Pigmented villonodular synovitis is an uncommon disease characterized by

hyperplastic synovium, large effusions and bone erosions. The disease was first
described as a distinct entity in 1941.1 Since then, many articles in the orthopedic,
radiologic and rheumatologic literature have discussed the radiologic features of
this disease. However, few articles have described its clinical presentation.
Pigmented villonodular synovitis remains a diagnostic challenge. On average, the
disease is not correctly identified until 4.4 years after presentation. 2 The difficulty
stems from the insidious onset and nonspecific presentation of the disease, as well
as its subtle radiographic findings. In addition, the disease can be difficult to
differentiate from conditions such as rheumatoid arthritis, osteoarthritis and other
inflammatory and neoplastic processes of the synovial lining. 3
The family physician plays a crucial role in the early diagnosis and treatment of
pigmented villonodular synovitis. Because patients often present initially with
vague complaints, early diagnosis requires knowledge of the common symptoms
and radiographic features of the disease.

Definition and Etiology

In the original description of the disease, the term pigmented villonodular
synovitis was applied to a lesion that occurred in the synovial membrane of joints
and tendon sheaths and was characterized by fibrous stroma, hemosiderin
deposition, histiocytic infiltrate and giant cells. 1Subsequently, two forms of the
disease were identified: a localized subtype characterized by a pedunculated lesion
and a subtype with diffuse joint involvement. 4
The etiology of pigmented villonodular synovitis remains controversial. 48 The most
widely held theory is that the disease is an inflammatory reaction of the
synovium.1,4 However, some evidence exists that it is a benign neoplastic process. 7

Epidemiology
The incidence of pigmented villonodular synovitis is 1.8 cases per 1 million people
per year, with no environmental, genetic, ethnic or occupational predilection. 9 Most
studies show equal prevalence in males and females, although some investigations
report a slightly greater predilection in males. 9 Pigmented villonodular synovitis
generally occurs in patients between the ages of 20 and 45 years, but it has been
found in patients as young as 11 years and as old as 70 years. 10
The vast majority of patients with pigmented villonodular synovitis have
monoarticular complaints of pain and swelling. Only a few reports of polyarticular
involvement have been published. In both the localized and diffuse subtypes, the
knee is the most commonly affected joint (about 80 percent of patients), 5 followed

by the hip, ankle, small joints of the hands and feet, shoulder and elbow.10 The
presentations of the disease in the knee and hip are somewhat different.

Pathology
Grossly, pigmented villonodular synovitis appears as a proliferative synovial
process with brownish villonodular fronds in the affected joints (Figures 1 and 2).
Histologically, diffuse disease is characterized by a mononuclear stromal cell
infiltrate in the synovial membrane(Figure 3). Hemosiderin-laden macrophages
give the characteristic brown color (Figure 4). Additional cell populations include
foam cells and multinucleated giant cells.
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FIGURE 1.
Intraoperative photograph showing characteristic hypertrophic synovium (arrow) and villonodular fronds in pigmented
villonodular synovitis.

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FIGURE 2.
Gross specimen after total synovectomy.

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FIGURE 3.
Low-power photomicrograph showing a villous formation with mononuclear stromal cells and populations of hemosiderinladen macrophages.

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FIGURE 4.
High-power photomicrograph demonstrating mononuclear stromal cell infiltrate (white arrow) and hemosiderin-laden
macrophages (black arrow).

Pigmented Villonodular Synovitis of the Knee

Diffuse pigmented villonodular synovitis of the knee can mimic many other
conditions. In one study of 25 patients,6 the two most common incorrect working
diagnoses were extensor mechanism malalignment (patellofemoral syndrome) and
meniscal lesion. The disease was also confused with inflammatory arthritis,
ligament instability and other conditions.
Patients with diffuse pigmented villonodular synovitis of the knee complain of the
insidious onset and slow progression of symptoms. One third of these patients
report previous trauma to the knee.2 Patients also tend to complain of intermittent
swelling and stiffness around the joint. Most report diffuse discomfort rather than
severe focal pain (such as occurs with a meniscal tear or fracture). 6 Hence, the
diagnosis of diffuse pigmented villonodular synovitis may be suggested by a
clinical history of swelling that has an insidious onset, is not preceded by trauma
and is out of proportion to the degree of discomfort. 6 Patients do not complain of
knee instability; however, they occasionally report a popping sensation in the
joint.

