All Topics  
Pigmented villonodular synovitis

 

 
   Email Print
   Bookmark   Link
 

 

Pigmented villonodular synovitis
 
 
  Topics Pigmented villonodular synovitis Topic Home

Pigmented villonodular synovitis (PVNS) is a rare, benign
Benign

A benign tumor is a tumor that lacks all three of the malignant properties of a cancer. Thus, by definition, a benign tumor does not grow in an unlimited, aggressive manner, does not invade surrounding tissue , and does not metastasize....
 condition that affects the synovium
Synovium

Synovial membrane is the soft biological tissue that lines the non-cartilage surfaces within joints with cavities . The word "synovium" comes from a Latin word meaning "with egg ," because the synovial fluid in joints that have a cavity between the bearing surfaces is like egg white....
 of joints, bursae, and tendon sheaths
Tendon

A tendon is a tough band of fibrous connective tissue that usually connects muscle to bone and is capable of withstanding tension . Tendons are similar to ligaments except that ligaments join one bone to another....
. The etiology
Etiology

Etiology is the study of Causality. The word is derived from the Ancient Greek , aitiologia, "giving a reason for" .The word is most commonly used in medical and philosophical theories, where it is used to refer to the study of why things occur, or even the reasons behind the way that things act, and is used in philosophy, physics, psy...
, or cause, of this proliferative disorder is unknown. Two forms of the disorder exist: localized and diffuse, the latter being more common.

ented villonodular synovitis, described distinctly in 1941, comes in two forms: localized and diffuse.






Discussion
Ask a question about 'Pigmented villonodular synovitis'
Start a new discussion about 'Pigmented villonodular synovitis'
Answer questions from other users
Full Discussion Forum



Encyclopedia


Pigmented villonodular synovitis (PVNS) is a rare, benign
Benign

A benign tumor is a tumor that lacks all three of the malignant properties of a cancer. Thus, by definition, a benign tumor does not grow in an unlimited, aggressive manner, does not invade surrounding tissue , and does not metastasize....
 condition that affects the synovium
Synovium

Synovial membrane is the soft biological tissue that lines the non-cartilage surfaces within joints with cavities . The word "synovium" comes from a Latin word meaning "with egg ," because the synovial fluid in joints that have a cavity between the bearing surfaces is like egg white....
 of joints, bursae, and tendon sheaths
Tendon

A tendon is a tough band of fibrous connective tissue that usually connects muscle to bone and is capable of withstanding tension . Tendons are similar to ligaments except that ligaments join one bone to another....
. The etiology
Etiology

Etiology is the study of Causality. The word is derived from the Ancient Greek , aitiologia, "giving a reason for" .The word is most commonly used in medical and philosophical theories, where it is used to refer to the study of why things occur, or even the reasons behind the way that things act, and is used in philosophy, physics, psy...
, or cause, of this proliferative disorder is unknown. Two forms of the disorder exist: localized and diffuse, the latter being more common.

Classification

Pigmented villonodular synovitis, described distinctly in 1941, comes in two forms: localized and diffuse. Diffuse PVNS affects the entire synovium and typically occurs in large joints such as the knee
Knee

----The knee is the lower extremity joint connecting the femur, patella, and the tibia and the surrounding anatomical region which includes the popliteal fossa, also known as "knee pit"....
 or hip. Localized, or nodular, PVNS is less common than the diffuse form and typically occurs in smaller joints such as the hands and feet
Foot

The foot is an anatomical structure found in many animals. It is the terminal portion of a limb which bears weight and allows locomotion. In many animals with feet, the foot is a separate organ at the terminal part of the leg made up of one or more segments or bones, generally including claws or nails....
. It often arises in the form of a benign giant cell tumor
Tumor

A tumor or tumour is the name for a swelling or lesion formed by an abnormal growth of cells . Tumor is not synonymous with cancer. A tumor can be Benign neoplasm, Carcinoma in situ or malignant, whereas cancer is by definition malignant....
 on the tendon sheaths of the joint. As the tumor grows in the joint, it damages the surrounding bone and tissues. Localized PVNS is predominantly found in females and is frequently found in the fingers. Although rare, localized PVNS may develop in large joints. In either case, the knee is the most commonly affected joint (80% of cases), followed by the hip, and less commonly the ankles and shoulders. PVNS is generally found more in men than women.

Clinical presentation

In general, pigmented villonodular synovitis often manifests initially as sudden onset, unexplained joint swelling and pain; the joint swelling is disproportionate to the amount of pain the patient feels at first. Decreased motion and increased pain occur as the disorder progresses as well as locking of the joint. The localized form often manifests initially as a painless, slow-growing mass and progresses to the other common symptoms of PVNS. The swelling often feels warm to the touch. Diffuse PVNS symptoms are often confused with those of Rheumatoid arthritis
Rheumatoid arthritis

Rheumatoid arthritis is a chronic, systemic disease inflammation that may affect many tissues and organs, but principally attacks the joints producing a inflammatory synovitis that often progresses to destruction of the articular cartilage and ankylosis of the joints....
. While pigmented villonodular synovitis can occur in both pediatric and geriatric patients, it is more common with ages 20-50.

Complications

PVNS is locally aggressive and can spread to surrounding tissues, causing bone erosion
Erosion

For morphological image processing operations, see Erosion 'For use of in dermatopathology, see Erosion Erosion is the removal of solids in the natural environment....
 and tissue damage. If not treated early, it can spread to areas outside the joint. The disorder also has, on average, a 45% rate of recurrence.

Diagnosis and treatment

PVNS is radiologically diagnosed by Magnetic Resonance Imaging (MRI). The disorder is difficult to identify and is often not diagnosed for four years or more after presentation due to nonspecific symptoms or a general paucity of symptoms. Once PVNS is confirmed by biopsy of the synovium of an affected joint, a synovectomy of the affected area is the most common treatment. Bone lesions caused by the disorder are removed and bone grafting is performed as needed. Because diffuse PVNS has a relatively high rate of recurrence, radiation therapy
Radiation therapy

Radiation therapy is the medicine use of ionizing radiation as part of cancer oncology to control malignant cell s . Radiotherapy may be used for curative or Adjuvant chemotherapy cancer treatment....
 may be considered as a treatment option. In some cases, a total joint replacement is needed to relieve symptoms when PVNS causes significant joint destruction.

See also

  • Giant cell tumor of the tendon sheath
    Giant cell tumor of the tendon sheath

    Giant cell tumor of the tendon sheath is a firm lesion, measuring 1 to 3cm in diameter, and is most commonly attached to the tendons of the fingers, hands, and wrists, with a predilection for the flexor surfaces....