Pigmented villonodular synovitis
Encyclopedia
Pigmented villonodular synovitis (PVNS) is a joint disease characterized by inflammation and overgrowth of the joint lining. It usually affects the hip or knee
. It can also occur in the shoulder, ankle, elbow, hand or foot. In PVNS the lining of the joint, called the synovium
, becomes swollen and grows. This growth harms the bone next to the joint. The lining
also makes extra fluid
that can cause swelling and make movement painful. PVNS is idiopathic
, it doesn't seem to run in families
or be caused by certain jobs or activities. Surgery
can help but, even with treatment, PVNS comes back about half the time. If the pain remains then radiation therapy
may help. In the worst cases the joint must be replaced
. http://www.ejbjs.org/cgi/content/full/80/1/76
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. Localised PVNS often arises in the form of a large benign tumour
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. 2 cases per million population; incidence of the localized form is 9 cases per million.
. While pigmented villonodular synovitis can occur in both pediatric and geriatric patients, it is more common with ages 20–50.
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.
PVNS, under the microscope
, looks as the name of the condition suggests; it is composed on nodules and/or villi and has an abundant number of (pigmented) hemosiderin
-laden macrophage
s.
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
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.
Knee
The knee joint joins the thigh with the leg and consists of two articulations: one between the fibula and tibia, and one between the femur and patella. It is the largest joint in the human body and is very complicated. The knee is a mobile trocho-ginglymus , which permits flexion and extension as...
. It can also occur in the shoulder, ankle, elbow, hand or foot. In PVNS the lining of the joint, called the synovium
Synovium
Synovial membrane is the soft tissue found between the articular capsule and the joint cavity of synovial joints....
, becomes swollen and grows. This growth harms the bone next to the joint. The lining
Synovium
Synovial membrane is the soft tissue found between the articular capsule and the joint cavity of synovial joints....
also makes extra fluid
Synovial fluid
Synovial fluid is a viscous, non-Newtonian fluid found in the cavities of synovial joints. With its yolk-like consistency , the principal role of synovial fluid is to reduce friction between the articular cartilage of synovial joints during movement.-Overview:The inner membrane of synovial joints...
that can cause swelling and make movement painful. PVNS is idiopathic
Idiopathic
Idiopathic is an adjective used primarily in medicine meaning arising spontaneously or from an obscure or unknown cause. From Greek ἴδιος, idios + πάθος, pathos , it means approximately "a disease of its own kind". It is technically a term from nosology, the classification of disease...
, it doesn't seem to run in families
Heredity
Heredity is the passing of traits to offspring . This is the process by which an offspring cell or organism acquires or becomes predisposed to the characteristics of its parent cell or organism. Through heredity, variations exhibited by individuals can accumulate and cause some species to evolve...
or be caused by certain jobs or activities. Surgery
Synovectomy
Synovectomy is the surgical removal of a part of the synovial membrane of a synovial joint.-Indications:It is one of the treatment options for certain diseases involving the synovium like:* Severe rheumatoid arthritis or juvenile idiopathic arthritis...
can help but, even with treatment, PVNS comes back about half the time. If the pain remains then radiation therapy
Radiation therapy
Radiation therapy , radiation oncology, or radiotherapy , sometimes abbreviated to XRT or DXT, is the medical use of ionizing radiation, generally as part of cancer treatment to control malignant cells.Radiation therapy is commonly applied to the cancerous tumor because of its ability to control...
may help. In the worst cases the joint must be replaced
Arthroplasty
Arthroplasty is an operative procedure of orthopedic surgery performed, in which the arthritic or dysfunctional joint surface is replaced with something better or by remodeling or realigning the joint by osteotomy or some other procedure.-Background:Previously, a popular form of arthroplasty was...
