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Pigmented villonodular synovitis (PVNS) is a rare, benign locally aggressive disorder of the synovium of joints, bursae and tendon sheaths and has three main subtypes. The World Health Organisation classifies intra-articular diffuse PVNS as ‘diffuse-type giant cell tumour’ (Dt-GCT), differentiating it from the localised intra-articular form of PVNS and from GCTTS (giant cell tumours of tendon sheaths) commonly encountered in fingers and toes. The histological appearances are similar across all three PVNS sub-types (https://www.sciencedirect.com/science/article/pii/S1877132712000991).
The pathoaetiology of PVNS has been linked to inflammatory, vascular and traumatic causes but chromosomal abnormalities, autonomous growth, bone invasion and the rare malignant transformation of PVNS suggest a neoplastic cause (https://online.boneandjoint.org.uk/doi/epub/10.1302/0301-620X.95B3.30192).
Typically patients are between the age of 20 and 50 years, although many examples have been reported involving infants, children and adolescents (https://online.boneandjoint.org.uk/doi/epub/10.1302/0301-620X.95B3.30192). As the diffuse-type insidiously infiltrates the synovial cavity lining leading to eventual osseous erosions and sub- chondral cysts, patients present with insidious swelling and/or pain which has been present for months or years.
The mainstay of treatment for diffuse PVNS remains radical surgical excision. The recurrence rate of diffuse PVNS of knee after attempted arthroscopic resection is very high, therefore open surgery is preferred as the recurrence rate is reported to be less than 30% after two years. (https://europepmc.org/article/PMC/2758986)
Readers will also find it useful to read Jonathan Stevensons complimentary technique Excision of synovial chondromatosis using a posterior approach to the knee

INDICATIONS
The indications for surgical resection are pain, stiffness and swelling after the histology has been confirmed as PVNS after a biopsy. Some patients may present with intermittent symptoms that are not too intrusive and radiologically the disease may be in areas of the joint difficult to access e.g. posterior capsule and posterior cruciate ligament. In such cases clinical and radiological observation is sufficient after the natural history of the condition has been explained to the patient.
SYMPTOMS & EXAMINATION
Examination may reveal firm swellings, diffuse tenderness, effusion and restricted movement. In one large series, swelling and pain were found in 79% of patients, restriction of movement in 26% and a palpable mass in 6% of cases at presentation (Granowicz SP, D’Antonio J, Mankin HL. Pathogenesis and long-term end results of pigmented villonodular synovitis. Clin Orthop 1976; 114: 335e51.) Haemarthrosis was present in 75% of patients with diffuse PVNS of the knee in whom synovial fluid was analysed.
IMAGING
Plain radiographs are mandatory. As the disease progresses, early intra-articular synovially based soft-tissue swellings without calcification and effusion with preserved joint spaces are typical, followed by juxta-articular and sub-chondral erosions. Cysts develop in advanced disease.
MRI is the investigation of choice enabling extent, erosion and invasion to be demonstrated which is crucial in planning surgical approaches. It is reported to be 100% sensitive and specific for detecting diffuse PVNS of the knee pre-operatively. It will demonstrate synovially based nodular swellings with heterogeneous low to intermediate signal on both T1 and T2 weighted sequences. The difference in magnetic susceptibility between haemosiderin-laden PVNS and surrounding tissue may cause the lesion to appear larger (‘blooming artefact’) on gradient echo images. Synovial masses may be extensive in diffuse PVNS involving adjacent synovial structures (semi- membranosus, semitendinosus and gastrocnemius bursae), beneath menisci and extending into the popliteal fossa.
ALTERNATIVE OPERATIVE TREATMENT
For localised PVNS, arthroscopic resection of pedunculated lesions may be possible, but for diffuse PVNS no alternative strategy to open synovectomy currently exists.
NON-OPERATIVE MANAGEMENT
Cryotherapy, external beam radiotherapy and intra-articular injections of radioactive colloid (synoviortheses or radiation synovectomy) have produced low recurrence rates for lesions involving the knee but complications described have included stiffness, skin necrosis and deep infection. Radiation therapy should probably be considered only in patients in whom lesions recur after surgical synovectomy (https://europepmc.org/article/med/770040).
The translocation of the colony-stimulating factor (CSF1) gene has been implicated in the pathogenesis of diffuse type PVNS; Imatinib (a tyrosine-kinase inhibitor) infusions, which block CSF receptor activation, may have a therapeutic role in refractory cases, although toxicity has been reported in up to 22% of patients (The translocation of the colony-stimulating factor (CSF1) gene have been implicated in the pathogenesis of Dt-GCT. Imatinib (a tyrosine-kinase inhibitor) infusions, which block CSF receptor activation, may have a therapeutic role in refractory Dt-GCT lesions, although toxicity has been reported in up to 22% of patients (https://online.boneandjoint.org.uk/doi/epub/10.1302/0301-620X.95B3.30192).
CONTRAINDICATIONS
Absolute contraindications include morbidity preventing general anaesthesia. Relative contraindications include absence of symptoms or absence of radiological features indicating bone invasion.


24 hours of intravenous antibiotics.
Drain to be removed when less than 100mls per 24 hours, no longer than 5 days.
Full and immediate weight bearing.
Venous thromboembolism prophylaxis: low molecular weight heparin for two weeks and thromboembolic stockings for six weeks.
Removal of skin clips at 14 days.
Clinic review at six weeks with outpatient physiotherapy.
To achieve full range of motion of the joint prior to discharge from hospital.

The estimated incidence of diffuse PVNS is 1.8 annually per million people, highlighting its rarity. A 1980 literature review revealed that of 1301 lesions, 69% were GCTTS, 8% localised PVNS and 29% diffuse type PVNS (https://www.ncbi.nlm.nih.gov/pubmed/7412554); 66% of lesions were found in the knee, 16% in the hip, 7% in the ankle, and 2% affected joints in the foot, with infrequent cases involving the wrist, shoulder or elbow. Diffuse PVNS commonly involves more than one joint and/or tendon sheath (https://online.boneandjoint.org.uk/doi/full/10.1302/0301-620X.95B3.30192).
Diffuse type PVNS of the knee has a high recurrence rate as it is locally aggressive and invades adjacent soft-tissue structures and bone. The two factors associated with a higher recurrence rate are diffuse-type PVNS and resection by arthroscopic partial synovectomy. Diffuse PVNS is more common and has a higher recurrence rate compared to the nodular type (https://europepmc.org/article/PMC/2758986).
Surgery is dictated by symptoms and in my unit we traditionally offer anterior resection and review clinically and use serial MRI scanning before moving onto a more risky posterior synovectomy of the knee if symptoms require it. The posterior approach carries additional risks of stiffness, contractures, reflex sympathetic dystrophy (complex regional pain syndrome), neurovascular and wound complications and is described on Orthoracle at Excision of synovial chondromatosis using a posterior approach to the knee; however this remains the most effective strategy to minimise the risk of recurrence (https://www.sciencedirect.com/science/article/abs/pii/S1877132712000991).
Infrequently, further surgery including arthroplasty (5%) or arthrodesis (6%) may be necessary after synovectomy of the knee. In contrast, most patients with hip PVNS subsequently require total joint arthroplasty. In advanced or recurrent PVNS CSF-1 receptor blocker Imatinib has proven to control disease in 48% of cases after five years although 66% of patients stopped taking the drug due to side-effects and toxicity (https://www.nature.com/articles/s41598-019-51211-y.pdf?origin=ppub).
Reference
- orthoracle.com

