On physical examination, up to 96 percent of patients have distention of the

suprapatellar pouch and a large effusion.6 As many as 40 percent of patients have a
diffuse palpable synovial mass.6Patients also have a slightly decreased ability to
flex and extend the joint. Up to 90 percent of patients complain of mild to
moderate tenderness, mainly over the medial patellofemoral area. 6In addition,
arthrocentesis yields blood-tinged synovial fluid in 44 to 69 percent of patients. 6,9
IMAGING STUDIES

In patients with pigmented villonodular synovitis of the knee, plain radiographs

often appear normal. However, radiographic findings in diffuse disease can include
a periarticular soft tissue density (in up to 80 percent of patients), expansion of the
suprapatellar pouch and local osseous changes mainly confined to the
patellofemoral articulation11 (Figure 5). The changes at the patellofemoral joint
result from abnormal patellar tracking because of the synovial mass lifting and
stretching the extensor mechanism, thereby allowing shear forces to act on the
articular cartilage. Osteopenia is occasionally found, and degenerative changes
may be detected in 30 to 40 percent of patients. 11 In general, bone and joint changes
are less common in the knee than in the hip, because of the ability of the knee
capsule to expand to accommodate the hyperplastic synovium.
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FIGURE 5.
Lateral radiograph of the knee showing a large cystic erosion in the inferior pole of the patella (arrows).

In patients with diffuse pigmented villonodular synovitis of the knee, magnetic

resonance imaging (MRI) may show a large effusion, low signal intensity on both
T1- and T2-weighted images (because of hemosiderin deposition), hyperplastic
synovium and occasional bony erosions2,3,12 (Figures 6,7 and 8).

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FIGURE 6.
Axial T2-weighted magnetic resonance image (MRI) of the knee in a patient with pigmented villonodular synovitis. The scan
shows a large effusion and villous proliferation arising from the synovial lining (arrow).

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FIGURE 7.
Axial T2-weighted MRI scan showing a lesion in the proximal fibula (arrow) that has a low intensity consistent with
pigmented villonodular synovitis.

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FIGURE 8.
Sagittal T2-weighted MRI scan demonstrating a cystic lesion in the proximal fibula (arrowhead), as well as proliferative
synovitis(arrow).

Pigmented Villonodular Synovitis of the Hip

The hip is the second most common location for pigmented villonodular synovitis.
Like patients with knee disease, those with hip disease usually present with deep
monoarticular pain of variable duration (a few months to a few years). The pain is
often alleviated only by rest. Patients may localize the pain to the anterior groin or
the lateral aspect of the hip. The pain is frequently intermittent, with completely
asymptomatic periods in which patients are able to participate in all activities.
Patients occasionally report episodes of extreme pain, which may represent
hemorrhage into the joint space. During these exacerbations, patients may be able
to relieve the pain by positioning their hip in a flexed and externally rotated
position. This positional relief of pain is typical of a joint effusion or inflammatory
process of the synovium, in that the repositioning minimizes pressure within the
joint.
Patients may also report decreased active and passive range of motion. A small
number of patients (6.9 percent) can recall a specific traumatic event associated
with the onset of symptoms,2 although this association may be coincidental.
IMAGING STUDIES

In pigmented villonodular synovitis of the hip, radiographs show bony erosions in

the head and neck of the femur and acetabulum in 95 percent of patients. 5 These
erosions are found early in the course of hip disease and appear as cystlike
structures on anteroposterior radiographs(Figure 9). An average of three to four
erosions, ranging from a few millimeters to 5 cm in diameter, are typically

seen5 (Figure 10). A thin sclerotic rim may also be present and is due to the slow
growth of the process.
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FIGURE 9.
Anteroposterior radiograph of the pelvis showing a cystic erosion in the right inferior medial femoral neck (black arrow),
medial femoral head (arrowhead) and acetabulum (white arrow).

