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Radiology > MUSCULOSKELETAL
Pigmented Villonodular Synovitis
Article Last Updated: Jun 21, 2007
AUTHOR AND EDITOR INFORMATION
Section 1 of 9  
Author: Johnny U V Monu, MD, Associate Professor of Radiology, University of Rochester School of Medicine, Program Director and Co-Head of Musculoskeletal Radiology, Head of Emergency Radiology, Department of Radiology, University of Rochester Strong Memorial Medical Center
Johnny U V Monu is a member of the following medical societies: Radiological Society of North America
Coauthor(s):
Simone Elvey, MD, Assistant Professor, Department of Radiology, Strong Memorial Hospital
Editors: Amilcare Gentili, MD, Clinical Professor of Radiology, University of California at San Diego; Consulting Staff, Department of Radiology, Thornton Hospital; Bernard D Coombs, MB, ChB, PhD, Consulting Staff, Department of Specialist Rehabilitation Services, Hutt Valley District Health Board, New Zealand; Lynne S Steinbach, MD, Chief of Musculoskeletal Radiology, Professor, Department of Radiology, University of California at San Francisco; Robert M Krasny, MD, Consulting Staff, Department of Radiology, The Angeles Clinic and Research Institute; Felix S Chew, MD, MBA, EdM, Professor, Department of Radiology, Vice Chairman for Radiology Informatics, Section Head of Musculoskeletal Radiology, University of Washington
Author and Editor Disclosure
Synonyms and related keywords:
PVNS, xanthoma, xanthogranuloma, fibroxanthoma, giant cell tumor of tendon sheath, giant cell fibrohemangioma, benign synovioma, villous arthritis
Background
Pigmented villonodular synovitis (PVNS) is a benign proliferative disorder of uncertain etiology that affects synovial lined joints, bursae, and tendon sheaths. The disorder results in various degrees of villous and/or nodular changes in the affected structures. Two primary forms are described, including a diffuse form that affects the entire synovial lining of a joint, bursa, or tendon sheath, and a rare focal, or localized, form. The diffuse form typically involves the large joints, while the localized form typically occurs around the small joints of the hands and feet. The localized form often appears around tendon sheaths, in which case it is termed giant cell tumor of the tendon sheath. Rarely, the localized form may develop around large joints. The term PVNS generally is used when the condition, in either diffuse or localized form, affects a joint. Imaging plays an important role in the diagnosis, treatment, and follow-up monitoring of the disorder.
Pathophysiology
Gross pathologic features include thickened synovium, with a combination of villous and nodular proliferation depending on the site of involvement. Two types of villi are present in the diffuse form of PVNS, including coarse villi with a "shag carpet" appearance, and fine or fernlike villi. The nodular component is seen predominantly in tendinous or extra-articular lesions. The nodules are well demarcated and may be sessile or pedunculated, although they lack a true capsule. On microscopy, PVNS is characterized by the presence of hemosiderin-laden, multinucleated, giant cells. In addition, lipid-laden macrophages, fibroblasts, and other large, polyhedral-shaped, mononuclear cells are present; they have abundant cytoplasm and possess oval nuclei. Hemosiderin also is found within the surrounding tissues. The ubiquitous presence of hemosiderin lends the tissue a characteristic pigmented appearance. The lesions tend to be hypervascular and demonstrate synovial hyperplasia. PVNS typically invades local tissues; the invasion of the subchondral bone, with resultant cyst formation, is a characteristic finding. Etiology remains controversial, although several theories have been formed based on the legion's histologic appearance and cellular components. Theories regarding the cause of PVNS include the following: - Localized lipid metabolic derangement
- Repeated nontraumatic inflammation
- A benign neoplastic process
- A response to blood or blood products within the joint
Frequency
United States
Annual incidence of PVNS is estimated at 2 cases per million population; incidence of the localized form is 9 cases per million.
Mortality/Morbidity
PVNS is a benign condition associated with decreased motion around the affected joint. Patients may also complain of a dull ache or pain in the affected joint.
Age
PVNS has been described in pediatric and elderly populations, but it occurs more often in patients aged 20-50 years.
Anatomy
When the incidence of both forms of PVNS is taken into account, the joint most often affected is the knee (approximately 80%), with the hip, ankle, and shoulder being less commonly impacted. The disease usually is monoarticular. Considered separately, the localized form occurs most frequently in the fingers—in particular, in the volar aspect of the first 3 fingers. It is the most common soft-tissue tumor of the hand.