. http://www.ejbjs.org/cgi/content/full/80/1/76
Classification
Pigmented villonodular synovitis, described distinctly in 1941 by Charles J. Sutro, L. Lichtenstein, and H.L. Jafe, comes in two forms: localized and diffuse. Diffuse PVNS affects the entire synovium and typically occurs in large joints such as the kneeKnee
The knee joint joins the thigh with the leg and consists of two articulations: one between the fibula and tibia, and one between the femur and patella. It is the largest joint in the human body and is very complicated. The knee is a mobile trocho-ginglymus , which permits flexion and extension as...
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. Localised PVNS often arises in the form of a large benign tumour
Giant cell tumor of the tendon sheath
Giant-cell tumor of the tendon sheath, also known as giant-cell synovioma and localized nodular tenosynovitis, is a firm lesion, measuring 1 to 3 cm in diameter, and is most commonly attached to the tendons of the fingers, hands, and wrists, with a predilection for the flexor surfaces...
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. 2 cases per million population; incidence of the localized form is 9 cases per million.
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 arthritisRheumatoid arthritis
Rheumatoid arthritis is a chronic, systemic inflammatory disorder that may affect many tissues and organs, but principally attacks synovial joints. The process produces an inflammatory response of the synovium secondary to hyperplasia of synovial cells, excess synovial fluid, and the development...
. 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 erosionErosion
Erosion is when materials are removed from the surface and changed into something else. It only works by hydraulic actions and transport of solids in the natural environment, and leads to the deposition of these materials elsewhere...
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
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.Pathology
The synovial fluid of the joint is often grossly hemorrhagic.PVNS, under the microscope
Microscope
A microscope is an instrument used to see objects that are too small for the naked eye. The science of investigating small objects using such an instrument is called microscopy...
, looks as the name of the condition suggests; it is composed on nodules and/or villi and has an abundant number of (pigmented) hemosiderin
Hemosiderin
thumb|Hemosiderin image of a kidney viewed under a microscope. The brown areas represent hemosiderinHemosiderin or haemosiderin is an iron-storage complex. It is always found within cells and appears to be a complex of ferritin, denatured ferritin and other material...
-laden macrophage
Macrophage
Macrophages are cells produced by the differentiation of monocytes in tissues. Human macrophages are about in diameter. Monocytes and macrophages are phagocytes. Macrophages function in both non-specific defense as well as help initiate specific defense mechanisms of vertebrate animals...
s.
Treatment
Once PVNS is confirmed by biopsy of the synovium of an affected joint, a synovectomySynovectomy
Synovectomy is the surgical removal of a part of the synovial membrane of a synovial joint.-Indications:It is one of the treatment options for certain diseases involving the synovium like:* Severe rheumatoid arthritis or juvenile idiopathic arthritis...
of the affected area is the most common treatment. Bone lesions caused by the disorder are removed and bone grafting
Grafting
Grafting is a horticultural technique whereby tissues from one plant are inserted into those of another so that the two sets of vascular tissues may join together. This vascular joining is called inosculation...
is performed as needed. Because diffuse PVNS has a relatively high rate of recurrence, radiation therapy
Radiation therapy
Radiation therapy , radiation oncology, or radiotherapy , sometimes abbreviated to XRT or DXT, is the medical use of ionizing radiation, generally as part of cancer treatment to control malignant cells.Radiation therapy is commonly applied to the cancerous tumor because of its ability to control...
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 sheathGiant cell tumor of the tendon sheathGiant-cell tumor of the tendon sheath, also known as giant-cell synovioma and localized nodular tenosynovitis, is a firm lesion, measuring 1 to 3 cm in diameter, and is most commonly attached to the tendons of the fingers, hands, and wrists, with a predilection for the flexor surfaces...
- Fibroma of tendon sheathFibroma of tendon sheathFibroma of tendon sheath is a cutaneous condition characterized by a benign tumor that presents as a small subcutaneous nodule that slowly increases in size. These lesions are composed of bland myofibroblasts in a dense fibrous background with cleft-like vessels. The tumor often has a multinodular...
- List of cutaneous conditions