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FIGURE 10.
Cystic erosions in the neck of the right femur.

Erosions are much more common in pigmented villonodular synovitis of the hip
than the knee, primarily because the tight capsule of the hip joint does not allow
the joint to expand to accommodate the hyperplastic synovium. As a result,
intracapsular pressure increases, and bone erosion occurs. In the late stages of the
disease, the articular joint space decreases, most often superolaterally, in 70 to 75
percent of patients.5
MRI is highly sensitive and specific for the diagnosis of pigmented villonodular
synovitis of the hip. Characteristic MRI findings include hip joint effusion, lifting

of the joint capsule, low signal intensity on both T1- and T2-weighted images
(because of hemosiderin deposition), hyperplastic synovium (that appears as a
lobulated synovial mass), bony erosions and preservation of bone density.10,11
On computed tomographic scans, the cystic erosions appear as a decreased signal
of the marrow of the femoral head (Figure 11).
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FIGURE 11.
Computed tomographic scan of the hip showing multiple erosions in the femoral neck (arrows).

Evaluation
Pigmented villonodular synovitis should be considered in the differential diagnosis
of patients from 20 to 45 years of age who have monoarticular symptoms (Table 1).
Most patients with this disease have a long history of pain and disability. The
presence of non-traumatic effusions of the hip or knee should further raise the
index of suspicion.
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TABLE 1
Differential Diagnosis of Nontraumatic Monoarticular Hip or Knee Problems*
Osteoarthritis
Inflammatory arthritis
Rheumatoid arthritis
Psoriatic arthritis
Systemic lupus erythematosus
Septic arthritis
Synovial chondromatosis
Pigmented villonodular synovitis
Benign or malignant bone tumor
Avascular necrosis (hip only)

*In patients 20 to 45 years of age.

Plain radiographs are the first studies that should be performed. In many patients,
radiographs will be normal or show only subtle findings. Patients with
nontraumatic knee effusions should then undergo arthrocentesis. The finding of
blood-tinged fluid is highly suggestive, although not pathognomonic, of pigmented
villonodular synovitis. MRI should be performed to further suggest the diagnosis
and define the extent of the disease process.
In patients with hip lesions, an effusion usually cannot be identified by palpation.
Thus, MRI is usually performed to search for hip effusion and hypertrophic
synovium before arthrocentesis is considered or attempted.
Technetium-99m etidronate bone scanning may show diffuse mild uptake when
bone erosions are present. However, the study may reveal no findings when the
disease is confined to the synovium. Thus, a normal bone scan does not exclude the
diagnosis of pigmented villonodular synovitis.

Treatment
The diagnosis of pigmented villonodular synovitis is confirmed by biopsy of the
synovium. The treatment of choice is synovectomy. Associated bony lesions should
be carefully curettaged, and bone grafting should be performed as necessary.
Diffuse pigmented villonodular synovitis has a high rate of local recurrence (up to
45 percent in one review4). The role of radiation therapy in the management of
refractory disease is not clear. In one retrospective series, 13 13 of 14 patients with
recurrent or extensive diffuse disease treated with radiation therapy were diseasefree at a mean follow-up period of 69 months. Eleven patients were characterized
as having good or excellent limb function, and three patients had fair function.
Radiotherapy can be considered in patients with previous adequate resection of
disease who experience local relapse and in patients with a large amount of disease
in whom complete resection is not possible. 13
Synovectomy may not relieve all symptoms in patients with significant destructive
changes in the joint. In these situations, arthrodesis or total joint replacement
should be considered. A series of 11 patients with active diffuse pigmented
villonodular synovitis of the knee treated with synovectomy and total knee
arthroplasty showed a local control rate of approximately 70 percent and good to
excellent joint function at a mean follow-up period of 10.8 years. 14