Clinical Details
Patients usually present with painless joint swelling of insidious onset that mimics joint effusion. Joint pain subsequently supervenes, but the swelling is disproportionate to the degree of pain. The pain is mild and of insidious onset, and it progressively worsens and frequently is accompanied by decreased range of motion and locking of the joint. Recurrent mild to moderate effusion creates the impression of recurrent joint swelling. Acute pain may occur with nodular torsion and/or infarction. The localized form of the disease usually has a female predominance and presents as a pain-free, slowly enlarging mass most frequently occurring on the flexor aspect of the fingers. Hemarthrosis is a relatively common finding; however, a history of preceding trauma is uncommon. Physical examination reveals one or more palpable nodules or diffuse joint swelling. Swelling may feel warm and be somewhat tender to palpation.
Preferred Examination
Clinical information and plain radiographs are not always sufficient to establish a correct diagnosis. Magnetic resonance imaging (MRI) findings are characteristic, but not pathognomonic, for this disorder. Rarely, biopsy is required to establish preoperative tissue diagnosis. Plain radiographs demonstrate signs similar to joint effusion or soft-tissue swelling. Calcifications are not a usual feature of PVNS. Rarely, foci of dystrophic calcification may be seen in an area of PVNS. Computed tomography (CT) scans demonstrate a hyperdense soft-tissue mass in the joint or tendon sheath. The hyperdensity of the mass is a reflection of repeated hemorrhage and of blood degradation products within the joint. MR images demonstrate various appearances ranging from low signal through isointense to hyperintense signals on spin-echo images, reflecting the presence of blood and its degradation products. Hemosiderin appears as low signal on T1- and T2-weighted images. Differentiating calcifications from hemosiderin-laden foci in the setting of PVNS may be difficult, and plain films should be used in this setting to confirm or deny the presence of calcifications. A combination of plain films and MRI should be used in preoperative evaluation of a patient with PVNS. This combination yields an accurate diagnosis and maps out the extent of disease for the surgeon prior to treatment.
Limitations of Techniques
Plain radiographs cannot confidently exclude effusion as a cause of symptoms, nor can they help determine the extent of disease. CT scan findings invariably are diagnostic; however, CT scanning is hobbled by its inability to completely show the extent of disease and other pathology around or within the joint. MRI findings are diagnostic in more than 95% of patients. Rarely, synovial osteochondromatosis (SOC) may demonstrate a similar appearance, and plain radiographs may be necessary to exclude SOC in the appropriate setting.
Gout
Hemochromatosis
Synovial Osteochondromatosis
Synovial Sarcoma
Other Problems to Be Considered
Differential diagnosis depends on predominating radiographic findings and includes tuberculosis, synovial osteochondromatosis, hemophilic arthropathy, synovial hemangioma, secondary osteoarthritis, and amyloid arthropathy.
Findings
Findings on plain radiographs are not specific. - In the diffuse form, PVNS presents as painless, monoarticular joint swelling.
- Findings of mineralization of the bones around the lesion are normal until late in the disease, with preservation of the cartilage space and no calcifications.
- Well-corticated pressure erosions (saucerization) and cysts may occur on either side or both sides of the joint.
- Secondary degenerative changes may occur in the affected joint in long-standing disease, with concentric cartilage space narrowing, subchondral cyst, and osteophyte formation.
- The diffuse form presents with joint effusion/soft-tissue swelling. Occasionally, the effusion is dense due to the presence of hemosiderin.
- The nodular form most commonly results in localized swelling of the palmar aspect of a finger.
Findings
CT scan findings are as follows: - Secondary to the presence of intracellular and extracellular hemosiderin, lesions have high attenuation and appear hyperdense on CT scans.
- Because of improved tissue contrast, CT scanning is valuable in delineating bone cysts and erosions.
- Affected synovium is hypervascular and generally enhances following administration of radiographic contrast.
- CT scanning is useful for needle biopsy guidance when a histologic diagnosis is required.
- CT scanning is especially useful for preoperative planning.
Arthrography findings
Radiographic contrast may be injected into the joint following joint aspiration. With contrast filling of the joint, findings demonstrate multiple, irregular, nodular filling defects of variable sizes. These produce the typical cobblestone appearance of the synovium seen on arthrography.
Findings
MRI has become the imaging modality of choice in the evaluation of synovial and soft-tissue lesions. PVNS demonstrates variable appearance on MRI, depending on the composition of the lesion and its relative proportions of hemosiderin, lipid, fibrous tissue, cyst formation, and cellular elements and on the imaging parameters. MRI findings are as follows:
- Characteristic findings include nodular intra-articular masses that demonstrate low signal intensity on T1-, T2-, and proton density–weighted sequences. Low signal intensity is due to hemosiderin deposits within the affected tissue and is accentuated on T2-weighted sequences, most notably on gradient-recalled echo sequences.
- The presence of fat signal also is apparent secondary to the presence of lipid-laden macrophages, resulting in focal regions of high signal intensity on T1-weighted images and intermediate signal on T2-weighted images.
- Hyperplastic and hypervascular synovium enhances following intravascular administration of gadolinium chelates.
