Pigmeted Villonodular Synovitis

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Original Editors

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Search Strategy

Databases:
• PubMed
• Web of Knowledge (Web of Science)
• Books
• Internet

Keywords: 
• (Localized/Diffuse) Pigmented Villonodular Synovitis
• Treatment
• Physiotherapy (Physical Therapy)
• Postoperative Therapy

Time Line: 
• 2000 – 2011

Definition/Description

Pigmented Villonodular Synovitis, also known as PVNS, is a rare idiopathic proliferative disorder of the synovium that leads to villous and or nodular formations within joints, tendon sheaths and bursae [5][6][9]. It affects adults between twenty and sixty years old, independent of the gender [1][12]. It may also uncommonly appear in children [8]. Despite the disease is benign, PVNS can be aggressive, spreading to surrounding synovial tissues and invading adjacent soft tissue structures and bone, resulting in effusions and bony erosion [9][11]. PVNS does not exhibit cellular atypia, but there is recent evidence of cytogenetic abnormalities [5], which may suggest a neoplastic origin [7]. In an early description were three forms of this disease defined [9]: an isolated lesion involving the tendon sheaths (giant cell tumour of the tendon sheath); a solitary intraarticular nodule (localized PVNS); and a diffused villous and pigmented lesion involving synovial tissue (diffuse PVNS) [5]. But more recently, PVNS is subdivided into two forms: isolated nodular lesions (localized form) and those with diffuse joint involvement (diffuse form) [9]. The localized form is also called ‘giant cell tumour of tendon sheath’ [4][8]. Both forms share the same histological characteristics [5][9], but it is essential to distinguish localized from diffuse because their clinical management differ, as do their responses to treatment [4].

Clinically Relevant Anatomy

Any joint can be affected but PVNS occurs mostly in the knee (80%), followed by the hip and the ankle (foot). To a lesser degree in the wrist, shoulder and elbow [1][5][8][9]. It may also appear in the spine, but it is infrequent [13].
The synovium, tendon sheaths, bursae and bone can be involved by patients with PVNS. In the spine, the disease originates from the vertebral articular facet joints [13].

Epidemiology /Etiology

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Characteristics/Clinical Presentation

The symptoms by patients with PVNS are sporadic or slowly progressive [5]. Some may experience pain, swelling, warmth and stiffness in the joint [5][8]. Locking and instability, mechanical symptoms, may develop [5]. PVNS in the spine may cause back pain, tenderness, and neurological dysfunction leading to acute paralysis [13].

PVNS is characterized by a unique gross and histologic structure consisting of brownish villous and nodular masses arising on the surface of synovial tissue and consisting of fibrous elements, hemosiderin containing macrophages, lipid-filled foam cells, multinucleated giant cells, and some inflammatory cells [1][5][7][9]. Hemosiderin is an iron-rich pigment, responsible for the brownish rusty colour [2][5][14]. 
Bilateral is rare, PVNS mostly occurs only in one joint (monoarticular) [1][8][9][6].
These characteristics count for both localized and diffuse PVNS [5][9].
The disease has a high risk of recurrence, but also a variant recurrence rate of 8% to 60% [1][11].

Differential Diagnosis

Diagnosing PVNS is relatively easily (on the basis of MRI etc.), but in the knee it is more difficult because the clinical presentation and symptoms are similar with more common disorders at the joint, such as internal derangements or arthritis [3][7].

Diagnostic Procedures

Results of laboratory studies, such as a complete blood count and erythrocyte sedimentation rate, are within normal limits and can help exclude infection and rheumatoid arthritis [5].
Joint aspiration reveals a brown, red or yellow fluid [5].
Radiographs reveal, in earlier stages, periarticular synovial swelling, absence of synovial calcification, normal bone density, and preservation of the cartilage space [1][5].
A magnetic resonance image (MRI) will show nodular foci of decreased signal on both T1 and T2 images, because of the release of hemosiderin. Additionally, low signal on fast field echo (FFE) sequences are helpful in the diagnosis of PVNS [5].
If the diagnosis still remains in question, an arthroscopic exam can show the gross appearance of the lesion [5].
A biopsy can confirm the diagnosis of PVNS [5].
The diagnosis by PVNS in the spine can be confirmed by histopathology [13].

Outcome Measures

Patients can be evaluated according to the Knee Society Score [1].
http://www.orthopaedicscore.com/scorepages/knee_society_score.html

Examination

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Medical Management

Good results can be obtained with local excision of a solitary nodule [5][7][11]. However, once diffuse PVNS is diagnosed, a total synovectomy is recommended [2][5][12]. Arthroscopic resection may be useful, if the MRI shows the lesion to be accessible by arthroscopy, and if it is associated with relatively low morbidity [5][9]. But an open synovectomy may be necessary because lesions extend beyond the reach of the arthroscope [1][5]. Unfortunately, synovectomy has a high recurrence rate [2][5][11].  This also applies  to arthroscopic synovectomy [5]. But actually it is the type of surgery and the disease subtype that is associated with recurrence [11].
If diffuse PVNS is diagnosed, especially in an older patient with degenerative joint disease, an arthroplasty can give good outcomes [5]. In a younger patient, arthrodesis may be considered as a savage procedure [5].
Radiation therapy is recommended as an adjuvant to surgery to control the disease process [1][5][7][9].
Intra-articular injection of yttrium 90 or dysprosium after surgery can be used to treat diffuse PVNS [5][10].  But the effectiveness is inconclusive [5][7].
Eventually, a total joint replacement can be required, particularly in the hip [7]. 

Physical Therapy Management

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Key Research

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Resources

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Clinical Bottom Line

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Recent Related Research (from Pubmed)

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References

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