, which joint is affected, and your job requirements. Your doctor will provide specific work restrictions.

Most patients can return to low-impact activities like swimming and cycling within 3-6 months. High-impact sports may be restricted depending on joint damage and recurrence risk. Your doctor will create a personalized activity plan based on your recovery.

Without treatment, PVNS progressively destroys joint cartilage and bone, leading to severe osteoarthritis. This can cause chronic pain, permanent joint deformity, and significant disability. Early treatment prevents these complications and preserves joint function.

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