- Gadolinium-based contrast agents (gadopentetate dimeglumine [Magnevist], gadobenate dimeglumine [MultiHance], gadodiamide [Omniscan], gadoversetamide [OptiMARK], gadoteridol [ProHance]) have recently been linked to the development of nephrogenic systemic fibrosis (NSF) or nephrogenic fibrosing dermopathy (NFD). For more information, see the eMedicine topic Nephrogenic Fibrosing Dermopathy. The disease has occurred in patients with moderate to end-stage renal disease after being given a gadolinium-based contrast agent to enhance MRI or magnetic resonance angiography scans. As of late December 2006, the Food and Drug Administration (FDA) had received reports of 90 such cases. Worldwide, over 200 cases have been reported, according to the FDA. NSF/NFD is a debilitating and sometimes fatal disease. Characteristics include red or dark patches on the skin; burning, itching, swelling, hardening, and tightening of the skin; yellowspots on the whites of the eyes; joint stiffness with trouble moving or straightening the arms, hands, legs, or feet; pain deep in the hip bones or ribs; and muscle weakness. For more information, see the FDA Public Health Advisory or Medscape.
- Bony erosions (when present) and extra-articular extension of the lesion are well demonstrated on MRI.
False Positives/Negatives
MRI findings are not pathognomonic for PVNS, and similar findings may be seen in rheumatoid arthritis, hemophilic arthropathy, amyloid arthropathy, synovial osteochondromatosis, gout, and degenerative joint disease.
Findings
Bone scans do not play a significant or routine role in the diagnosis of PVNS. On bone scan, the hypervascularity and areas of erosion may result in increased radionuclide uptake. Soft-tissue masses often demonstrate increased uptake on blood-pool images.
| Media file 1:
Pigmented villonodular synovitis of the knee. Plain radiographic findings of the knee apparently are normal except for the curvilinear calcification seen peripherally to the medial femoral condyle. |
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| Media file 2:
Pigmented villonodular synovitis of the knee. Coronal T2-weighted magnetic resonance imaging (MRI) scan shows low signal mass above the medial femoral condyle, posteriorly. |
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| Media file 3:
Pigmented villonodular synovitis of the knee. Sagittal T1-weighted magnetic resonance imaging scan (MRI) of the knee shows inhomogeneous foci of low signal in the suprapatellar pouch and, posteriorly, just above the femoral condyle. |
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| Media file 4:
Pigmented villonodular synovitis of the knee. Axial T1- and T2-weighted magnetic resonance imaging (MRI) scans show tissue areas with abnormally low and high signals anteriorly in the suprapatellar pouch (see also Image 5) and, posteriorly, deep and superficial to the medial head of the gastrocnemius (Baker and popliteal bursae). |
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Media type: MRI
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| Media file 5:
Pigmented villonodular synovitis of the knee. Axial T1- and T2-weighted magnetic resonance imaging (MRI) scans show tissue areas with abnormal low and high signals anteriorly in the suprapatellar pouch (same patient as in Image 4) and, posteriorly, deep and superficial to the medial head of the gastrocnemius (Baker and popliteal bursae). |
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Media type: MRI
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| Media file 6:
Pigmented villonodular synovitis of the knee. Axial T1- and T2-weighted magnetic resonance imaging (MRI) scans performed at a higher level above the knee joint (same patient as in Images 4-5) show mixed signal pattern in the suprapatellar pouch. Nodular, low-signal foci are interspersed within inhomogeneous low- and high-signal areas (see also Image 7). The foci correspond to areas of hemosiderin-laden hyperplastic synovium of pigmented villonodular synovitis. |
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Media type: MRI
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| Media file 7:
Pigmented villonodular synovitis of the knee. Axial T1- and T2-weighted magnetic resonance imaging (MRI) scans performed at a higher level above the knee joint (same patient as in Images 4-6) show mixed signal pattern in the suprapatellar pouch. Nodular, low-signal foci are interspersed within inhomogeneous low- and high-signal areas (see also Image 6). The foci correspond to areas of hemosiderin-laden hyperplastic synovium of pigmented villonodular synovitis. |
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Media type: MRI
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| Media file 8:
Giant cell tumor of tendon sheath. Anteroposterior plain film of the foot shows a circumferential mass, which is more apparent on the medial aspect of the proximal phalanx of the great toe. Note the degenerative cystic changes at the interphalangeal joint of the great toe. Mild saucerization or pressure erosion of the medial cortex of the proximal phalanx is seen. |
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| Media file 9:
Giant cell tumor of tendon sheath. Axial T1-weighted magnetic resonance imaging (MRI) scan of the foot shows intermediate signal mass surrounding the proximal phalanx of the great toe and displacing the tendons away from the bone, especially in the plantar aspect of the foot. |
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Pigmented Villonodular Synovitis excerpt Article Last Updated: Jun 21, 2007
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