eMedicine Specialties > Radiology > Musculoskeletal

Pigmented Villonodular Synovitis

Author: Johnny U V Monu, MD, Associate Professor of Radiology and Orthopedics, 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
Contributor Information and Disclosures

Updated: Jun 21, 2007

Introduction

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.

Presentation

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.

Differential Diagnoses

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.

More on Pigmented Villonodular Synovitis

Overview: Pigmented Villonodular Synovitis
Imaging: Pigmented Villonodular Synovitis
Multimedia: Pigmented Villonodular Synovitis
References
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References

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Further Reading

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Keywords

PVNS, xanthoma, xanthogranuloma, fibroxanthoma, giant cell tumor of tendon sheath, giant cell fibrohemangioma, benign synovioma, villous arthritis

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Contributor Information and Disclosures

Author

Johnny U V Monu, MD, Associate Professor of Radiology and Orthopedics, 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, MD is a member of the following medical societies: Radiological Society of North America
Disclosure: Nothing to disclose.

Medical Editor

Amilcare Gentili, MD, Clinical Professor of Radiology, University of California at San Diego; Consulting Staff, Department of Radiology, Thornton Hospital
Amilcare Gentili, MD is a member of the following medical societies: American Roentgen Ray Society, Radiological Society of North America, and Society of Skeletal Radiology
Disclosure: Nothing to disclose.

Pharmacy Editor

Bernard D Coombs, MB, ChB, PhD, Consulting Staff, Department of Specialist Rehabilitation Services, Hutt Valley District Health Board, New Zealand
Disclosure: Nothing to disclose.

Managing Editor

Lynne S Steinbach, MD, Chief of Musculoskeletal Radiology, Professor, Department of Radiology, University of California at San Francisco
Disclosure: Nothing to disclose.

CME Editor

Robert M Krasny, MD, Consulting Staff, Department of Radiology, The Angeles Clinic and Research Institute
Robert M Krasny, MD is a member of the following medical societies: American Roentgen Ray Society and Radiological Society of North America
Disclosure: Nothing to disclose.

Chief Editor

Felix S Chew, MD, MBA, EdM, Professor, Department of Radiology, Vice Chairman for Radiology Informatics, Section Head of Musculoskeletal Radiology, University of Washington
Felix S Chew, MD, MBA, EdM is a member of the following medical societies: American Roentgen Ray Society, Association of University Radiologists, and Radiological Society of North America
Disclosure: Nothing to disclose.

 
 
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